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CP Reviewer 2 > Hematology > Flashcards

Hematology Flashcards

1
Q

Hereditary spherocytosis: Incidence (2).

A

United States: 1 in 5000.

Northern Europe: 1 in 1000.

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2
Q

Hereditary spherocytosis: Possible clinical manifestations (4).

A

All patients: Anemia.

Infants: Jaundice.

Adults: Gallstones, splenomegaly.

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3
Q

Hereditary spherocytosis: Complete blood count (3).

A

Elevated MCHC.

Normal MCV, MCH.

Reticulocytosis.

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4
Q

Hereditary spherocytosis: Tests of stability of red cells.

A

Increased osmotic fragility.

Increased autohemolysis.

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5
Q

Hereditary spherocytosis: Other laboratory findings (2).

A

Increased unconjugated bilirubin.

Increased LDH.

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6
Q

Hereditary elliptocytosis: Incidence (2).

A

United States: 1 in 2500.

Parts of Africa: 1 in 100.

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7
Q

Hereditary elliptocytosis: Clinical manifestations.

A

Mild disease.

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8
Q

Hereditary elliptocytosis:

A. Inheritance.
B. Molecular defect.

A

A. Autosomal dominant.

B. The α chain of spectrin.

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9
Q

Hereditary elliptocytosis: Types.

A

Common: Found in Africans; includes hereditary pyropoikilocytosis.

Spherocytic: Double heterozygosity of HE and HS.

Stomatocytic: Found in Malaysians.

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10
Q

Hereditary elliptocytosis, stomatocytic type:

A. Synonym.
B. Molecular defect.
C. Evolutionary advantage.

A

A. Southeast Asian ovalocytosis.

B. Band 3.

C. Protection against Plasmodium vivax.

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11
Q

Glucose-6-phosphate dehydrogenase deficiency: Examples of oxidative stress (6).

A

Fava beans.
Methylene blue.

Sulfa drugs.
Primiquine.
Infections.
Nitrofurantoin.

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12
Q

Glucose-6-phosphate dehydrogenase deficiency: Peripheral smear.

A

Heinz bodies (revealed by supravital stain).

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13
Q

Glucose-6-phosphate dehydrogenase deficiency: Inheritance.

A

X-linked recessive.

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14
Q

Pyruvate kinase deficiency:

A. Effect on cell.
B. Clinical presentation.
C. Laboratory findings.

A

A. Cannot produce enough ATP; cannot maintain ion pumps.

B. Chronic hemolysis of variable severity.

C. Echinocytosis; evidence of extravascular hemolysis.

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15
Q

Pyruvate kinase deficiency:

A. Epidemiology.
B. Inheritance.

A

A. Occurs worldwide.

B. Autosomal recessive.

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16
Q

Hemoglobin chains:

A. Hb A.
B. Hb A₂.
C. Hb F.

A

A. α₂β₂.

Β. α₂δ₂.

C. α₂γ₂.

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17
Q

Chains of early fetal hemoglobins.

A

Hb Gower 1: ζ₂ε₂.

Hb Gower 2: α₂ε₂.

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18
Q

Hemoglobin S:

A. Mutation.
B. Incidence of trait in the United States.

A

A. β₆ Glu to Val.

B. 8% in blacks.

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19
Q

Sickle-cell trait: Hemoglobins.

A

Hb A: 50-65%.

Hb S: 35-45%.

Hb A₂: Less than 3%.

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20
Q

Sickle-cell trait:

A. Peripheral smear.
B. Screening tests.

A

A. Normal.

B. Positive metabisulfite and dithionate tests.

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21
Q

Sickle-cell trait: Renal complications (4).

A

Hematuria.

Isosthenuria.

Papillary necrosis.

Renal medullary carcinoma.

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22
Q

Sickle-cell trait: Other possible complications (2).

A

Hypoxia-induced splenic infarct.

Exercise-induced rhabdomyolysis.

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23
Q

Sickle-cell disease: Lifespan of red cells.

A

17 days.

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24
Q

Sickle-cell disease: Hemoglobins.

A

Hb S: More than 80%.

Hb F: 1-20%.

Hb A: 0%.

Hb A₂: 1-4%.

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25
Q

Sickle-cell disease: Onset of symptoms.

A

Occurs at 6 months of age, when Hb S is 50% of total hemoglobin.

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26
Q

Sickle-cell disease: Effect of Hb F.

A

Prevents polymerization of Hb S.

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27
Q

SC disease:

A. Clinical severity.
B. Hemoglobins.

A

A. Worse than sickle-cell trait but not as bad as sickle-cell disease.

B. Hb S makes a little more than half of total hemoglobin; Hb C makes up the rest.

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28
Q

Sickle-cell trait/α thalassemia: Percentage of Hb S (2).

A

One mutated α gene: 30-35% Hb S.

Two mutated α genes: 25-30% Hb S.

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29
Q

Sickle-cell trait/β thalassemia:

A. Percentage of Hb S.
B. Clinical severity.

A

A. Usually more than 50%.

B. May be severe.

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30
Q

Hemoglobin C: Mutation.

A

β₆ Glu to Lys.

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31
Q

Hemoglobin C trait:

A. Peripheral smear.
B. Clinical severity.

A

A. Target cells.

B. Generally asymptomatic.

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32
Q

Hemoglobin C disease: Hemoglobins.

A

Hb C: 90%.

Hb F: 7%.

Hb A: 0%.

Hb A₂: 3%.

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33
Q

Hemoglobin C disease:

A. Peripheral smear.
B. Clinical severity.

A

A. Target cells; rod-shaped or hexagonal crystals.

B. Mild hemolytic anemia; splenomegaly.

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34
Q

Hemoglobin E: Mutation.

A

β₂₆ Glu to Lys.

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35
Q

Hemoglobin E:

A. Peripheral smear and CBC.
B. Clinical severity.

A

A. Target cells, thalassemic indices.

B. Mild anemia unless there is concurrent β thalassemia.

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36
Q

Hemoglobins D and G:

A. Clinical severity.
B. Defect.

A

A. Asymptomatic.

B. Hb D: Defect in β chain; Hb G: Defect in α chain.

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37
Q

Hemoglobins D and G:

A. Gel electrophoresis.
B. Identification.

A

A. Both run with Hb S on alkaline gel but not on citrate gel.

B. Negative metabisulfite, dithionate tests.

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38
Q

Hemoglobin Lepore: Epidemiology.

A

Found in Italy and other Mediterranean lands.

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39
Q

Hemoglobin Lepore: Identification (4).

A

Makes up 15% of total hemoglobin.

Thalassemic indices.

Runs with Hb S on alkaline gel.

Negative metabisulfite, dithionate tests.

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40
Q

Hemoglobin Lepore: Mutation.

A

Fusion of genes for δ and β chains.

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41
Q

Hemoglobin Constant Spring:

A. Epidemiology.
B. CBC.

A

A. More common in Southeast Asia.

B. Thalassemic indices.

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42
Q

Hemoglobin Constant Spring: Mutation.

A

Loss of stop codon in the gene for the α chain results in an abnormally long transcript.

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43
Q

pH of ___ gel.

A. cellulose-acetate
B. citrate

A

A. 8.6.

B. 6.2.

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44
Q

Cellulose-acetate gel: Layout.

A

+

A
F
S D G Lepore
A₂ C E O

(carbonic anhydrase)
(origin)

−

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45
Q

Citrate gel: Layout.

A

+

C
S

(origin)

A D A₂ G E O
F

−

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46
Q

Causes of shift of the hemoglobin-oxygen dissociation curve to the right.

A

Acidosis.

Hyperthermia.

Increased 2,3-DPG.

Hemoglobin with low affinity for oxygen.

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47
Q

Clinical features of a hemoglobin with ___ affinity for oxygen.

A. low
B. high

A

A. Anemia, cyanosis.

B. Erythrocytosis.

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48
Q

Unstable hemoglobins:

A. Definition.
B. Peripheral smear.
C. Examples.

A

A. Easily oxidized.

B. Heinz bodies, bite cells.

C. Hb Köln, Hb Hammersmith, Hb Ann Arbor.

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49
Q

Methemoglobin:

A. Synonym.
B. Definition.
C. Importance.

A

A. Hemiglobin.

B. Hemoglobin that contains ferric iron.

C. Cannot carry oxygen.

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50
Q

Methemoglobin: Amount in normal blood.

A

No more than 1.5%.

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51
Q

Methemoglobin: Inherited causes of abnormal levels (2).

A

Deficiency of methemoglobin reductase.

Abnormal hemoglobin that resists the reductase.

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52
Q

Methemoglobin: Acquired causes of abnormal levels (4).

A

Nitrites.

Phenacetin.

Quinones.

Sulfonamides.

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53
Q

Methemoglobin: Level at which cyanosis appears.

A

At 1.5 g/dL or about 10% of total hemoglobin.

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54
Q

Methemoglobin:

A. Detection.
B. Treatment.

A

A. Co-oximetry.

B. Methylene blue.

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55
Q

Sulfhemoglobin: Peripheral smear.

A

Heinz bodies.

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56
Q

Sulfhemoglobin:

A. Amount in normal blood.
B. Causes of increase.

A

A. No more than 1%.

B. Sulfonamides; bacteremia with Clostridium perfringens.

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57
Q

Sulfhemoglobin: Level at which cyanosis appears.

A

At 0.5 g/dL or about 3-4% of total hemoglobin.

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58
Q

Tetramers in thalassemia:

A. Significance.
B. In α thalassemia.
C. In β thalassemia.

A

A. May precipitate and shorten the life span of the red cell.

B. β₄, γ₄.

C. α₄.

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59
Q

Thalassemia: Formula for distinguishing from iron-deficiency anemia.

A

MCV ÷ RBC

− Less than 13: Probable thalassemia.
− More than 15: Probable iron deficiency.

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60
Q

Thalassemia: Peripheral smear (3).

A

Target cells.

Microcytosis.

Basophilic stippling.

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61
Q

β-Thalassemia:

A. Chromosome.
B. Type of mutation.

A

A. 11.

B. Point mutation.

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62
Q

β-Thalassemia: Alleles.

A

β+: Some β chains are produced.

β⁰: No β chains.

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63
Q

β-Thalassemia: Age at presentation.

A

6 to 9 months.

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64
Q

β-Thalassemia: Hemoglobins in homozygotes.

A

Hb F: 50-95%.

Hb A: Very low or absent.

Hb A₂: Normal or increased.

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65
Q

β-Thalassemia: Difference between major and intermedia.

A

Major: Transfusion dependent.

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66
Q

δβ-Thalassemia: Definition.

A

Mutation involving the δ and β genes.

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67
Q

δβ-Thalassemia: Hemoglobins.

A

Hb F: 5-20%.

Hb A: Decreased.

Hb A₂: Normal.

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68
Q

δβ-Thalassemia vs. Hb Lepore on hemoglobin electrophoresis.

A

Hb Lepore runs with Hb S on alkaline gel and makes up no more than 15% of total hemoglobin.

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69
Q

α-Thalassemia:

A. Chromosome.
B. Typical mutation.

A

A. 16.

B. Large deletion.

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70
Q

α-Thalassemia 1 haplotype:

A. Synonym.
B. Definition.
C. Epidemiology.

A

A. α⁰.

B. Deletion of both genes from the chromosome.

C. Asians.

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71
Q

α-Thalassemia 2 haplotype:

A. Synonym.
B. Definition.
C. Epidemiology.

A

A. α+.

B. Deletion of one gene from the chromosome.

C. Blacks.

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72
Q

α-Thalassemia, silent carrier:

A. Genotype.
B. CBC and peripheral smear.

A

A. -α/αα.

B. Normal.

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73
Q

α-Thalassemia trait:

A. Genotype.
B. CBC and peripheral smear.

A

A. -α/-α or –/αα.

B. Thalassemia indices and morphology.

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74
Q

Hemoglobin H disease:

A. Genotype.
B. CBC and peripheral smear.
C. Abnormal hemoglobin.

A

A. –/-α or –/αCSα.

B. Thalassemic indices and morphology; Heinz bodies.

C. Hb H = β₄.

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75
Q

Hemoglobin Barts disease:

A. Genotype.
B. Peripheral smear.
C. Abnormal hemoglobin.

A

A. –/–.

B. Hypochromia; nucleated RBCs.

C. Hb Barts = γ₄.

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76
Q

α-Thalassemia: Migration of abnormal hemoglobins.

A

Both Hb H and Hb Barts are fast migrators.

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77
Q

α-Thalassemia: Age at presentation.

A

Birth.

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78
Q

α-Thalassemia: Population at risk for Hb H disease and Hb Barts disease.

A

Asians, because of the relative frequency of the α⁰ haplotype.

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79
Q

How ___-thalassemia affects the concentration of Hb S (or Hb C, etc.).

A. α.
B. β.

A

A. Decrease.

B. Increase.

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80
Q

Hemoglobin F: Normal percentage at different ages (4).

A

Birth: 30-40%.

6 months: Less than 10%.

1 years: Less than 5%.

2 years: Less than 1%.

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81
Q

Hereditary persistence of hemoglobin F: Recognition.

A

Hereditary: Pancellular.

Acquired: Heterocellular.

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82
Q

Combined sickle-cell disease and hereditary persistence of hemoglobin F:

A. Frequency.
B. Percentage of Hb F.
C. Clinical features.

A

A. About 1 in 100 with sickle-cell disease.

B. About 25%.

C. No anemia, no vaso-occlusive episodes.

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83
Q

Combined sickle-cell disease and hereditary persistence of hemoglobin F vs. combined sickle-cell disease and β-thalassemia.

A

On electrophoresis, both diseases show a mixture of Hb S, Hb F, and Hb A₂.

Combined sickle-cell disease and β-thalassemia is clinically more severe.

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84
Q

Acquired causes of elevated hemoglobin F (6).

A

Anemias: Megaloblastic, aplastic, Fanconi’s.

Leukemias: JMML, acute erythrocytic.

Paroxysmal nocturnal hemoglobinuria.

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85
Q

Warm autoimmune hemolytic anemia:

A. Specificity of antibody.
B. Detection.

A

A. Broad reactivity with Rh antigens.

B. Positive DAT with anti-IgG or polyspecific reagent; all cell react in the AHG phase.

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86
Q

Warm autoimmune hemolytic anemia:

A. Location of hemolysis.
B. Peripheral smear.

A

A. Extravascular.

B. Spherocytes.

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87
Q

Warm autoimmune hemolytic anemia: Causes (5).

A

Idiopathic.

Thymoma.

Collagen-vascular diseases.

Hematological malignancy (esp. CLL/SLL).

Inherited immunodeficiencies.

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88
Q

Cold-agglutinin disease:

A. Isotype of antibody.
B. Detection.

A

A. IgM.

B. Positive DAT with anti-C3; cells react in the IS and AHG phases.

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89
Q

Cold-agglutinin disease: Specificities of the antibody.

A

Anti-I, anti-i, anti-H, anti-IH, anti-Pr.

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90
Q

Cold-agglutinin disease: Causes.

A

Anti-I: Mycoplasma pneumoniae, lymphoma.

Anti-i: Infectious mononucleosis.

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91
Q

Cold-agglutinin disease: How to determine the specificity of the antibody.

A

Reaction with (type O) cord blood but not with adult blood: Anti-i.

Reaction with adult blood but not with cord blood: Anti-I.

Reaction with type O blood only: Anti-H.

Reaction with type O and type A₂ blood only: Anti-IH.

Reaction with all types of blood only: Anti-Pr.

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92
Q

Cold agglutinins: Other tests for specificity of the antibody.

A

Anti-H and anti-IH are neutralized by saliva.

Pr antigen is destroyed by enzymes.

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93
Q

Cold agglutinins: Characteristics of benign ones (2).

A

Reactivity at 4-22⁰C (best at 4⁰C).

Titer at 4⁰C is less than 1 : 64.

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94
Q

Cold agglutinins: Characteristics of pathologic ones (3).

A

Broad thermal amplitude.

Titer at 4⁰C is more than 1 : 1000.

Spontaneous agglutination at room temperature.

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95
Q

Cold agglutinins: Characteristics of idiopathic ones (3).

A

Chronic.

Monoclonal IgM.

Often affects elderly patients.

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96
Q

Paroxysmal cold hemoglobinuria: Clinical associations (2).

A

Classic: Adult with syphilis.

Modern: Child with viral infection or otitis media.

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97
Q

Paroxysmal cold hemoglobinuria:

A. Peripheral smear.
B. Treatment.

A

A. Neutrophils with ingested red cells (rare).

B. Keep the patient warm and transfuse warmed blood as needed.

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98
Q

Paroxysmal cold hemoglobinuria:

A. Antibody.
B. Detection of the antibody.

A

A. Cold-reacting IgG anti-P.

B. Blood kept at 4⁰C for 30 minutes and then at 37⁰C for 30 minutes undergoes hemolysis.

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99
Q

Cryoglobulins: How to isolate them.

A

Let drawn blood clot at 37⁰C; centrifuge it at 37⁰C.

Chill the serum at 4⁰C for 3 days; centrifuge it at 4⁰C.

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100
Q

Cryoglobulinemia:

A. Histology.
B. Peripheral smear.

A

A. Vasculitis.

B. Cloudy, pale purple aggregates of protein.

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101
Q

Paroxysmal nocturnal hemoglobinuria:

A. Gene and chromosome.
B. Mutant protein.
C. LAP score.

A

A. PIG-A on the X chromosome.

B. GPI (glycosyl phosphatidylinositol) anchors.

C. Decreased.

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102
Q

Paroxysmal nocturnal hemoglobinuria: Secondarily affected proteins (5).

A

CD59 (MIRL).

CD55 (DAF).

CD16, CD48.

Acetylcholinesterase.

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103
Q

Paroxysmal nocturnal hemoglobinuria: Cause of the hemoglobinuria.

A

Loss of protection against complement-mediated destruction.

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104
Q

Paroxysmal nocturnal hemoglobinuria: Clinical consequences (6).

A

Early: Chronic hemolytic anemia.

Later: Thrombocytopenia, leukopenia.

Possible: Aplastic anemia, AML, thrombosis.

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105
Q

Paroxysmal nocturnal hemoglobinuria: Relation to aplastic anemia.

A

Either disease can lead to the other.

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106
Q

Paroxysmal nocturnal hemoglobinuria: Diagnostic tests.

A

Insensitive: Sucrose lysis, acidified serum (Ham’s).

Preferred: Flow cytometry.

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107
Q

Paroxysmal nocturnal hemoglobinuria: Principle of diagnosis by flow cytometry.

A

FLAER (fluorescent aerolysin derived from Aeromonas hydrophila) binds specifically to GPI anchors.

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108
Q

Paroxysmal nocturnal hemoglobinuria: Typical flow-cytometric plots for

A. Neutrophils.
B. Monocytes.
C. Erythrocytes.

A

A. CD24 vs. FLAER.

B. CD14 vs. FLAER.

C. CD135 vs. CD59 or CD55.

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109
Q

Paroxysmal nocturnal hemoglobinuria: Flow-cytometry classification of red blood cells.

A

Type I: Normal expression of CD59 (or CD55).

Type II: Partial expression.

Type III: No expression.

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110
Q

Paroxysmal nocturnal hemoglobinuria: Use of flow cytometry to predict morbidity.

A

Hemolysis and thrombosis are likely if

− More than 20% of red blood cells are of type III and/or
− More than 50% of neutrophils are abnormal.

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111
Q

Iron-deficiency anemia:

A. Total iron-binding capacity.
B. Iron saturation.
C. Zinc protoporphyrin.

A

A,C. Increased.

B. Decreased.

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112
Q

Causes of increased soluble transferrin receptors in the serum (4).

A

Iron-deficiency anemia.

Hemolytic anemia.
Hemorrhage.
Polycythemia.

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113
Q

Causes of increased zinc protoporphyrin and free erythrocyte protoporphyrin (3).

A

Iron-deficiency anemia.

Lead toxicity.

Anemia of chronic disease.

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114
Q

Folate: Purpose.

A

Cofactor in the transfer of methyl groups, e.g. to dUMP to form dTMP for the synthesis of DNA.

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115
Q

Folate: Absorption.

A

Occurs in the proximal small intestine.

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116
Q

B₁₂: Purpose.

A

Essential in the conversion of methylfolate to active tetrahydrofolate.

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117
Q

B₁₂: Absorption.

A

Stomach: Carried by R factor.

Duodenum: Freed from R factor and bound to gastric-derived intrinsic factor.

Ileum: Absorbed by the enterocytes and bound to transcobalamins I and II for export into the bloodstream.

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118
Q

How can pancreatic insufficiency cause megaloblastic anemia?

A

Release of B₁₂ from R factor depends on pancreatic enzymes.

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119
Q

Drugs that can cause megaloblastic anemia.

A

Methotrexate: Folate deficiency.

Phenytoin: B₁₂ deficiency.

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120
Q

Folate deficiency: Laboratory findings (5).

A

Increased LDH.

Increased unconjugated bilirubin.

Increased forminoglutamic acid.

Decreased serum folate.

Decreased red-cell folate.

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121
Q

B₁₂ deficiency: Laboratory findings (6).

A

Increased LDH.

Increased unconjugated bilirubin.

Increased urinary methylmalonic acid.

Decreased red-cell folate (in ⅔ of cases).

Normal serum folate.

Decreased serum B₁₂.

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122
Q

Serum B₁₂: Causes of spurious abnormalities.

A

Falsely low serum B₁₂: HIV infection.

Falsely high: Liver disease, renal insufficiency, myeloproliferative neoplasms.

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123
Q

B₁₂ deficiency: Other laboratory findings (2).

A

Even mild B₁₂ deficiency causes increased

− Serum methylmalonic acid.
− Serum homocysteine.

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124
Q

Assay for antibodies to intrinsic factor.

A

Sensitive and specific for pernicious anemia.

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125
Q

Anemia of chronic disease:

A. Serum iron.
B. Total iron-binding capacity.
C. Percent saturation of transferrin.
D. Soluble transferrin receptors.

A

A. Low or normal.

B. Low or normal (?)

C. Greater than 15%.

D. Normal.

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126
Q

Sideroblastic anemia: Peripheral smear (4).

A

Hypochromia.

Microcytosis, normocytosis, or macrocytosis.

Biphasic population of red cells.

Pappenheimer bodies.

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127
Q

Sideroblastic anemia: Bone marrow (3).

A

Erythroid hyperplasia.

Increased iron stores.

Ringed sideroblasts.

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128
Q

Sideroblastic anemia: Laboratory findings (3).

A

Increased

− Serum iron.
− Ferritin.
− Percent saturation of transferrin.

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129
Q

Acquired sideroblastic anemia: Causes (5).

A

Myelodysplasia.

Irradiation.

Copper deficiency.

Alcohol abuse.

Drugs: isoniazid, chloramphenicol, chemotherapy.

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130
Q

Inherited sideroblastic anemia:

A. Most common gene and its location.
B. Treatment.

A

A. ALAS2 on the X chromosome.

B. Large doses of pyridoxine (B₆).

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131
Q

Pearson’s syndrome:

A. Clinical manifestations.
B. Mutation.

A

A. Sideroblastic anemia, pancreatic insufficiency.

B. Microdeletion in mitochondrial DNA.

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132
Q

Congenital dyserythropoietic anemia, type II:

A. Synonym.
B. Inheritance.

A

A. Hereditary erythroid multinucleation with positive acidified serum.

B. Autosomal recessive.

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133
Q

Congenital dyserythropoietic anemia, type II: Morphology.

A

Dysplastic erythroid precursors with frequent internuclear bridges.

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134
Q

Congenital dyserythropoietic anemias: Antigen.

A

Overexpression of the i antigen.

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135
Q

Fanconi’s anemia:

A. Inheritance.
B. Hematological features (3).

A

A. Autosomal recessive.

B. Aplastic anemia, MDS, AML (esp. monocytic or monoblastic),

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136
Q

Fanconi’s anemia: Harbingers of pancytopenia.

A

Macrocytic anemia.

Thrombocytopenia.

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137
Q

Fanconi’s anemia: Other manifestations (6).

A

Short stature.

Café-au-lait spots.

Renal abnormalities.

Elevated hemoglobin F.

Absent thumbs or radii.

Microcephaly.

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138
Q

Fanconi’s anemia: Genes.

A

FANCA.

FANCC.

FANCG.

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139
Q

Fanconi’s anemia: Epidemiology.

A

Highest incidence is in white South Africans.

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140
Q

Fanconi’s anemia: Diagnosis.

A

Expose cultured cells to DNA-cross-linking agents (e.g. mitomycin C, cisplatin, diepoxybutane) and look for chromosomal breakage.

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141
Q

Diamond-Blackfan syndrome:

A. Synonym.
B. Bone marrow.
C. Other abnormalities of red cells.

A

A. Congenital pure red-cell aplasia.

B. Few or no erythroid precursors.

C. Increased expression of i antigen, increased Hb F, increased adenosine deaminase.

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142
Q

Diamond-Blackfan syndrome:

A. Inheritance.
B. Gene and its location.

A

A. Autosomal dominant.

B. DBA1 (RPS19) on 19q13.2.

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143
Q

β-Thalassemia: Hemoglobins in heterozygotes.

A

Hb A: Decreased.

Hb F: Increased.

Hb A₂: Increased unless there is also iron deficiency.

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144
Q

Diamond-Blackfan syndrome: Non-hematological abnormalities (3).

A

Short stature.

Anomalies of thumbs or radii.

Cardiac septal defects.

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145
Q

Diamond-Blackfan syndrome: Treatment.

A

Corticosteroids help 75% of children.

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146
Q

Acquired pure red-cell aplasia: Causes (5).

A

Thymoma.

Collagen-vascular diseases.

Drugs.

Parvovirus B19.

Large-granular-lymphocytic leukemias.

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147
Q

Transient erythrocytopenia of childhood: Clinical features.

A

Self-limited.

Affects previously healthy children aged 1 to 4 years.

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148
Q

Congenital amegakaryocytic thrombocytopenia:

A. Bone marrow.
B. Course.

A

A. No megakaryocytes.

B. Thrombocytopenia progressing to pancytopenia by 10 years of age.

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149
Q

Congenital amegakaryocytic thrombocytopenia:

A. Inheritance.
B. Gene and its location.

A

A. Autosomal recessive.

B. MPL (thrombopoietin receptor) on 1p34.

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150
Q

Kostmann’s syndrome:

A. Course.
B. Inheritance.
C. Gene and its location.

A

A. Neutropenia, possibly manifesting as omphalitis, progressing to pancytopenia or to leukemia.

B. Autosomal dominant.

C. ELA2 (neutrophil elastase) on 19p13.

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151
Q

Cyclic neutropenia: Synonym.

A

Benign familial neutropenia.

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152
Q

Cyclic neutropenia:

A. Clinical presentation.
B. Neutrophil count.
C. Affected gene and its location.

A

A. Fever, ulcers, and other inflammations during periods of neutropenia.

B. Ranges from nil to normal over about 21 days.

C. ELA2 on 19p13.

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153
Q

Dyskeratosis congenita: Clinical features (6).

A

Aplastic anemia.

Reticulated skin pigmentation.

Nail dystrophy.

Oral leukoplakia.

Lacrimal-duct atresia.

Testicular atrophy.

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154
Q

Dyskeratosis congenita: Gene and its location.

A

DKC1 on Xq28.

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155
Q

Shwachman-Diamond syndrome: Clinical features (5).

A

Aplastic anemia.

Pancreatic exocrine insufficiency.

Short stature.

Severe combined immunodeficiency.

Reticular dysgenesis.

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156
Q

Shwachman-Diamond syndrome:

A. Inheritance.
B. Gene and its location.

A

A. Autosomal recessive.

B. SBDS on 7q11.

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157
Q

Percentage of cases of plastic anemia caused by ___.

A. drugs or toxins
B. viral hepatitis
C. unknown agent

A

A. 10%.

B. 5%.

C. 70%.

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158
Q

Aplastic anemia: Mimics (5).

A

Hairy-cell leukemia.

Hypoplastic MDS.

Hypoplastic AML.

Paroxysmal nocturnal hemoglobinuria.

Leukemia of T-cytotoxic LGLs.

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159
Q

Intravascular hemolysis: Causes (6).

A

Complement.

Oxidative stress.

Microangiopathic hemolytic anemia.

Mechanical destruction.

Infections.

Toxins.

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160
Q

Extravascular hemolysis: Causes.

A

Anything not included among the causes of intravascular hemolysis.

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161
Q

Intravascular hemolysis: Laboratory findings (5).

A

Schistocytes.

Increased LDH.

Decreased haptoglobin.

Increased free hemoglobin.

Hemosiderinuria.

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162
Q

Extravascular hemolysis: Laboratory findings (5).

A

Microspherocytes.

Increased LDH.

Increased unconjugated bilirubin.

Normal or decreased haptoglobin.

Increased urinary urobilinogen.

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163
Q

Acanthocyte:

A. Synonym.
B. Morphology.
C. Associations (3).

A

A. Spur cell.

B. Projections have blunt, bulbous ends and are unevenly distributed.

C. Liver disease, abetalipoproteinemia, McLeod phenotype.

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164
Q

Basophilic stippling: Composition.

A

Clumps of ribosomes (RNA).

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165
Q

Basophilic stippling: Associations (7).

A

Thalassemia.
Hemolytic anemia.
Arsenic toxicity.
Lead toxicity.

Megaloblastic anemia.
Alcohol abuse.
5’-Nucleotidase deficiency.

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166
Q

Teardrop cells: Clinical associations (3).

A

Myelophthisis.

Megaloblastic anemia.

Thalassemia.

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167
Q

Echinocyte:

A. Synonym.
B. Morphology.

A

A. Burr cell.

B. Projections are sharp and evenly distributed.

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168
Q

Echinocyte: Associations (5).

A

Gastric ulcer.

Uremia.

Pyruvate kinase deficiency.

Splenectomy.

Artifact.

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169
Q

Elliptocyte: Morphology.

A

Red cell is twice as long as it is wide.

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170
Q

Elliptocyte: Associations (4).

A

Deficiency of folate or of B₁₂.

Iron deficiency.

Myelodysplasia.

Elliptocytosis, hereditary.

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171
Q

Howell-Jolly body: Composition.

A

DNA (nuclear remnant).

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172
Q

Cabot ring:

A. Morphology.
B. Association.

A

A. Pink or red-purple ring or figure-of-eight.

B. Disorders of erythropoiesis.

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173
Q

Macrocytosis: Associations.

A

Round macrocytosis: Liver disease, hypothyroidism, myelodysplasia.

Oval macrocytosis: Deficiency of folate or of B₁₂.

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174
Q

Pappenheimer bodies:

A. Morphology.
B. Composition.
C. Associations (3).

A

A. Larger and more irregular than basophilic stippling.

B. Iron-containing mitochondria.

C. Sideroblastic anemia, asplenia, myelodysplasia.

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175
Q

Rouleaux: Causes.

A

Monoclonal protein.

Marked hyperfibrinogenemia.

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176
Q

Schistocytes in healthy adults.

A. Frequency.
B. As a percentage of RBCs.

A

A. 58%.

B. 0.05%.

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177
Q

Stomatocyte: Associations (5).

A

Stomatocytosis, hereditary.

Alcohol abuse.

Rh-null phenotype.

Phenytoin.

Artifact.

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178
Q

Target cells: Associations (3).

A

Thalassemia.

Abnormal hemoglobins such as Hb C, Hb E.

Liver disease.

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179
Q

Workup of anemia: First step.

A

Determine whether the anemia is

− Hyperregenerative (high reticulocyte count).
− Hyporegenerative (low or normal reticulocyte count).

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180
Q

Hyperregenerative normocytic anemia: Differential diagnosis.

A

Hemorrhage vs. hemolysis.

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181
Q

Hyporegenerative normocytic anemia: Workup.

A

Step 1. Is there bi- or pancytopenia?
− Yes: MDS vs. marrow infiltration vs. aplastic anemia.
− No: Step 2.

Step 2. Is the cause anemia of chronic disease; renal failure; early deficiency of Fe, folate, or B₁₂; drugs?
− No: MDS vs. pure red-cell aplasia vs. PNH.

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182
Q

Hyperregenerative microcytic anemia: Workup.

A

Step 1. Are there spherocytes?
− Yes: Step 2.
− No: Inherited defect in membrane or enzyme.

Step 2: Is the DAT positive?
− Yes: Immune-mediated hemolytic anemia.
− No: Hereditary spherocytosis.

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183
Q

Hyporegenerative microcytic anemia: Workup.

A

Step 1: Obtain iron studies (serum iron, ferritin, percent saturation of transferrin).
− Low: Iron-deficiency anemia.
− Normal: Step 2.

Step 2: Is hemoglobin electrophoresis abnormal (e.g. Hb C, Hb E, thalassemia)?
− No: Anemia of chronic disease or sideroblastic anemia.

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184
Q

Hyperregenerative macrocytic anemia: Differential diagnosis.

A

Hemorrhage vs. hemolysis.

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185
Q

Hyporegenerative macrocytic anemia: Workup

A

Step 1. Is the MCV greater than 115, or are there hyperlobate neutrophils?
− Yes: Step 2.
− No: Step 3.

Step 2. Studies of B₁₂ and folate.
− Low: Deficiency of B₁₂ and folate.
− Normal: Step 3.

Step 3. Is the cause hypothyroidism, liver disease, alcohol abuse, drugs (MTX, thioguanines, antiretrovirals), early deficiency of B₁₂ and folate, or Down’s syndrome?
− No: Probable MDS.

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186
Q

Paraneoplastic erythrocytosis:

A. Solid neoplasms that cause it (4).
B. Mechanism.

A

A. HCC, RCC, epithelioid hemangioblastoma, uterine leiomyoma.

B. Secretion of erythropoietin.

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187
Q

Reactive neutrophilia: Usual upper limit.

A

30,000 per μL.

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188
Q

Reactive neutrophilia: Pharmacological causes (3).

A

Corticosteroids.

GM-CSF.

Epinephrine.

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189
Q

Reactive neutrophilia: Physiologic causes.

A

Exercise.

Pregnancy.

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190
Q

Reactive lymphocytosis: Leading causes.

A

Viral infection.

Toxoplasmosis.

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191
Q

Transient stress lymphocytosis:

A. Proliferating cells.
B. Morphology of predominant cell type.

A

A. B cells, T cells, NK cells.

B. Small, eccentric, indented nucleus; cytoplasm may contain granules.

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192
Q

Persistent polyclonal B lymphocytosis:

A. Typical patient.
B. Typical HLA type.

A

A. Young female smoker.

B. DR7.

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193
Q

Persistent polyclonal B lymphocytosis:

A. Morphology.
B. Additional laboratory finding.

A

A. Small, eccentric, indented or bilobate nucleus; much pale cytoplasm.

B. Polyclonal hypergammaglobulinemia.

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194
Q

Reider cells:

A. Morphology.
B. Association.

A

A. Mature small lymphocytes with cleft nuclei.

B. Reactive lymphocytosis in children (classically associated with pertussis).

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195
Q

Age at which absolute lymphocytosis must be considered suspicious for neoplasm.

A

40 years and older.

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196
Q

Reactive monocytosis: Causes (4).

A

Chronic infections (e.g. tuberculosis, listeriosis).

Collagen-vascular diseases.

Recovery from neutropenia.

Solid tumors.

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197
Q

Leading cause of reactive eosinophilia:

A. In the United States.
B. Worldwide.

A

A. Allergy.

B. Helminths.

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198
Q

Paraneoplastic reactive eosinophilia: Causes (3).

A

Hodgkin’s lymphoma.

T-cell neoplasms.

Colonic carcinoma.

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199
Q

Reactive eosinophilia: Systemic inflammatory causes.

A

Eosinophilic cellulitis (Wells’ syndrome).

Eosinophilic fasciitis (Shulman’s syndrome).

Eosinophilic pneumonia (Löffler’s syndrome).

Eosinophilic vasculitis (Churg-Strauss syndrome).

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200
Q

Reactive eosinophilia: Other causes (2).

A

Collagen-vascular diseases.

Inflammatory bowel disease.

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201
Q

Neutropenia:

A. Mechanisms (3).
B. Leading cause.

A

A. Increased destruction, decreased production, splenic sequestration.

B. Drugs.

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202
Q

Neutropenia: Causative drugs (9).

A

Methimazole.
Carbimazole.
Propylthiouracil.

Penicillins.
Chloramphenicol.
Sulfasalazine.

Carbamazepine.
Valproic acid.

Procainamide.

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203
Q

Autoimmune neutropenia:

A. Causes (3).
B. Syndrome.

A

A. SLE or rheumatoid arthritis in adults; usually idiopathic in children.

B. Felty’s syndrome: Rheumatoid arthritis, neutropenia, splenomegaly.

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204
Q

Neutropenia: Infectious causes (5).

A

Typhoid fever.
Tularemia.

Brucellosis.
Rickettsial infections.

Overwhelming sepsis in infants and in the elderly.

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205
Q

Neutropenia: Causes of decreased production (3).

A

Drugs.

Inherited neutropenias.

Leukemias of large granular lymphocytes.

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206
Q

Lymphopenia:

A. Autoimmune cause.
B. Infectious causes.

A

A. Systemic lupus erythematosus.

B. HIV, SARS.

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207
Q

Lymphopenia: Causative drugs.

A

Steroids.

Rituximab.

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208
Q

Lymphopenia: Congenital causes (3).

A

Bruton’s X-linked agammaglobulinemia.

Combined variable immunodeficiency.

DiGeorge’s syndrome.

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209
Q

Deficiency of which type of lymphocyte is most likely to affect the total lymphocyte count?

A

The T lymphocyte.

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210
Q

Monocytopenia: Causes (3).

A

Hairy-cell leukemia.

Steroids.

Onset of neutropenia.

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211
Q

Pseudothrombocytopenia:

A. Cause.
B. Frequency.
C. Resolution.

A

A. EDTA.

B. 1% of hospitalized patients.

C. Collect a new sample in citrate or in acid-citrate-dextrose.

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212
Q

Thrombocytopenia: Causes of large or variably sized platelets (3).

A

May-Hegglin anomaly.

Bernard-Soulier syndrome.

ITP.

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213
Q

Thrombocytopenia: Meaning of schistocytes.

A

Microangiopathic hemolytic anemia: TTP, HUS, DIC, or HELLP.

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214
Q

Thrombocytopenia: Inherited causes with giant platelets (6).

A

May-Hegglin anomaly and other MYH9 syndromes.

Bernard-Soulier syndrome.

Gray-platelet syndrome.

DiGeorge’s syndrome.

Microthrombocytopenias (Mediterranean and X-linked).

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215
Q

Thrombocytopenia: Inherited causes with small platelets.

A

Wiscott-Aldrich syndrome.

Congenital amegakaryocytic thrombocytopenia.

Thrombocytopenia−absent radii syndrome.

Glanzmann’s thrombasthenia.

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216
Q

Thrombocytopenia: Causes in neonates (5).

A

Aneuploidies: +13, +18, +21, -X.

Maternal ITP.

Inherited causes.

Neonatal alloimmune thrombocytopenia.

TORCH infections.

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217
Q

Thrombocytopenia in children:

A. Leading cause and how to recognize it.
B. How to recognize a different cause.

A

A. ITP responds rapidly to steroids.

B. Inherited thrombocytopenias respond poorly to steroids but well to platelet transfusions.

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218
Q

Thrombocytopenia: Main causes in adults.

A

Drugs.

ITP.

Splenic sequestration.

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219
Q

Thrombocytopenia: Causative drugs (6).

A

Antibiotics.
Antiarrhythmics.
Alcohol.
Abciximab.

Heparin.
Thiazides.

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220
Q

Thrombocytopenia: How quinidine may cause it.

A

Induction of antibodies against GP IX.

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221
Q

Thrombocytopenia: Targets of antibodies in ITP (4).

A

GP IIIa.

GP IIb.

GP Ib.

GP V.

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222
Q

Thrombocytopenia: Principles of diagnosis of ITP (3).

A

Usually a diagnosis of exclusion.

Best test is trial of immunomodulation.

Tests for anti-platelet antibodies are nonspecific and usually not readily available.

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223
Q

Thrombocytopenia: ___ causes in adults.

A. Infectious
B. Neoplastic
C. Autoimmune

A

A. HIV, HCV, H. pylori.

B. MDS, B-cell neoplasms.

C. Antiphospholipid syndrome.

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224
Q

Thrombotic thrombocytopenic purpura: Pentad.

A

Fever.

Thrombocytopenia.

Microangiopathic hemolytic anemia.

Neurological dysfunction.

Renal dysfunction.

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225
Q

Thrombotic thrombocytopenic purpura:

A. Peripheral smear.
B. Laboratory finding.

A

A. Many schistocytes.

B. Very high serum LDH.

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226
Q

Thrombotic thrombocytopenic purpura: Causes of sporadic form (4).

A

Idiopathic.

Pregnancy.

Ticlopidine.

Antibodies to ADAMTS-13.

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227
Q

Thrombotic thrombocytopenic purpura: Cause of familial form.

A

Inherited deficiency of ADAMTS-13.

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228
Q

Thrombotic thrombocytopenic purpura: Treatment.

A

Daily plasmapheresis with FFP.

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229
Q

Thrombocytopenia: Workup.

A

Peripheral smear: Platelet clumps, schistocytes, leukemic cells, variably (ITP) or abnormally sized platelets.

Laboratory studies: Coagulation, renal function.

History: Radiation, chemotherapy, drugs.

Physical examination: Spleen, signs of inherited syndromes.

Cause unknown, even after examination of bone marrow: Peripheral destruction, collagen-vascular diseases, ITP.

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230
Q

Thrombocytosis: Causes in children (4).

A

Iron deficiency.

Infections.

Kawasaki’s syndrome.

ALL.

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231
Q

Thrombocytosis: Causes in adults (6).

A

Systemic infection.
Iron deficiency.

Splenectomy.

Malignancy.
CML.
ET.

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232
Q

Thrombocytosis: Likelihood of myeloproliferative neoplasm at different platelet counts.

A

600,000: 70%.

1,000,000: 80%.

2,000,000: 95%.

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233
Q

Lymphomas of B cells that are ___ likely to involve the bone marrow.

A. most (3)
B. least (3)
C. moderately

A

A. Mantle-cell lymphoma, follicular lymphoma, DLBCL.

B. Marginal-zone lymphoma, Burkitt’s lymphoma.

C. Lymphoplasmacytic lymphoma.

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234
Q

Typical pattern of infiltration of marrow by ___.

A. follicular lymphoma
B. diffuse large B-cell lymphoma
C. CLL/SLL

A

A. Paratrabecular.

B. Diffuse.

C. Non-paratrabecular in any pattern.

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235
Q

Typical pattern of infiltration of marrow by ___.

A. mantle-cell lymphoma
B. lymphoplasmacytic lymphoma

A

A. Nodular.

B. Interstitial.

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236
Q

Typical pattern of infiltration of marrow by ___.

A. marginal-zone lymphoma
B. Burkitt’s lymphoma

A

A. Nodular.

B. Diffuse.

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237
Q

Lymphomas of B cells that are ___ likely to involve the peripheral blood.

A. most (2)
B. least
C. moderately (2)

A

A. Mantle-cell lymphoma, Burkitt’s lymphoma.

B. Lymphoplasmacytic lymphoma.

C. Follicular lymphoma, DLBCL.

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238
Q

Lymphomas of B cells: Relation of grade to patient’s age.

A

High-grade lymphomas in younger patients.

Low-grade lymphomas in older patients.

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239
Q

Lymphomas of B cells that are more common in females (3).

A

Primary mediastinal lymphoma.

Follicular lymphoma.

Extranodal marginal-zone lymphoma.

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240
Q

Lymphomas of B cells that is most to run in families.

A

CLL/SLL.

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241
Q

Relation of CLL/SLL to ___.

A. autoimmunity.
B. immunodeficiency

A

A. 30% of patients have a positive DAT.

B. 30-50% of patients have hypogammaglobulinemia.

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242
Q

Prolymphocyte counts in leukemias of B cells.

A

11-55%: PLL/CLL.

Less: CLL.

More: Prolymphocytic leukemia.

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243
Q

CLL: Significance of ___.

A. smudge cells
B. lymphocyte count

A

A. Seen in EDTA but not in heparin.

B. Less than 5000/μL: Monoclonal B-cell lymphocytosis.

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244
Q

CLL/SLL: The dim markers (3).

A

CD20.

sIg.

CD11c (weak and variable).

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245
Q

CLL/SLL and FISH:

A. Percentage of cases with normal result.
B. Most common abnormality.
C. Other abnormalities.

A

A. 20%.

B. del(13q14): More than 50%.

C. +12, -11q, -14q, -17p.

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246
Q

CLL/SLL: Transformations (3).

A

Most common: CLL/PLL or PLL.

Others: DLBCL, classic Hodgkin’s lymphoma.

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247
Q

CLL/SLL:

A. Favorable karyotypes (2).
B. Unfavorable karyotypes (2).

A

A. Normal karyotype or isolated -13q.

B. -11q or -17p.

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248
Q

CLL/SLL:

A. Unfavorable status of immunoglobulin gene.
B. Normal lymphocyte corresponding to this status.
C. How to test for this status.

A

A. Unmutated IgVH.

B. The pregerminal-center B cell.

C. Expression of ZAP70 or of CD38 by more than 30% of cells.

249
Q

CLL/SLL: Other prognostic factors (5).

A

“B” symptoms.

High stage.

Initial lymphocyte count exceeds 30,000.

Doubling of lymphocyte count within 1 year.

Diffuse infiltration of bone marrow.

250
Q

CLL/SLL: Original Rai stages.

A

0: Lymphocytosis greater than 5000.

I: Lymphadenopathy.

II: Hepatosplenomegaly.

III: Hemoglobin less than 11 g/dL.

IV: Platelets fewer than 100,000.

251
Q

SLL/CLL: Modified Rai stages, including survival.

A

Low risk (0): More than 13 years.

Intermediate risk (I and II): 8 years.

High risk (III and IV): 2 years.

252
Q

SLL/CLL: Lymphoid areas of Binet stages.

A

Each side counts as one group:
− Cervical lymph nodes.
− Axillary lymph nodes.
− Inguinal lymph nodes.

Liver.
Spleen.

253
Q

SLL/CLL: Binet stages, including survival.

A

A (fewer than 3 lymphoid areas): 15 years.

B (more than 3 lymphoid areas): 5 years.

C (Hb less than 11 g/dL or PLT less than 100,000): 3 years.

254
Q

Mantle-cell lymphoma:

A. Frequent extranodal sites (2).
B. Pattern of infiltration.

A

A. Gastrointestinal tract, Waldeyer’s ring.

B. Vaguely nodular or diffuse.

255
Q

Mantle-cell lymphoma: Variants (4).

A

Pleomorphic, blastoid: More aggressive.

Small-cell: Resembles SLL.
Marginal-zone-like: Resembles marginal-zone lymphoma.

256
Q

Mantle-cell lymphoma: The bright markers.

A

CD20.

sIg.

257
Q

Mantle-cell lymphoma: Translocation.

A

t(11;14)(q13;q32) :: CCND1−IGH.

258
Q

Mantle-cell lymphoma: Histologic predictors of poor prognosis.

A

More than 40% of nuclei express Ki67.

More than 10 mitotic figures per hpf.

259
Q

Expression of ___ by mantle-cell lymphoma.

A. CD23.
B. CD11c.

A

A. Dim or absent.

B. Absent.

260
Q

Follicular lymphoma: Traditional grading.

A

6-15 centroblasts per hpf: Grade 2.

Fewer: Grade 1.

More: Grade 3.
− 3a: Some centrocytes.
− 3b: No centrocytes.

261
Q

Follicular lymphoma: Modified grading.

A

Grades 1 and 2 are considered low-grade follicular lymphoma.

262
Q

Follicular lymphoma with diffuse growth:

A. Definition.
B. How diagnosed.

A

A. No residual germinal centers; CD23 and CD21 reveal no follicular dendritic cells.

B. As focal diffuse growth if in the setting of low-grade follicular lymphoma; otherwise, as DLBCL.

263
Q

Follicular lymphoma, intrafollicular variant:

A. Synonym.
B. Definition.
C. Significance.

A

A. Follicular lymphoma in situ.

B. Confined to follicles; open sinuses.

C. Unclear.

264
Q

Follicular lymphoma, isolated cutaneous variant:

A. Prognosis.
B. Immunophenotype.
C. Cytogenetics.

A

A. Excellent.

B. Lacks CD10, bcl-2; expresses bcl-6.

C. No rearrangement of BCL2.

265
Q

Follicular lymphoma, isolated gastrointestinal:

A. Prognosis.
B. Typical site.

A

A. Excellent.

B. Duodenum.

266
Q

Typical grade of follicular lymphoma in ___.

A. children
B. the marrow

A

A. Grade 3.

B. Discordant.

267
Q

Relevance of grade of follicular lymphoma to expression of ___.

A. CD10.
B. Ki67.

A

A. Lost in some follicular lymphomas of higher grade.

B. Low grade: less than 20% of nuclei express it; high grade: more than 20%.

268
Q

Follicular lymphoma: Translocation.

A

t(14;18)(q32;q21) :: IGH−BCL2.

269
Q

Significance of t(14;18) in other lymphomas.

A

No overexpression of bcl-2.

270
Q

Follicular lymphoma: Adverse prognostic factors (7).

A

“B” symptoms.
Infiltration of bone marrow.

High stage.
High LDH.
Advanced age.

Low performance status.
Anemia.

271
Q

Follicular lymphoma: Expression of ___.

A. CD23.
B. CD19.
C. CD38.

A

A,B. Usually absent.

C. Present.

272
Q

Marginal-zone lymphoma: Translocations.

A

t(11;18)(q21;q21) :: API2−MALT1: Stomach, lungs.

t(14;18)(q32;q21) :: IGH−MALT1: Eyes, parotid, skin.

t(3;14)(p14;q32) :: FOXP1−IGH: Eyes, thyroid, skin.

t(1;14)(p22;q32) :: BCL10−IGH: Lung, small intestine.

273
Q

Marginal-zone lymphoma: Expression of CD43.

A

Occurs in about 30% of cases.

274
Q

Disease associated with ___ MALT lymphoma.

A. salivary
B. thyroidal

A

A. Sjögren’s syndrome.

B. Hashimoto’s thyroiditis.

275
Q

Organism associated with ___ MALT lymphoma.

A. gastric
B. ocular
C. small-intestinal (IPSID)
D. cutaneous 
E. splenic
A

A. Helicobacter pylori.

B. Chlamydophila psittaci.

C. Campylobacter jejuni.

D. Borrelia burgdorferi.

E. HCV.

276
Q

Splenic marginal-zone lymphoma:

A. Location in the spleen.
B. Other possible sites.

A

A. White pulp.

B. Splenic hilar lymph nodes; liver.

277
Q

Splenic lymphoma with villous lymphocytes:

A. Origin.
B. Differences from hairy-cell leukemia (3).

A

A. Peripheral expression of splenic marginal-zone lymphoma.

B. Polar villi, visible nucleolus, no expression of annexin A1.

278
Q

Hairy-cell leukemia: Presentation.

A

Splenomegaly.

New monocytopenia, neutropenia, or aplastic anemia.

279
Q

Hairy-cell leukemia: Location of infiltrate in the ___.

A. spleen
B. liver

A

A. Red pulp.

B. Sinusoids.

280
Q

Hairy-cell leukemia:

A. Ultrastructure.
B. Staining for TRAP.
C. Staining for cyclin D1.

A

A. Ribosomal lamellar complexes.

B. Positive; weak staining is nonspecific.

C. Nuclear; does not imply rearrangement of CCND1 (bcl-1).

281
Q

Hairy-cell leukemia: Relatively specific markers (5).

A

Bright: CD25, CD11c.

Others: CD103, annexin A1, DBA.44.

282
Q

Hairy-cell leukemia: CD10.

A

Expressed in about 10% of cases.

283
Q

Lymphomas of mature small B cells: FMC-7.

A

Expressed in MCL, MZL, FL, PLL, HCL.

Not expressed in CLL/SLL.

284
Q

Lymphomas of mature small B cells that can be plasmacytoid (4).

A

CLL/SLL, MCL, MZL.

Lymphoplasmacytic lymphoma is diagnosed when the others have been excluded.

285
Q

Waldenström’s macroglobulinemia: Definition.

A

Lymphoplasmacytic lymphoma with
− Monoclonal IgM.
− Infiltration of the bone marrow.

286
Q

Lymphoplasmacytic lymphoma: Special stain.

A

There may be PAS-positive matter in the nodal sinuses.

287
Q

Heavy-chain disease: Types.

A

α: Most common; associated with IPSID.

γ: Franklin’s disease; found in some cases of LPL.

μ: Found in some cases of CLL/SLL.

288
Q

Diffuse large B-cell lymphoma: Presentation (2).

A

Rapid enlargement of node or other lymphoid tissue.

Bone marrow is involved initially in about 10% of cases.

289
Q

Diffuse large B-cell lymphoma: CD5.

A

Such cases must be distinguished from blastoid mantle-cell lymphoma.

290
Q

Diffuse large B-cell lymphoma: Ki67.

A

Expressed in 60-99% of nuclei.

291
Q

Diffuse large B-cell lymphoma: Translocations.

A

t(14;18)(q32;q21) :: IGH−BCL2: Germinal-center-B-cell-like.

t(3;14)(q27;q32) :: BCL6−IGH: Activated-B-cell-like.

292
Q

Prognostic types of diffuse large B-cell lymphoma:

A. How best to distinguish them.
B. Significance.

A

A. Gene-expression profiling.

B. GBC responds better than ABC to treatment.

293
Q

Prognostic types of diffuse large B-cell lymphoma: Immunohistochemistry.

A

CD10
− Expressed by more than 30% of cells: GBC type.
− Expressed by fewer than 30% of cells: bcl-6.

bcl-6
+: MUM1.
−: ABC type.

MUM1
+: ABC type.
−: GBC type.

294
Q

Primary diffuse large B-cell lymphoma of the CNS:

A. Median age.
B. Radiography.
C. Alternate presentation.

A

A. 60 years.

B. Can mimic glioblastoma multiforme.

C. Intraocular lymphoma.

295
Q

Primary diffuse large B-cell lymphoma of the CNS:

A. Histology.
B. Mutation.

A

A. Infiltrates perivascular spaces.

B. Rearrangement of BCL6; overexpression of bcl-6.

296
Q

T-cell/histiocyte-rich diffuse large B-cell lymphoma:

A. Median age.
B. Immunophenotype of small lymphocytes.

A

A. 40 years.

B. Expression of CD4 or CD8; no CD57 or B-cell markers.

297
Q

T-cell/histiocyte-rich diffuse large B-cell lymphoma: Immunophenotype of tumor cells (2,1,3).

A

Positive: B-cell markers, bcl-6.

Variable: EMA.

Negative: CD15, CD30, EBV.

298
Q

T-cell/histiocyte-rich diffuse large B-cell lymphoma: Pattern of infiltration of marrow.

A

Paratrabecular.

299
Q

Primary mediastinal large B-cell lymphoma: Immunophenotype (2,3).

A

Positive: B-cell markers, CD30.

Negative: CD5, CD10, sIg.

300
Q

Primary mediastinal large B-cell lymphoma: Mutations (3).

A

Mutation of MAL gene.

+9p (JAK2).

No rearrangement of BCL2 or of BCL6.

301
Q

B-cell neoplasms with plasmablastic morphology (5).

A

Plasmablastic lymphoma.

Primary effusion lymphoma.

Post-transplant lymphoproliferative disorder.

ALK-positive DLBCL.

Anaplastic plasmacytoma.

302
Q

Plasmablastic lymphoma:

A. Site.
B. Associated viruses.

A

A. Extranodal, often in the oral cavity.

B. EBV, HIV; HHV8 if arising from multicentric Castleman’s disease.

303
Q

Plasmablastic lymphoma: Immunophenotype (4,3).

A

Positive: CD38, CD138, IRF4/MUM1, cIg.

Negative: CD45, CD20, CD56.

304
Q

Primary-effusion lymphoma:

A. Presentation.
B. Cytology.

A

A. Pleural, pericardial, or peritoneal effusion.

B. Large lymphocytes with plasmablastic, immunoblastic, or anaplastic appearance; cytoplasmic vacuoles.

305
Q

Primary-effusion lymphoma: Associated viruses.

A

HIV, HHV8, EBV.

306
Q

Primary-effusion lymphoma: Immunophenotype (5,3).

A

Positive: CD38, CD138, CD30, CD45, EMA.

Negative: B-cell markers, T-cell markers, NK-cell markers.

307
Q

Primary cutaneous diffuse large B-cell lymphoma, leg type: Typical patient.

A

Elderly woman.

308
Q

Intravascular large B-cell lymphoma: Presentation.

A

Signs and symptoms related to occlusion of vessels by the lymphoma.

Nodal disease is rare.

309
Q

EBV-positive neoplasms of large B cells (6).

A

Plasmablastic lymphoma.

Primary effusion lymphoma.

Lymphomatoid granulomatosis.

DLBCL associated with chronic inflammation.

EBV-positive DLBCL of the elderly.

EBV-positive DLBCL, NOS.

310
Q

Lymphomatoid granulomatosis: Sites (5).

A

Lungs.

Liver.

Kidneys.

Brain.

Upper aerodigestive tract.

311
Q

Lymphomatoid granulomatosis: Associations.

A

EBV.

Immunodeficiency.

312
Q

Lymphomatoid granulomatosis: Histology.

A

Lymphocytes infiltrate vessel walls in a vasculitis-like pattern.

Plasma cells, histiocytes, reactive T cells.

Granulomas are uncommon.

313
Q

Diffuse large B-cell lymphoma associated with chronic inflammation: Association.

A

Longstanding pyothorax.

314
Q

EBV-positive DLBCL ___: Association.

A. of the elderly
B. NOS.

A

A. Age greater than 50 years; typically Asian.

B. Immunodeficiency (usually) or age less than 50 years.

315
Q

ALK-positive large B-cell lymphoma: Associated viruses.

A

None.

316
Q

Most common primary immunodeficiencies associated with lymphoproliferative disorders (5).

A

Wiskott-Aldrich syndrome.

Ataxia-telangiectasia.

X-linked lymphoproliferative disorder.

Combined variable immunodeficiency.

Nijmegen syndrome.

317
Q

Most common lymphoproliferative disorders in primary immunodeficiency (3).

A

DLBCL.

Lymphomatoid granulomatosis.

T-cell neoplasms.

318
Q

Most common lymphomas in HIV infection (5).

A

DLBCL.

Plasmablastic lymphoma.

Primary effusion lymphoma.

Hodgkin’s lymphoma.

Burkitt’s lymphoma.

319
Q

Post-transplant lymphoproliferative disorder:

A. When it occurs.
B. Warning sign.
C. Variant.

A

A. Within a year after transplant.

B. Rise in EBV-DNA.

C. Late-onset: More than 5 years after transplant, no EBV, more aggressive.

320
Q

Post-transplant lymphoproliferative disorder, risk factors:

A. Relative to age.
B. Relative to type of transplant.
C. Relative to EBV status before the transplant.

A

A. More common in children.

B. More common in heart-lung and liver-bowel transplants than in transplants of kidney or bone marrow.

C. More common EBV-negative patients.

321
Q

Post-transplant lymphoproliferative disorder:

A. Origin of neoplastic cells.
B. Site.

A

A. The recipient.

B. The allograft.

322
Q

Burkitt’s lymphoma: Types.

A

Endemic (African): Child with jaw mass; associated with EBV.

Sporadic (Western): Child or young adult with intraabdominal disease; not associated with EBV.

Immunodeficiency-associated: Immunodeficient person with nodal disease.

323
Q

Burkitt’s lymphoma: Phenotype (5,5).

A

Positive: CD20, CD10, bcl-6, c-myc, sIg.

Negative: CD5, CD23, bcl-2, CD34, TdT.

324
Q

Burkitt’s lymphoma: Genetics.

A

t(8;14).

t(2;8).

t(8;22).

325
Q

Most common lineage of ___.

A. Acute lymphoblastic leukemia.
B. Lymphoblastic lymphoma.

A

A. B-cell (about 80%).

B. T-cell (about 80%).

326
Q

B-ALL/LBL, NOS: Immunophenotype (7,2).

A

Positive: CD10, CD19, PAX5, TdT (nuclear), CD34, HLA-DR, CD99.

Negative: CD20, sIg.

327
Q

B-ALL/LBL, NOS:

A. Loss of CD10.
B. Expression of myeloid markers.

A

A. May indicate mutation of MLL.

B. CD13 and/or CD33 is expressed in 30-50% of cases.

328
Q

B-ALL/LBL, NOS: Favorable prognostic factors (5).

A

Female sex.

Low initial lymphocyte count.

Age 2-10 years.

Complete remission at 14 days after induction chemotherapy.

Hyperdiploidy

329
Q

B-ALL/LBL, NOS: Unfavorable prognostic factor.

A

Hypodiploidy.

330
Q

B-ALL/LBL, NOS vs. hematogones (2).

A

Flow cytometry: On plots of CD10, CD20, CD34, TdT, sIg, B-ALL is homogeneous and hematogones show a continuum of expression.

CD34-stained sections of bone marrow: Similar distinction.

331
Q

B-ALL/LBL, NOS: Genetics.

A

By definition: No recurrent abnormalities.

Sometimes: Abnormalities of 6q, 9p, 12p.

332
Q

B-ALL/LBL with recurrent cytogenetic abnormalities:

A. Most common abnormality.
B. Most common breakpoint.
C. Resulting protein.

A

A. t(9;22)(q34;q11.2) :: ABL−BCR.

B. m-bcr (minor).

C. Ph, 190 kD.

333
Q

B-ALL/LBL with t(9;22)(q34;q11.2):

A. Epidemiology.
B. Prognosis.

A

A. More common in adults than in children.

B. Poor.

334
Q

B-ALL/LBL with t(9;22)(q34;q11.2): Immunophenotype (5+2).

A

Positive: CD10, CD19, CD34, TdT, CD25.

Weak: CD13, CD33.

335
Q

B-ALL/LBL with t(v;11q23):

A. Typical “v” chromosome.
B. Affected gene.

A

A. 4.

B. MLL on 11q23.

336
Q

B-ALL/LBL with t(v;11q23):

A. Epidemiology.
B. Prognosis.

A

A. Affects infants.

B. Poor.

337
Q

B-ALL/LBL with t(v;11q23): Immunophenotype (3,1).

A

Positive: CD19, CD15, FLT3.

Negative: CD10.

338
Q

B-ALL/LBL with t(12;21)(p13;q22):

A. Involved genes.
B. Epidemiology.
C. Prognosis.

A

A. ETV6−RUNX1 (TEL−AML1).

B. Occurs in children.

C. Good.

339
Q

B-ALL/LBL with t(12;21)(p13;q22): Immunophenotype (2+2,1).

A

Positive: CD10, CD19.

Weak: CD13, CD33.

Negative: CD9.

340
Q

B-ALL/LBL with hyperdiploidy:

A. Definition.
B. Epidemiology.
C. Prognosis.

A

A. More than 50 chromosomes, esp. with extra copies of 4, 14, 21, X.

B. Occurs in children.

C. Good.

341
Q

B-ALL/LBL with hyperdiploidy: Immunophenotype (4).

A

Positive: CD10, CD19, CD34, TdT.

342
Q

B-ALL/LBL with hypodiploidy:

A. Epidemiology.
B. Prognosis.

A

A. Rare in children and in adults.

B. Poor.

343
Q

B-ALL/LBL with t(1;19)(q23;p13.3):

A. Involved genes.
B. Epidemiology.
C. Prognosis.

A

A. TCF3−PBX1 (E2A−PBX1).

B. Uncommon in children.

C. Responds to intensive chemotherapy.

344
Q

B-ALL/LBL with t(1;19)(q23;p13.3): Immunophenotype (2,1).

A

Positive: CD10, CD19.

Negative: CD34.

345
Q

B-ALL/LBL with t(5;14)(q31;q32):

A. Involved genes.
B. Morphology.

A

A. IL3−IGH.

B. Eosinophilia.

346
Q

B-ALL/LBL with t(5;14)(q31;q32):

A. Epidemiology.
B. Prognosis.

A

A. Rare in children and in adults.

B. Intermediate.

347
Q

B-ALL/LBL with t(5;14)(q31;q32): Immunophenotype (4).

A

Positive: CD10, CD19, CD34, TdT.

348
Q

T-ALL/LBL: Immunophenotype (6,1,1).

A

Positive: CD2, CD3 (cytoplasmic), CD5, CD7, TdT, CD99.

Variable: CD34.

Negative: HLA-DR.

349
Q

T-ALL/LBL:

A. CD4 and CD8.
B. Myeloid antigens.

A

A. May be double-positive or double-negative.

B. Some cases express CD13, CD33.

350
Q

T-ALL/LBL: Mutations.

A

Rearrangement of TCR in nearly all cases.

Rearrangement of IgH in 10-20% of cases.

351
Q

T-ALL/LBL vs. thymoma (3).

A

Thymoma expresses EMA.

On plots of CD4 vs. CD8, or CD45 vs. CD3
− Thymoma shows dispersal.
− T-ALL/LBL shows clusters.

352
Q

Multiple myeloma: Familial trend.

A

First-degree relatives have a three- to fourfold risk of multiple myeloma.

353
Q

Multiple myeloma: Renal complications (4).

A

Myeloma cast nephropathy.

Amyloidosis (mainly λ chains).

Light-chain-deposition disease (mainly κ).

Tubular injury (hypercalcemia, hyperuricemia).

354
Q

Multiple myeloma: Frequency of isotypes.

A

IgG: 55%.
IgA: 22%.
Light-chain only: 18%.

IgD, biclonal, IgE.

355
Q

Multiple myeloma:

A. Most common idiotype.
B. Frequency of nonsecretory type.

A

A. κ.

B. About 5%.

356
Q

Multiple myeloma, symptomatic: Criteria.

A

Monoclonal protein.

Clonal plasma cells in the bone marrow.

Organ impairment (“CRAB”).

357
Q

Multiple myeloma, smoldering: Criteria.

A

Monoclonal protein >3.0 mg/dL.

  • or -

Clonal plasma cells >10%.

358
Q

Multiple myeloma: Immunophenotype (5,6).

A

Positive: CD38, CD138, CD56, κ or λ, PCA1.

Negative: CD45, CD19, CD20, CD21, CD22, sIg.

359
Q

Multiple myeloma: Variable markers (9).

A

Cyclin D1: Associated with t(11;14).

Myeloid markers: CD13, CD15, CD33, CD11b, CD117.

Others: CD30, CD10, EMA.

360
Q

Multiple myeloma:

A. Locus of most common mutation.
B. Most common translocation.

A

A. 14q32.

B. t(11;14)(q13;q32) :: CCND1−IGH.

361
Q

Multiple myeloma: How prognosis depends on karyotype.

A

Best: Normal karyotype or isolated t(11;14).

Intermediate: Isolated del(13q14).

Worst: t(4;14), t(14;16), or del(17p13.1).

362
Q

Multiple myeloma: Other adverse prognostic factors (3).

A

High stage.

High β₂-microglobulin.

High plasma-cell-labeling index.

363
Q

Plasma-cell leukemia:

A. Definition.
B. Presentation (3).

A

A. Circulating plasma cells are more than 2.0 × 10⁹/L or more than 20%.

B. Half of cases occur de novo; abrupt onset, aggressive course.

364
Q

Plasma-cell leukemia:

A. Mutation.
B. Immunophenotypic abnormality.

A

A. −13.

B. Loss of CD56.

365
Q

Solitary osseous plasmacytoma:

A. Sites.
B. Frequency of detectable M protein.
C. Frequency of progression to multiple myeloma.

A

A. Vertebrae, ribs, pelvis.

B. About 50%.

C. About 75% in 10 years.

366
Q

Solitary extraosseous plasmacytoma:

A. Sites.
B. Frequency of detectable M protein.
C. Frequency of progression to multiple myeloma.

A

A. Nasal cavity, oropharynx, larynx.

B. Less than 50%.

C. Less than 50%.

367
Q

Monoclonal gammopathy of unknown significance: Risk of progression (2).

A

Each year: 0.5% to 1%.

After 20 years: 1 in 3.

368
Q

Monoclonal gammopathy of unknown significance: Criteria (4).

A

Monoclonal protein less than 3.0 g/dL.

Clonal plasma cells less than 10%.

No organ impairment.

No B-cell lymphoma.

369
Q

T-cell neoplasms: Four most common.

A

Peripheral T-cell lymphoma, NOS.

Angioimmunoblastic T-cell lymphoma.

Anaplastic large-cell lymphoma.

Adult T-cell leukemia/lymphoma.

370
Q

Peripheral T-cell lymphoma, NOS:

A. Pattern in lymph node.
B. Cytology of tumor cells.
C. Background cells.

A

A. Diffuse; conspicuous high-endothelial venules.

B. Small and large lymphocytes; multilobate nuclei.

C. Plasma cells, histiocytes, eosinophils.

371
Q

Peripheral T-cell lymphoma, NOS: Immunophenotype.

A

Positive: CD4.

Negative: CD8, CD25.

Many pan-T-cell markers may be lost.

372
Q

Angioimmunoblastic T-cell lymphoma:

A. Age group.
B. Associated virus.

A

A. Older adults.

B. EBV.

373
Q

Angioimmunoblastic T-cell lymphoma: Presentation (4).

A 
Abrupt onset of
− "B" symptoms.
− Generalized lymphadenopathy.
− Pruritic rash.
− Pleural effusion.
374
Q

Angioimmunoblastic T-cell lymphoma: Possible immunological abnormalities (5).

A

Polyclonal hypergammaglobulinemia.

Anti-smooth-muscle antibody.

Rheumatoid factor.

Cold agglutinins.

Hemolytic anemia with positive DAT.

375
Q

Angioimmunoblastic T-cell lymphoma: Pattern in lymph node.

A

Diffuse, with obscuring of follicles.

Conspicuous high-endothelial venules.

Tumor cells form clusters.

376
Q

Angioimmunoblastic T-cell lymphoma:

A. Cytology of tumor cells.
B. Background cells.

A

A. Small to medium-sized; pale or clear cytoplasm; minimal atypia.

B. Plasma cells, histiocytes, eosinophils, immunoblasts.

377
Q

Angioimmunoblastic T-cell lymphoma: Immunophenotype (5,1).

A

Positive: CD4; EBV; CXCL13, bcl-6, CD10 (markers of follicular T helper cells).

Negative: CD8.

One or more pan-T-cell markers may be lost.

378
Q

Anaplastic large-cell lymphoma: Location of anaplastic cells.

A

In clusters near blood vessels.

379
Q

Anaplastic large-cell lymphoma: Prognosis.

A

Better if it expresses Alk.

Alk-negative lymphomas have a better prognosis than peripheral T-cell lymphoma, NOS.

380
Q

Anaplastic large-cell lymphoma: Immunophenotype (4,3,2).

A

Positive: CD30 (membranous and Golgi pattern), CD45, clusterin, EMA.

Variable: CD4, CD13, CD33.

Negative: CD15, EBV.

381
Q

Anaplastic large-cell lymphoma: Translocations and staining patterns.

A

t(2;5)(p23;p25) :: ALK−NPM: Cytoplasmic and nuclear.

t(1;2)(q25;p23) :: TPM3−ALK: Cytoplasmic and membranous.

382
Q

Anaplastic large-cell lymphoma: Additional mutation.

A

Rearrangement of TCR.

383
Q

Adult T-cell leukemia/lymphoma: Lifetime risk.

A

About 5% in those infected before 20 years of age.

384
Q

Adult T-cell leukemia/lymphoma: Presentation (8).

A

Lymphadenopathy.
Hepatosplenomegaly.
Jaundice.
Weight loss.

Involvement of CNS or of viscera.

Hypercalcemia.
Lytic bone lesions.

Rash.

385
Q

Adult T-cell leukemia/lymphoma: Immunophenotype (5,2).

A

Positive: CD2, CD3, CD4, CD5, CD25.

Negative: CD7 (usually), CD8.

386
Q

Large-granular-lymphocytic leukemia: Criterion.

A

Large granular lymphocytes are more than 2.0 × 10⁹ for more than 6 months, with no other explanation.

387
Q

Large-granular-lymphocytic leukemia: Frequent concurrent hematologic abnormalities (3).

A

Neutropenia.

Splenomegaly.

Polyclonal hypergammaglobulinemia.

388
Q

Large-granular-lymphocytic leukemia of cytotoxic T cells:

A. Epidemiology.
B. Clinical course.

A

A. More common in males; median age is 60 years.

B. Indolent unless CD56 positive with blastoid cells.

389
Q

Large-granular-lymphocytic leukemia of cytotoxic T cells: Immunophenotype (7,3).

A

Positive: CD2, CD3, CD8, CD16, CD57, granzyme B, granzyme M.

Negative: CD4; CD5 and CD7 may be dim or absent.

390
Q

Large-granular-lymphocytic leukemia of cytotoxic T cells: Mutation.

A

Rearrangement of TCR.

391
Q

Large-granular-lymphocytic leukemia of NK cells: Presentation.

A

Neutropenia.

Anemia.

Jaundice.

Hepatosplenomegaly.

Fever.

392
Q

Large-granular-lymphocytic leukemia of NK cells: Immunophenotype (4,3,2).

A

Positive: CD2, CD3ε, CD16, CD56.

Variable: CD7, CD8, CD57.

Negative: CD4, surface CD3.

393
Q

Large-granular-lymphocytic leukemia of NK cells:

A. Associated virus.
B. Mutation.

A

A. None.

B. No rearrangement of TCR.

394
Q

Aggressive NK-cell leukemia:

A. Epidemiology.
B. Associated virus.

A

A. Occurs in Asians; mean age is 40 years.

B. EBV.

395
Q

Nasal-type NK/T-cell lymphomas:

A. Epidemiology.
B. Pattern in lymph node.

A

A. Occurs in Asians and in natives of Central and South America.

B. Angioinvasive.

396
Q

Nasal-type NK/T-cell lymphoma: Associated virus.

A

EBV.

397
Q

Enteropathy-associated T-cell lymphoma:

A. Antecedent disease.
B. Associated HLA types.

A

A. Ulcerative jejunoileitis (refractory celiac disease) in many cases.

B. DQA10501, DQB0201.

398
Q

Enteropathy-associated T-cell lymphoma: Immunophenotype.

A

Positive: CD30, CD3.

Negative (usually): CD4, CD8.

399
Q

Hepatosplenic T-cell lymphoma, γδ type:

A. Epidemiology.
B. Immunophenotype.
C. Mutation.

A

A. Affects young males.

B. Expresses CD8.

C. i(7q).

400
Q

Hepatosplenic T-cell lymphoma, αβ type: Epidemiology.

A

Affects females; ages vary.

401
Q

Cutaneous T-cell lymphoma:

A. Immunophenotype.
B. When it gets called mycosis fungoides.

A

A. Expresses CD4.

B. When it involves lymph nodes.

402
Q

Nodular lymphocyte-predominant Hodgkin’s lymphoma vs. TCRBCL (3).

A

NLP-HL:
− T cells that express CD3 and CD57 surround the tumor cells.
− Cells in the background are B cells.
− CD21 stain demonstrates network of follicular dendritic cells.

403
Q

Nodular lymphocyte-predominant Hodgkin’s lymphoma: Immunophenotype of tumor cells (6,3).

A

Positive: CD45, CD20, sIg, bcl-6, Oct2, BOB.1.

Negative: CD15, CD30, EBV.

404
Q

Classical Hodgkin’s lymphoma: Immunophenotype of tumor cells (4,3,4).

A

Positive: CD30, fascin, IRF4/MUM1, PAX5.

Variable: CD20, EBV, Oct2.

Negative: CD45, CD79a, BOB.1, EMA.

405
Q

Classical Hodgkin’s lymphoma: Spread.

A

Contiguous (to adjacent lymphoid regions), except in the lymphocyte-depleted subtype.

406
Q

Classical Hodgkin’s lymphoma: Most common sites of presentation.

A

Cervical lymph nodes.

Mediastinum.

407
Q

Classical Hodgkin’s lymphoma in the bone marrow:

A. Overall frequency.
B. Most common subtypes.
C. Definition.

A

A. 10%.

B. Lymphocyte-depleted, HIV-associated.

C. Presence of atypical, mononuclear, CD30-positive tumor cells in the appropriate background.

408
Q

Subtype of classical Hodgkin’s lymphoma most strongly associated with ___.

A. Syncytial histology.
B. EBV.
C. Lacunar cells.
D. Intraabdominal disease.

A

A,C. Nodular sclerosis.

B,D. Mixed cellularity.

409
Q

Blast equivalents counted in acute leukemias (3).

A

Promonocytes:
− Acute monocytic/monoblastic leukemia.
− Acute myelomonocytic leukemia.

Promyelocytes: Acute promyelocytic leukemia.

Erythroblasts: “Pure” acute erythroblastic leukemia.

410
Q

Mutations associated with ___.

A. Polycythemia vera (2).
B. Primary myelofibrosis (3).
C. Essential thrombocythemia (3).

A

A. JAK2: V617F or mutation in exon 12.

B,C. JAK2: V617F; MPL: W151L or W151K.

411
Q

Mutations associated with ___.

A. Some eosinophilic myeloproliferative neoplasms (3).
B. Mastocytosis.

A

A. PDGFRA, PDGRFB, FGFR1.

B. KIT: D816V.

412
Q

Secondary MDS:

A. Epidemiology.
B. Causes.
C. Mutations.

A

A. Can occur in younger patients.

B. Benzene, alkylating agents, radiation, Fanconi’s anemia.

C. Anomaly of 5q or of 7q is associated with alkylating agents.

413
Q

Refractory cytopenia with unilineage dysplasia: Allowance of blasts.

A

Blood: Less than 1%.

Marrow: Less than 5%.

No Auer rods.

414
Q

Refractory cytopenia with unilineage dysplasia: Allowance of ringed sideroblasts.

A

No more than 15%.

415
Q

Refractory anemia with ringed sideroblasts: Allowance of blasts.

A

Blood: Less than 1%.

Marrow: Less than 5%.

No Auer rods.

416
Q

Refractory anemia with ringed sideroblasts: Peripheral smear.

A

May show
− Dimorphic population of red cells.
− Pappenheimer bodies.

417
Q

Refractory cytopenias with multilineage dysplasia: Allowance of blasts.

A

Blood: Less than 1%.

Marrow: Less than 5%.

No Auer rods.

418
Q

Refractory cytopenias with multilineage dysplasia: Allowance of dysplasia.

A

More than 10% in more than 1 cell line.

419
Q

Refractory anemia with excess blasts: Allowances of blasts.

A

RAEB-1
− Blood: Less than 5%.
− Marrow: 5-9%.
− No Auer rods.

RAEB-2
− Blood: 5-19%.
− Marrow: 10-19%.
* or Auer rods *

420
Q

Myelodysplastic syndrome with del(5q): Allowance of blasts.

A

Blood: Less than 1%.

Marrow: Less than 5%.

No Auer rods.

421
Q

Myelodysplastic syndrome with del(5q): Bone marrow.

A

Megakaryocytes with nuclear hypolobation.

422
Q

Ringed sideroblast: Definition.

A

Contains at least 5 siderosomes that follow at least one third of the circumference of the nucleus.

423
Q

Functional indicators of dyserythropoiesis (5).

A

Complement, increased susceptibility to.

Hemoglobin F, elevated.

Enzymatic defects.

Antigens, abnormal expression of.

Thalassemia, acquired.

424
Q

Myelodysplasia: Secondary causes (7).

A

HIV.
Drugs.

Copper deficiency / zinc toxicity.
Arsenic toxicity.
B₁₂ or folate deficiency.
Lead toxicity.
Ethanol.
425
Q

Myelodysplasia: Most common karyotypic abnormalities.

A

Complex karyotype (#1).

−7, −7q.

−5q.

426
Q

Myelodysplasia: Relevance of karyotype to prognosis.

A

Best: Normal karyotype or isolated −5q, −20q, −Y.

Worst: Complex karyotype (at least 3 abnormalities) or anomaly of chromosome 7.

Intermediate: Anything else.

427
Q

Chronic myelomonocytic leukemia: Criteria (4).

A

Persistent monocytosis of more than 1 × 10⁹ per L.

Myelodysplasia (usually granulocytic).

Blasts are less than 20% of nucleated cells in the marrow.

No Ph.

428
Q

Chronic myelomonocytic leukemia: Other frequent findings (3).

A

Hepatosplenomegaly.

Anemia, thrombocytopenia.

Atypical monocytes.

429
Q

Chronic myelomonocytic leukemia: Types.

A

CMML-1: Blasts + promonocytes are less than 5% in the blood and less than 10% in the marrow; no Auer rods.

CMML-2: Blasts + promonocytes are 5-19% in the blood or 10-19% in the marrow.

430
Q

Chronic myelomonocytic leukemia: Mutation.

A

JAK2 is mutated in a few cases.

Eosinophilic CMML: Mutation of PDGFRA or of PDGFRB is not allowed.

431
Q

Atypical CML vs. conventional CML.

A

Atypical CML
− Dysplasia.
− Usually no significant basophilia.
− No rearrangement involving BCR and ABL.

432
Q

Atypical CML: Mutations.

A

+8, −20q in many cases.

JAK2 is mutated in a few cases.

433
Q

JMML:

A. Most common karyotypic abnormality.
B. Frequent comorbidity.

A

A. −7.

B. Neurofibromatosis, type 1.

434
Q

JMML: Confirmatory test.

A

Spontaneous formation in vitro of granulocyte-macrophage colonies that show increased sensitivity to GM-CSF.

435
Q

CML: Translocation.

A

t(9;22)(q34;q11.2) :: ABL−BCR.

436
Q

CML: Breakpoints of the translocation.

A

M-bcr (major breakpoint): p210; usual mutation of CML.

m-bcr (minor breakpoint): p190
− Marked monocytosis.
− Also seen in Ph+ ALL.

μ-bcr: p230; marked thrombocytosis, mature neutrophils.

437
Q

CML: Bone marrow (3).

A

“Dwarf” megakaryocytes.

Mild reticulin fibrosis.

Thickened paratrabecular generative cuffs.

438
Q

CML: Chemical findings (2).

A

Decreased LAP score.

Markedly increased serum B₁₂.

439
Q

CML, accelerated phase: Criteria.

A

Progressive basophilia (more than 20%).

Progressive thrombocytosis (more than 1000) or thrombocytopenia (less than 100).

Increasing blasts (between 10% and 20%).

Clonal cytogenetic progression (+8, +19, +Ph, i(17q)).

440
Q

CML, blast phase: Criteria.

A

Blasts in the marrow (more than 20% or in a large aggregate).

Blasts in the blood (more than 20%).

Myeloid sarcoma.

441
Q

CML, blast phase: Outcomes.

A

AML: 70%.

ALL: 30%.

442
Q

CML: Most powerful predictor of prognosis.

A

Response to tyrosine-kinase inhibitors as determined by quantitative RT-PCR.

443
Q

Resistance to imatinib in CML:

A. Frequency.
B. Mechanisms.

A

A. About 5% initially; increases with time.

B. Mutation in the tyrosine-kinase domain or in the P loop.

444
Q

Polycythemia vera: Major criteria.

A

Hb exceeds 18.5 g/dL in men or 16.5 g/dL in women, or increased red-cell mass.

Mutation in JAK2: V617F or functionally similar.

445
Q

Polycythemia vera: Minor criteria.

A

Panmyelosis.

Endogenous formation of erythroid colonies in vitro.

Normal serum erythropoietin.

446
Q

Polycythemia vera, chronic phase: Peripheral blood.

A

Erythrocytosis.

There may also be neutrophilia, basophilia, and/or thrombocytosis.

447
Q

Polycythemia vera, chronic phase: Bone marrow (3).

A

Hypercellularity.

Marked hyperplasia of megakaryocytes.

Low or absent stainable iron.

448
Q

Polycythemia vera, spent phase (3).

A

Marrow: Reticulin fibrosis.

Blood: Myelophthisis.

Tissues: Extramedullary hematopoiesis.

449
Q

Polycythemia vera: Required number of criteria.

A

Both major criteria and one minor criterion

  • or -

One major criterion and two minor criteria.

450
Q

Polycythemia vera: Main causes of death.

A

Thrombosis.

Acute leukemia.

451
Q

JAK2:

A. Frequency of mutation in myeloproliferative neoplasms.
B. Purpose of protein.

A

A. More than 90% in PV; more than 50% in ET and in PMF.

B. Stimulates the STAT pathway.

452
Q

JAK2: Most common mutations.

A

V617F (G to T at position 1849).

Activating mutation in exon 12.

453
Q

Hereditary spherocytosis:

A. Inheritance.
B. Molecular defect.

A

A. Autosomal dominant.

B. Ankyrin (ANK1 gene).

454
Q

Essential thrombocythemia: Criteria.

A

Sustained thrombocytosis (more than 450).

Megakaryocytic hyperplasia without panmyelosis.

Does not meet criteria of PV, PML, CML, or MDS.

Presence of V617F in JAK2, or exclusion or reactive causes of thrombocytosis.

455
Q

Essential thrombocythemia: Epidemiology.

A

Peaks around 30 years and 60 years.

456
Q

Essential thrombocythemia: Peripheral blood.

A

Isolated thrombocytosis.

457
Q

Essential thrombocythemia: Marrow (4).

A

Megakaryocytes
− Large, hyperlobate nuclei.
− Paratrabecular.
− May exhibit emperipolesis.

Stainable iron is present, unlike in iron-deficiency anemia.

458
Q

Primary myelofibrosis, cellular phase: Peripheral blood (3).

A

Anemia.

Mild leukocytosis.

Thrombocytosis.

459
Q

Primary myelofibrosis, cellular phase: Marrow (3).

A

Hypercellularity.

Megakaryocytes
− Dark, clumped chromatin.
− Next to trabeculae and sinuses.

460
Q

Primary myelofibrosis, fibrotic phase: Peripheral blood.

A

Leukoerythroblastic pattern.

461
Q

Primary myelofibrosis, fibrotic phase: Marrow (3).

A

Reticulin fibrosis.

Hematopoiesis in the sinuses.

Atypical, clumped megakaryocytes.

462
Q

Chronic eosinophilic leukemia: Epidemiology.

A

Nine times more common in males.

Ages 25-45.

463
Q

Chronic eosinophilic leukemia: Number and morphology of eosinophils.

A

More than 1.5 × 10⁹ per L.

Cyanine-resistant MPO stain highlights hypogranular eosinophils.

464
Q

Chronic eosinophilic leukemia: Sites of infiltration of tissues.

A

Heart.

Lungs.

Gastrointestinal tract.

CNS.

465
Q

Chronic eosinophilic leukemia vs. the hypereosinophilic syndrome.

A

Both are diagnoses of exclusion.

Increased blasts and clonal cytogenetic abnormalities occur only in CEL.

466
Q

Genes associated with eosinophilia in hematolymphoid neoplasms.

A

PDGFRA.

PDGFRB.

FGFR1.

467
Q

Rearrangement of PDGFRA:

A. Usual neoplasm.
B. Other neoplasms.

A

A. CEL-like disease.

B. AML with eosinophils, T-ALL with eosinophils.

468
Q

Rearrangement of PDGFRA:

A. Epidemiology.
B. Karyotype.

A

A. 17 times more common in males; median age is 40 years.

B. Normal; rearrangement with NFIP1L1 leads to cryptic deletion of 4q12.

469
Q

Rearrangement of PDGFRB:

A. Usual neoplasm.
B. Other neoplasms.

A

A. CMML-like disease.

B. AML with eosinophils.

470
Q

Rearrangement of PDGFRB:

A. Epidemiology.
B. Karyotype.

A

A. Twice as common in males; median age is 40 years.

B. t(5;12) :: PDFGRB−ETV6.

471
Q

Rearrangement of FGFR1:

A. Usual neoplasm.
B. Other neoplasms.

A

A. T-ALL with eosinophils.

B. CEL-like disease.

472
Q

Rearrangement of FGFR1:

A. Epidemiology.
B. Karyotype.

A

A. 15 times more common in males; median age is 30 years.

B. t(8;13) :: FGFR1−ZNF198.

473
Q

AML: Median age at presentation.

A

65 years.

474
Q

Usual blast count in AML:

A. Percentage.
B. Exceptions (3).

A

A. 20%.

B. Pure erythroleukemia, myeloid sarcoma, recurrent genetic abnormality.

475
Q

AML: Usual phenotype.

A

Positive: CD13, CD33, CD34, HLA-DR.

Weak: CD45.

476
Q

AML: Exceptions to the usual phenotype (2).

A

Promyelocytic leukemia: No expression of CD34 or of HLA-DR.

Expression of CD7 or of CD19 by some leukemias.

477
Q

AML: Major categories (4).

A

AML with recurrent genetic abnormalities.

AML secondary to therapy.

AML with myelodysplasia-related changes.

AML, NOS.

478
Q

AML with t(8;21):

A. Genes.
B. Role of one of the genes.

A

A. t(8;21)(q22;q22) :: RUNX1T1−RUNX1.

B. RUNX1 encodes the α chain of core-binding factor.

479
Q

AML with t(8;21): Morphology.

A

Much gray-blue cytoplasm.

Large azurophilic granules.

Auer rods.

480
Q

AML with t(8;21): Immunophenotype.

A

Usual AML phenotype, but also expresses CD56 and sometimes CD19.

481
Q

AML with t(8;21):

A. Age group.
B. Prognosis.

A

A. Young adults.

B. Favorable.

482
Q

AML with t(8;21): Mutation in many relapsed cases.

A

Activating mutation of KIT.

483
Q

AML with inv(16) or t(16;16): Genes.

A

inv(16)(p13;q22) or t(16;16)(p13;q22) :: MYH11−CBFβ.

484
Q

AML with inv(16) or t(16;16): Properties of eosinophils.

A

Large granules, including basophilic ones.

Take up α-naphthylacetate esterase.

Usually not abundant in the peripheral blood.

485
Q

AML with inv(16) or t(16;16): Immunophenotype.

A

Usual AML phenotype plus expression of CD14, CD64, CD2, CD11b, lysozyme.

486
Q

AML with inv(16) or t(16;16):

A. Age group.
B. Prognosis.

A

A. Young adults.

B. Favorable.

487
Q

AML with t(15;17): Genes.

A

t(15;17)(q22;q12) :: PML−RARA.

488
Q

AML with t(15;17): Morphology.

A

Promyelocytes with bilobate or reniform nuclei.

Intensely granular or apparently agranular (microgranular).

489
Q

AML with t(15;17): Properties of the microgranular variant.

A

Reacts with MPO stain.

Auer rods.

490
Q

AML with t(15;17): Immunophenotype.

A

Positive: CD13, CD33.

Dim: CD15.

Negative: CD34, HLA-DR.

491
Q

AML with t(15;17): Medical emergency.

A

DIC on presentation.

492
Q

Promyelocytic leukemia: Translocations of ATRA-resistant disease.

A

t(11;17) sometimes.

PML with t(5;17) appears to respond to ATRA.

493
Q

Promyelocytic leukemia: Complication of therapy with ATRA.

A

Differentiation (retinoic acid) syndrome.

494
Q

AML with t(15;17):

A. Age group.
B. Prognosis.

A

A. Middle-aged adults.

B. Favorable.

495
Q

AML with t(9;11): Genes.

A

t(9;11)(p22;q23) :: MLLT3−MLL.

496
Q

AML with t(9;11): Morphology.

A

Monocytic.

497
Q

AML with t(9;11): Immunophenotype (5+3).

A

Positive: CD33, CD4, CD64, CD11b, HLA-DR.

Weak: CD13, CD14, CD34.

498
Q

AML with t(9;11):

A. Age group.
B. Prognosis.

A

A. Children.

B. Intermediate.

499
Q

AML with t(6;9): Genes.

A

t(6;9)(p23;q34) :: DEK−NUP214.

500
Q

AML with t(6;9): Morphology (3).

A

May resemble FAB M2 or M4.

Often exhibits basophilia.

Often exhibits multilineage dysplasia.

501
Q

AML with t(6;9): Immunophenotype.

A

Usual immunophenotype of AML.

About half of cases also express TdT.

502
Q

AML with t(6;9):

A. Age group.
B. Prognosis.

A

A. Children and adults.

B. Poor.

503
Q

AML with t(1;22): Genes.

A

t(1;22)(p13;q13) :: RBM15−MKL1.

504
Q

AML with t(1;22): Morphology.

A

Megakaryocytic.

505
Q

AML with t(1;22): Immunophenotype (4,2).

A

Positive: CD13, CD33, CD41, CD61.

Negative: CD34, HLA-DR.

506
Q

AML with t(1;22):

A. Age group.
B. Prognosis.

A

A. Infants.

B. Intermediate.

507
Q

AML with inv(3) or t(3;3): Genes.

A

inv(3)(q21q26) :: RPN1−EVI1.

t(3;3)(q21;q26) :: RPN1−EVI1.

508
Q

AML with inv(3) or t(3;3): Morphology.

A

May resemble FAB M1, M4, or M7.

Thrombocytosis; giant agranular platelets.

509
Q

AML with inv(3) or t(3;3):

A. Age group.
B. Prognosis.

A

A. Adults.

B. Poor.

510
Q

AML related to therapy: Genes.

A

Inhibitors of topoisomerase II may affect
− RUNX1 on 21q22.
− MLL on 11q23.

511
Q

AML related to therapy: Morphology (4).

A

Multilineage dysplasia.

Erythroid hyperplasia.

Ringed sideroblasts.

Basophilia.

512
Q

AML related to therapy: Causes.

A

Inhibitors of topoisomerase II.

Alkylating agents.

Ionizing radiation.

513
Q

AML related to therapy: Immunophenotype.

A

Usual immunophenotype of AML.

May also expresses CD56 and/or CD7.

514
Q

AML related to therapy:

A. Time of onset.
B. Prognosis.

A

A. About 5 years after therapy.

B. Poor.

515
Q

AML with myelodysplasia-related changes: Criteria.

A

Blasts are more than 20% in the marrow.

One of the following:
− History of MDS.
− Cytogenetic abnormality related to MDS.
− Multilineage dysplasia (more than 50% of cells in more than 1 cell line).

No history of cytotoxic chemotherapy.
No recurrent cytogenetic abnormality associated with AML.

516
Q

AML with myelodysplasia-related changes:

A. Age group.
B. Prognosis.

A

A. Elderly patients.

B. Progresses slowly; does not respond to therapy.

517
Q

AML, NOS, with minimal differentiation: Morphology.

A

Undifferentiated blasts without Auer rods and without granules.

Less than 3% of blasts are positive for MPO or Sudan black B.

518
Q

AML, NOS, with minimal differentiation: Immunophenotype.

A

Usual immunophenotype of AML, plus CD117.

About half of cases express TdT.

519
Q

AML, NOS, without maturation: Morphology.

A

Rare Auer rods and granules.

3-10% of blasts are positive for MPO or SBB.

520
Q

AML, NOS, without maturation: Immunophenotype.

A

Usual immunophenotype of AML, plus CD117.

521
Q

AML, NOS, with maturation: Criteria (3).

A

Morphologically similar to AML with t(8;21).

Maturation in more than 10% of blasts.

Less than 20% of nonerythroid cells show monocytic differentiation.

522
Q

AML, NOS, with maturation: Immunophenotype.

A

Positive: Usual immunophenotype of AML, sometimes also with CD15.

523
Q

AML, NOS, myelomonocytic: Criteria (2).

A

At least 20% of nucleated cells show monocytic differentiation.

At least 20% of nucleated cells show granulocytic differentiation.

524
Q

AML, NOS, myelomonocytic: Immunophenotype (2).

A

Expresses myeloid markers and monocytic markers.

525
Q

AML, NOS, monocytic/monoblastic: Criteria (3).

A

At least 80% of nucleated cells show monocytic differentiation.

Acute monoblastic leukemia: Most of the monocytic cells are monoblasts.

Acute monocytic leukemia: Most of the monocytic cells are promonocytes.

526
Q

AML, NOS, monocytic/monoblastic: Immunophenotype (2,1).

A

Positive: Monocytic markers, HLA-DR.

Variable: Myeloid markers.

527
Q

AML, NOS, monocytic/monoblastic:

A. Age group.
B. Tissues subject to infiltration.

A

A. Younger patients.

B. Gingiva, CNS.

528
Q

Erythroleukemia: Criteria (2).

A

At least 50% of nucleated cells are erythroblasts.

At least 20% of non-erythroid cells are myeloblasts.

529
Q

Pure erythroid leukemia: Criteria (2).

A

At least 80% of nucleated cells are erythroblasts.

No excess of myeloblasts.

530
Q

Acute erythroid leukemia: Peripheral smear.

A

Anemia.

Many nucleated red blood cells.

531
Q

Acute erythroid leukemia: Aspirate smear (4).

A

Dysplasia.

Megaloblastoid change.

Sometimes: Vacuoles, globular staining with PAS.

532
Q

Immunophenotype of ___ in acute erythroid leukemia:

A. myeloblasts
B. erythroblasts (4)

A

A. Usual immunophenotype of AML, plus CD117.

B. CD34, HLA-DR, CD235 (glycophorin), CD71 (may be weak).

533
Q

Acute megakaryoblastic leukemia: Morphology.

A

At least 50% of blasts are megakaryoblasts.

Megakaryoblasts exhibit nuclear blebs.

534
Q

Acute megakaryoblastic leukemia:

A. How to demonstrate megakaryocytic lineage.
B. Associations.

A

A. Platelet-peroxidase technique; flow cytometry for CD41, CD61.

B. Mediastinal germ-cell tumors, i(12p).

535
Q

Acute megakaryoblastic leukemia: Immunophenotype (2,2).

A

Positive: CD41, CD61.

Negative: CD34, HLA-DR.

536
Q

AML, NOS: Prognosis.

A

Variable: AML with maturation, acute myelomonocytic leukemia.

Poor: All other types.

537
Q

Acute leukemia in Down’s syndrome: Types.

A

ALL: Similar to typical ALL.

AML.

538
Q

AML in Down’s syndrome:

A. Morphology.
B. Immunophenotype.

A

A. Megakaryoblastic.

B. Expresses CD13 and CD11b but not CD34.

539
Q

AML in Down’s syndrome:

A. Age.
B. Prognosis.
C. Treatment.

A

A. One to five years of age.

B. Relatively favorable.

C. Responds well to methotrexate.

540
Q

Transient myeloproliferative disorder / transient abnormal myelopoiesis:

A. Frequency in Down’s syndrome.
B. Age at onset.
C. Karyotype.

A

A. About 10%.

B. One week or less.

C. +21: May be limited to the neoplastic clone.

541
Q

Transient myeloproliferative disorder / transient abnormal myelopoiesis:

A. Clinical presentation.
B. Course (2).

A

A. Marked leukocytosis, hepatosplenomegaly.

B. Usually resolves without treatment; neonates are at high risk for AML.

542
Q

Transient myeloproliferative disorder / transient abnormal myelopoiesis:

A. Immunophenotype.
B. Mutation.

A

A. Expresses CD34 but not CD13 or CD11b (inverse of AML).

B. Somatic mutation of GATA1 (also occurs in AML).

543
Q

Congenital acute leukemia:

A. Age at presentation.
B. Differential diagnosis and how to resolve it.

A

A. Four weeks or less (by definition).

B. TMD / TAM, leukemoid reaction; flow cytometry, cytogenetics.

544
Q

Congenital acute leukemia: Most common type.

A

About 65% of cases are AML, especially monocytic/monoblastic.

545
Q

Congenital acute leukemia:

A. Presentation.
B. Mutation.

A

A. There may be blue spots on the skin (leukemia cutis).

B. MLL (11q23) in 10% of cases.

546
Q

Mast-cell neoplasms: Chemical findings.

A

Increase in

− Serum tryptase.
− Urinary N-methylhistamine.
− Urinary prostaglandin D2.

547
Q

Mast-cell neoplasms: Immunophenotype (7+1).

A

Positive: CD25, CD2; CD45, CD11b, CD33, CD43, FcεRI.

Weak: CD117.

548
Q

Mast-cell neoplasms: Most common mutation.

A

D816V in KIT: Corresponds with aberrant expression of CD25.

549
Q

Hematopoietic disorders associated with elevated histamine.

A

Mast-cell neoplasms.

The hypereosinophilic syndrome.

550
Q

Hemocytometer: Dimensions of one large square.

A

0.1 cm × 0.1 cm × 0.01 cm deep = 0.1 μL.

551
Q

Manual CBC: Measured indices.

A

RBC (using the hemocytometer).

Hematocrit (using the centrifuge).

552
Q

Manual CBC: Calculated indices.

A

MCV = Hematocrit / RBC × 10.

MCHC = Hemoglobin / hematocrit × 100.

553
Q

Automated CBC: Limitations (3).

A

Relatively low precision in counting of platelets and basophils.

Inability to count band neutrophils.

554
Q

Automated CBC: How hemoglobin is measured.

A

Red blood cells are lysed.

Potassium ferrocyanide and KCN are added to form HiCN.

Absorbance at 540 nm is measured.

555
Q

Automated CBC: Limitations of the hemiglobin cyanide method (3).

A

Cannot detect sulfhemoglobin.

Paraproteins and lipemia cause overestimation of hemoglobin.

556
Q

Automated CBC: How red blood cells, leukocytes, and platelets are counted.

A

Red blood cell: Any particle in the range of 36 fL to 360 fL.

After lysis of red blood cells
− Leukocyte: Any particle greater than 36 fL.
− Platelet: Any particle smaller than 36 fL.

557
Q

Automated CBC: Determination of MCV and RDW.

A

In a Gaussian distribution of sizes of red blood cells,

− MCV is the mean.
− RDW is the coefficient of variation.

558
Q

Automated CBC: Calculated indices.

A

Hematocrit = MCV × RBC / 10.

MCHC = Hemoglobin / hematocrit × 100.

559
Q

MCHC: Causes of increase (3).

A

Spherocytosis.

Cold agglutinins.

Lipemia.

560
Q

Reticulocyte count: Methods.

A

Manual: Light microscopy.

Automated: Optical light scatter, flow cytometry.

561
Q

Reticulocyte count: Dyes.

A

Light microscopy and optical light scatter: Supravital stain (new methylene blue or azure B).

Flow cytometry: RNA-specific fluorochrome.

562
Q

Reticulocyte count: Formulas.

A

Absolute reticulocyte count = % Reticulocytes × RBC.

Corrected reticulocyte count = % Reticulocytes × Hct / 45.

Reticulocyte-production index = Corrected reticulocyte count / maturation index.

563
Q

Reticulocyte count: Maturation index.

A

Hematocrit

= 36-45%: 1.0.
= 26-35%: 1.5.
= 16-25%: 2.0.
15 or less: 2.5.

564
Q

Automated CBC: Plot of leukocytes.

A

Mo Ne
Eo
Ly

– forward scatter –>

565
Q

Hemoglobin-solubility test:

A. Synonym.
B. Principle.
C. Causes of true positive (5).
D. Cause of false negative.

A

A. Dithionate test.

B. Insoluble hemoglobins cause marked turbidity of the lysate.

C. SS, SA, SC, SD, C-Harlem, others.

D. Low concentration of insoluble hemoglobin, e.g. in a neonate.

566
Q

Sickling test:

A. Synonym.
B. Principle.
C. Causes of true positive (5).
D. Cause of false negative.

A

A. Metabisulfite test.

B. Reagent causes sickling, which can be demonstrated microscopically.

C. SS, SA, SC, SD, C-Harlem, others.

D. [Hb S] is less than 10%.

567
Q

Acid-elution technique: Interpretation.

A

Heterocellular pattern: Fetal-maternal hemorrhage or thalassemia.

Pancellular pattern: Hereditary persistence of fetal hemoglobin.

568
Q

Alkali-denaturation technique.

A

Hb F resists denaturation by 1.25 M NaOH.

The optical density of the supernatant reflects the amount of Hb F.

569
Q

Highly accurate method of quantifying Hb F.

A

HPLC.

570
Q

Fast hemoglobins:

A. Definition.
B. Examples.
C. Artifact.

A

A. Those that move faster (i.e. closer to the ‘+’ end) than Hb A on alkaline gel.

B. Hb H, Hb Barts.

C. Bilirubin.

571
Q

Hemoglobin electrophoresis and the thalassemias.

A

α-Thalassemia: Normal Hb A₂.

β-Thalassemia: Elevated Hb A₂ (unless there is also iron deficiency).

572
Q

HPLC for hemoglobin studies: Limitations (3).

A

The following get eluted together:

− Hb A₂ and Hb E.
− Hb C and Hb O-Arab.
− Hb Barts and bilirubin.

573
Q

Pulse oximetry: Principle.

A

LEDs emit light at 2 wavelengths

… 660 nm (red) for deoxyhemoglobin.
… 940 nm (infrared) for oxyhemoglobin.

574
Q

Pulse oximetry: Limitations.

A

Cannot detect sulfhemoglobin, carboxyhemoglobin, or methemoglobin.

Oxygen saturation is overestimated in the presence of these substances.

575
Q

Analyzers of arterial blood gases:

A. Technique.
B. Limitations (3).

A

A. Calculate oxygen saturation based on direct measurement of pH, pO₂, pCO₂.

B. Assume normal curve of oxygen saturation, normal level of 2,3-DPG, normal hemoglobins.

576
Q

Co-oximeter: Technique.

A

Emits many wavelengths to measure oxyhemoglobin, deoxyhemoglobin, carboxyhemoglobin, methemoglobin.

577
Q

Cytochemical stains: Undifferentiated blasts.

A

Negative: MPO, SBB, CAE, NSE, PAS.

578
Q

Cytochemical stains: Myeloblasts.

A

Positive: MPO, SBB, CAE.

579
Q

Cytochemical stains: Promyelocytes.

A

Positive: MPO, SBB, CAE.

580
Q

Cytochemical stains: Monoblasts.

A

Positive: NSE.

NaF inhibits staining for NSE.

581
Q

Cytochemical stains: Erythroblasts.

A

Positive: PAS (diffusely granular).

Variable: MPO, SBB, NSE.

NaF does not affect staining for NSE.

582
Q

Cytochemical stains: Megakaryoblasts.

A

Positive: PAS (diffusely granular).

Variable: NSE.

NaF does not affect staining for NSE.

583
Q

Cytochemical stains: Lymphoblasts.

A

Positive: PAS (blocky, “rosary bead” pattern).

Variable: SBB (faintly gray).

584
Q

Myeloperoxidase stain:

A. Target.
B. Limitation.

A

A. Azurophilic granules.

B. Rapid degradation.

585
Q

Sudan black B stain: Target.

A

Lipids.

586
Q

Chloroacetate esterase stain:

A. Synonym.
B. Cells that take it up.

A

A. Leder’s stain.

B. Granulocytes, mast cells.

587
Q

Nonspecific esterases: Types.

A

α-Naphthylacetate esterase.

α-Naphthylbutyrate esterase.

588
Q

Oil red O stain: Use in neoplastic hematopathology.

A

Stains the cytoplasmic vacuoles of Burkitt’s lymphoma.

589
Q

LAP score:

A. Principle.
B. Computation.

A

A. Leukocyte alkaline phosphate hydrolyzes granules within neutrophils to yield a colored product.

B. The intensity of the reaction is graded 0 to 4+ in each one of 100 neutrophils and bands.

590
Q

LAP score: Normal value.

A

40-120.

591
Q

LAP score: Causes of decrease (5).

A

PNH.

CML.

MDS (some cases).

Congenital hypophosphatasia.

Neonatal sepsis.

592
Q

LAP score: Causes of increase (5).

A

Reactive neutrophilia.

Glucocorticoids.

Polycythemia vera.

Primary myelofibrosis.

Pregnancy (3rd trimester).

593
Q

Cyclin-D1: Positive staining (4).

A

Mantle-cell lymphoma.

Hairy-cell leukemia.

Plasma-cell myeloma.

Many epithelia.

594
Q

bcl-2: Normal positive staining (3).

A

In a reactive lymph node:

− Mantle-zone B cells in the mantle zone.
− Mantle-zone B cells in the follicular centers.
− T cells.

595
Q

bcl-6: Normal expression.

A

B cell of the germinal center.

596
Q

CD1a: Normal expression.

A

Cortical thymocytes.

Langerhans’ cells.

597
Q

CD3:

A. Chains that compose the molecule.
B. Association with the T-cell receptor.

A

A. γ, δ, ε.

B. Tight but not covalent.

598
Q

CD4: Normal expression.

A

Helper T cells.

Monocytes.

Dendritic cells.

599
Q

CD7: Normal expression.

A

T cells.

NK cells.

600
Q

CD10: Normal expression.

A

Cells of the follicular center, whether benign or neoplastic.

601
Q

CD11b and CD11c: Normal expression.

A

Monocytes.

Granulocytes.

602
Q

CD15: Significance in AML.

A

Indicates maturation.

603
Q

CD14 and CD16: Normal expression.

A

CD14: Monocytes.

CD16: NK cells, granulocytes.

604
Q

CD19: Normal expression.

A

B cells.

Normal plasma cells.

605
Q

CD20: Normal expression.

A

B cells.

NOT plasma cells.

606
Q

FMC-7:

A. Definition.
B. Expression in B-cell lymphomas.

A

A. An antibody to an epitope of CD20.

B. Expressed by most B-cell lymphomas, but not by CLL/SLL, which has dim expression of CD20.

607
Q

CD23: Synonym.

A

Low-affinity IgE receptor.

608
Q

CD45: Intensity of expression by normal hematopoietic cells.

A

Weakest: Plasma cells, blasts, erythrocytes.

Intermediate: Granulocytes.

Strongest: Mature lymphocytes, monocytes.

609
Q

CD45RO: Normal expression.

A

T cells.

610
Q

CD71:

A. Synonym.
B. Significance in neoplastic hematopathology.

A

A. Transferrin receptor.

B. Expression correlates with grade of tumor.

611
Q

Clusterin:

A. Normal expression.
B. Abnormal expression.

A

A. Megakaryocytes: Cytoplasmic.

B. Anaplastic large-cell lymphoma: Golgi pattern.

612
Q

HLA-DR: Normal expression.

A

Monocytes.

B cells.

Activated T cells.

613
Q

PAX5: Abnormal expression.

A

Nuclear staining in

− Neoplasms of B cells.
− Classic Hodgkin’s lymphoma.

614
Q

ZAP-70: Normal expression.

A

T cells.

NK cells.

615
Q

How many ___ show clonal rearrangement of TCR?

A. non-Hodgkin’s lymphomas of mature B cells
B. B-ALLs

A

A. 5% to 7%.

B. 20% to 40%.

616
Q

How many ___ show clonal rearrangement of IgH?

A. lymphomas of mature T cells
B. T-ALLs

A

A. 4% to 6%.

B. 10% to 15%.

617
Q

Immunophenotypic evolution of B cells.

A

Lymphoid stem cells: CD34, TdT, HLA-DR; germline IgH, TCR.

Pro-B cell: Gain of CD19, CD10; rearrangement of IgH.

Pre-B-cell: Loss of CD34, TdT; gain of CD20, cμ chains; rearrangement of Ig light chain.

Mature B cell: Gain of CD21, CD22, sIg.

Plasma cell: Loss of most B-cell markers, sIg; retention of cIg.

618
Q

Immunophenotypic evolution of T cells.

A

Lymphoid stem cells: CD34, TdT, HLA-DR; germline IgH, TCR.

Prothymocyte: Loss of HLA-DR; gain of CD2, CD7.

Immature thymocyte: Loss of CD34; gain of CD1a, cCD3, CD4, CD5, CD8; rearrangement of TCR.

Mature thymocyte: Loss of TdT, CD1a; loss of either CD4 or CD8.

Mature T cell: Gain of surface CD3.

CP Reviewer 2 (8 decks)

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