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Hematology/Oncology-First Aid 2017 > Pathology > Flashcards

Pathology Flashcards

1
Q

What kind of cells are seen with liver disease and states of cholesterol dysregulation?

A

Acanthocytes or spur cells

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2
Q

What is seen in RBCs with lead poisoning, in sideroblastic anemias and myelodysplastic syndromes?

A

Basophilic stippling

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3
Q

What causes basophilic stippling seen on peripheral blood smears?

A

Aggregation of residual ribosomes

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4
Q

What type of RBCs are seen in bone marrow infiltration like myelofibrosis?

A

Tear drop cells or dacrocyte

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5
Q

What type of RBCs are seen in G6PD deficiency?

A

Bite cells or degmacyte

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6
Q

What type of RBCs are seen in end-stage renal disease, liver disease, or pyruvate kinase deficiency?

A

Burr cells or echinocytes

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7
Q

What type of RBCs are seen in hereditary ellptocytosis?

A

Elliptocytes

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8
Q

What causes elliptocytes to form?

A

Spectrin mutation (membrane protein)

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9
Q

What type of RBCs are seen in megaloblastic anemia?

A

Macro-ovalocyte

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10
Q

What type of neutrophils are seen in megaloblastic anemia?

A

Hypersegmented PMNs

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11
Q

What is seen on RBCs in sideroblastic anemia?

A

Ringed sideroblasts

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12
Q

What causes ringed sideroblasts seen in bone marrow?

A

Excess iron in mitochondria

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13
Q

What type of RBCs are seen in microangiopathic hemolytic anemias, including DIC, TTP/HUS, HELLP syndrome, and mechanical hemolysis?

A

Schistocytes

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14
Q

Fragmented RBCs and helmet cells are referred to what?

A

Schistocytes

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15
Q

What type of RBCs are seen in sickle cell anemia?

A

Sickle cells

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16
Q

What type of RBCs are seen in hereditary spherocytosis, drug- and infection-induced hemolytic anemia?

A

Spherocytes

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17
Q

What type of RBCs are seen in HbC disease, asplenia, liver disease, ant thalassemias?

A

Target cells

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18
Q

In what are Heinz bodies seen in RBCs?

A

G6PD deficiency

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19
Q

What causes Heinz bodies in G6PD deficiency?

A

Precipitation of Hb from oxidation of Hb-SH to S-S

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20
Q

In what disease are Howell-Jolly bodies seen?

A

Functional hyposplenia or asplenia

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21
Q

What type of anemia will have decreased iron, increased TIBC, decreased ferritin and increased free erythrocyte protoporphyrin?

A

Iron deficiency anemia

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22
Q

Iron deficiency anemia lab findings

A

Increased TIBC, protoporphyrin

Decreased iron, ferritin

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23
Q

What phenomenon is seen on RBCs in iron deficiency anemia?

A

Central pallor

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24
Q

Fatigue, conjunctival pallor, pica, and koilonychia is seen in which type of anemia?

A

Iron deficiency anemia

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25
Q

Triad of iron deficiency anemia, esophageal webs, and dysphagia seen in iron deficiency anemia?

A

Plumber-Vinson syndrome

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26
Q

Glossitis, cheilosis and Plumber-Vinson syndrome are common manifestations in what type of anemia?

A

Iron deficiency anemia

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27
Q

A cis deletion in a-thalassemia occurs more commonly in which race?

A

Asian populations

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28
Q

A trans deletion in a-thalassemia occurs more commonly in which race?

A

African populations

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29
Q

How many allele deletions are seen in a-thalassemia with no a-chains?

A

4 allele deletion

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30
Q

How many allele deletions are seen in a-thalassemia with HbH disease and very little a-chain?

A

3 allele deletion

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31
Q

How many allele deletions are seen in a-thalassemia with less clinically severe anemia?

A

2 allele deletion

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32
Q

How many allele deletions are seen in a-thalassemia with no anemia and is clinically silent?

A

1 allele deletion

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33
Q

What is a complication of a 4 allele deletion in a-thalassemia in a developing fetus?

A

Hydrops fetalis

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34
Q

What globulin type is produced in excess in a-thalassemia with a 4 allele deletion?

A

y-globulin (forms y4: Hb Barts)

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35
Q

What is a complication of Hb Barts in developing fetus?

A

Hydrops fetalis

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36
Q

What globulin type is produced in excess in a-thalassemia with a 3 allele deletion?

A

B-globin (forms HbH)

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37
Q

Which type of thalassemia is prevalent in Mediterranean populations?

A

B-thalassemia

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38
Q

What causes B-thalassemia?

A

Point mutations in splice sites and promoter sequences

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39
Q

An HbA2 > 3.5% on electrophoresis is diagnostic of which type of B-thalassemia?

A

B-thalassemia minor

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40
Q

Individual with chipmunk facies with extramedullary hematopoiesis with hepatosplenomegaly has which type of B-thalassemia?

A

B-thalassemia major

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41
Q

What risk is increased in B-thalassemia major?

A

Parvovirus B19 induced aplastic crisis

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42
Q

When does B-thalassemia major become symptomatic in a newborn

A

After 6 months (HbF is protective)

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43
Q

Which B-thalassemia has underproduction of B chain and usually asymptomatic?

A

B-thalassemia minor

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44
Q

Which B-thalassemia has absent B chains with severe microcytic, hypochromic anemia with target cells and increased anisopoikilocytosis?

A

B-thalassemia major

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45
Q

Which type of B-thalassemia presents with mild to moderate sickle cell disease?

A

HbS/B-thalassemia heterozygote

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46
Q

Which B-thalassemia is a heterozygote, major or minor?

A

Minor

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47
Q

Which B-thalassemia is a homozygote, major or minor?

A

Major

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48
Q

What enzymes does lead inhibit that cause decreased heme synthesis and increased RBC protoporphyrin

A

Ferrochelatase and ALA dehydrogenase

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49
Q

Which RNA molecule is inhibited by lead?

A

rRNA (cause of basophilic stippling)

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50
Q

Where are lead lines seen in lead poisoning?

A

Gingivae and metaphyses of long bones

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51
Q

What is used for chelation in kids?

A

Succimmer

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52
Q

What are first line treatments for lead poisoning?

A

Dimercaprol and EDTA

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53
Q

What are genetic causes for sideroblastic anemia?

A

X-linked defect in ALA synthase gene

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54
Q

What are acquired causes for sideroblastic anemia?

A

Myelodysplastic syndromes

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55
Q

What is the most common cause of reversible causes for sideroblastic anemia?

A

Alcohol

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56
Q

What are reversible causes for sideroblastic anemia?

A

Alcohol, lead, vitamin B6 deficiency, copper deficiency and isoniazid

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57
Q

Lab findings in sideroblastic anemia

A

Increased iron, ferritin

Normal to low TIBC

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58
Q

What is the treatment for sideroblastic anemia?

A

Pyridoxine

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59
Q

Which B vitamin is a cofactor for ALA synthase?

A

Pyridoxine (B6)

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60
Q

Cancer drug that causes megaloblastic anemia and folate deficiency

A

Methotrexate

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61
Q

What enzyme is inhibited by methotrexate that causes megaloblastic anemia?

A

Dihydrofolate reductase

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62
Q

What are findings seen in folate deficiency?

A

Increased homocysteine
Normal methylmalonic acid
No neurological symptoms
Glossitis

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63
Q

Where is folate absorbed in the body?

A

Jejunum

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64
Q

What is the source for folate?

A

Green veggies and fruits

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65
Q

What fish parasite can cause vitamin B12 deficiency?

A

Diphyllobothrium latum

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66
Q

What are findings seen in vitamin B12 deficiency?

A

Increased homocysteine and methylmalonic acid

Neurological symptoms

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67
Q

What neurological tracts are affected in vitamin B12 deficiency?

A

Spinocerebellar tract, lateral corticospinal tract, and dorsal column dysfunction

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68
Q

Where is vitamin B12 stored in the body?

A

Liver

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69
Q

Which depletes first, folate or B12?

A

Folate

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70
Q

How is vitamin B12 deficiency diagnosed?

A

Schilling test (malabsorption vs diet)

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71
Q

What is a source for B12?

A

Meats and eggs

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72
Q

Where in the body is vitamin B12 absorbed?

A

Ileum

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73
Q

What is needed for proper absorption of vitamin B12?

A

Intrinsic factor

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74
Q

What are the most common causes of extravascular hemolysis?

A

Hereditary spherocytosis and autoimmune hemolytic anemia

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75
Q

What are common findings in extravascular hemolysis?

A

Urobilinogen in urine
No hemoglobinuria or hemosiderinuria
Spherocytes in blood smear
Decreased haptoglobin

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76
Q

What are common lab findings in intravascular hemolysis?

A

Hemosiderinuria, hemoglobinuria, urobilinogen in urine
Increased unconjugated bilirubin (maybe)
Schistocytes in blood smear

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77
Q

What are common causes of intravascular hemolysis?

A

Mechanical hemolysis
Paroxysmal nocturnal hemoglobinuria
Microangiotpathic hemolytic anemias

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78
Q

Produced by liver and binds ferroportin on intestinal mucosal cells and macrophages inhibiting iron transport

A

Hepcidin

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79
Q

Findings in anemia of chronic disease

A

Decreased iron

Increased TIBC, ferritin

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80
Q

Viral agents that can cause aplastic anemia?

A

Parvovirus B19, EBV, HIV, hepatitis virus

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81
Q

Common causes of aplastic anemia

A

Radiation and drugs, viruses, Fanconi anemia, and idiopathic

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82
Q

Common lab findings in aplastic anemia

A

Decreased reticulocyte count

Increased EPO

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83
Q

What immunosuppressive agents can be used for aplastic anemia?

A

Antithymocyte globulin and cyclosporine

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84
Q

Autosomal dominant disorder causing extravascular hemolysis due to a defect in proteins interacting with RBC membrane skeleton and plasma membrane

A

Hereditary spherocytosis

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85
Q

What are the membrane proteins that interact with RBC membrane skeleton?

A

Ankyrin, band 3, protein 4.2 and Spectrin

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86
Q

What do RBCs look like in hereditary spherocytosis?

A

Small, round RBCs with no central pallor

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87
Q

What is the MCHC in hereditary spherocytosis?

A

Increased

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88
Q

What is the treatment for hereditary spherocytosis?

A

Splenectomy

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89
Q

What lab findings are seen in hereditary spherocytosis?

A

Positive osmotic fragility test

Normal to decreased MCV

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90
Q

Common infection that is seen in hereditary spherocytosis

A

Parvovirus B19 causing aplastic crisis

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91
Q

What organ in enlarged in hereditary spherocytosis?

A

Spleen

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92
Q

Most common enzymatic disorder of RBCs

A

G6PD deficiency

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93
Q

What is the inheritance pattern of hereditary spherocytosis?

A

Autosomal dominant

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94
Q

Presents with back pain, hemoglobinuria a few days after oxidant stress with Heinz bodies on blood smear

A

G6PD deficiency

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95
Q

Autosomal recessive disease that causes hemolytic anemia in newborn and presents with increased levels o f 2,3 BPG

A

Pyruvate kinase deficiency

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96
Q

What ATP levels are seen in pyruvate kinase deficiency?

A

Decreased

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97
Q

Defect in GPI anchor for decay-accelerating factor causes hemolysis of RBC at night due to complement-mediated lysis and red-pink urine in the morning due to hemoglobinuria

A

Paroxysmal nocturnal hemoglobinuria

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98
Q

What are lab findings in Paroxysmal nocturnal hemoglobinuria?

A

CD55/59 negative RBCs on flow cytometry

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99
Q

What is a common triad in Paroxysmal nocturnal hemoglobinuria?

A

Coombs negative hemolytic anemia
Pancytopenia from aplastic crisis
Venous thrombosis = main cause of death

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100
Q

What is the treatment for Paroxysmal nocturnal hemoglobinuria?

A

Eculizumab

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101
Q

What is the target of eculizumab?

A

Terminal complement inhibitor

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102
Q

What population with HbS is usually asymptomatic?

A

Newborns

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103
Q

Autosplenectomized patients are at increased risk of what type of organisms?

A

Encapsulated organisms

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104
Q

Which bacteria is common in patients with autosplenectomy due to sickle cell anemia?

A

S pneumoniae

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105
Q

What is a common bacterial cause of osteomyelitis in sickle cell anemia?

A

Salmonella osteomyelitis

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106
Q

Common cause of death of adults with sickle cell anemia

A

Acute chest syndrome

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107
Q

How is sickle cell anemia diagnosed?

A

With gel electrophoresis

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108
Q

What is the treatment for sickle cell anemia?

A

Hydroxyurea, hydration, and folic acid supplementation

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109
Q

What is aplastic crisis in sickle cell anemia caused by?

A

Parvovirus B19

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110
Q

What is the mutation in HbC disease?

A

Glutamic acid to lysine in B-globin

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111
Q

What is seen on blood smear of HbC patient?

A

Crystals inside RBCs, target cells

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112
Q

Dactylitis, acute chest syndrome, priapism, avascular necrosis, and stroke are considered what in sickle cell disease?

A

Painful crisis (vaso-occlusive)

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113
Q

Autoimmune hemolytic anemia seen in SLE and CLL or drugs like a-methyldopa are associated with what type of immunoglobulins?

A

IgG

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114
Q

Is IgG autoimmune hemolytic anemia considered warm or cold?

A

Warm

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115
Q

Autoimmune hemolytic anemia seen in CLL, Mycoplasma pneumoniae infections and infectious Mononucleosis are associated with what type of immunoglobulins?

A

IgM

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116
Q

Is IgM autoimmune hemolytic anemia considered warm or cold?

A

Cold

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117
Q

RBCs that are damaged when passing through obstructed or narrowed vessel lumen as seen in DIC, TTP/HUS, SLE, HELLP syndrome and malignant HTN causes what type of hemolytic anemia?

A

Microangiopathic hemolytic anemia

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118
Q

RBCs damaged by prosthetic heart valves and aortic stenosis cause what type of hemolytic anemia?

A

Macroangiopathic hemolytic anemia

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119
Q

What type of RBC findings are seen in micro and macroangiopathic hemolytic anemia?

A

Schistocytes (helmet cells)

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120
Q

What type of infections also cause hemolytic anemia?

A

Malaria and Babesia infections

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121
Q

What are lab values in iron deficiency anemia?

A

Serum iron: decreased
Transferrin/TIBC: increased
Ferritin: decreased
% transferrin saturation: markedly decreased

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122
Q

What are lab values in anemia of chronic disease?

A

Serum iron: decreased
Transferrin/TIBC: decreased
Ferritin: increased
% transferrin saturation: no change

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123
Q

What are lab values in hemochromatosis?

A

Serum iron: increased
Transferrin/TIBC: decreased
Ferritin: increased
% transferrin saturation: markedly increased

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124
Q

What are lab values in pregnancy/OCP use?

A

Serum iron: no change
Transferrin/TIBC: increased
Ferritin: no change
% transferrin saturation: decreased

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125
Q

Which protein transports iron in blood?

A

Transferrin

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126
Q

Which protein is primary storage for iron?

A

Ferritin

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127
Q

What is used to indirectly measure transferrin value?

A

TIBC

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128
Q

Which receptors are decreased in immature neutrophils?

A

CD16 and Fc receptors

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129
Q

Increased neutrophil precursors like band cells and metamyelocytes is what kind of shift?

A

Left shift

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130
Q

What are the group of hereditary or acquired conditions of defective heme synthesis that lead to the accumulation of heme precursors called?

A

Porphyrias

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131
Q

What substance accumulates with ferrochelatase and ALA dehydratase inhibition or defect?

A

Protoporphyrin and ALA

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132
Q

What conditions can inhibit ferrochelatase and ALA synthase?

A

Lead poisoning

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133
Q

Common cause of lead poisoning in adults

A

Environmental exposure

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134
Q

Common symptoms of lead poisoning in adults

A

Headache, memory loss, demyelination

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135
Q

Common cause of lead poisoning in children

A

Exposure to lead paint

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136
Q

Common symptoms of lead poisoning in children

A

Mental deterioration

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137
Q

Autosomal dominant mutation that causes a deficiency in porphobilinogen deaminase leads to an accumulation of which heme precursors?

A

Porphobilinogen and ALA

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138
Q

Autosomal dominant mutation in which there is increased porphobilinogen and ALA heme precursors causing painful abdomen, port-wine colored urine, polyneuropathy and psychological disturbances

A

Acute intermittent porphyria

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139
Q

Blistering cutaneous photosensitivity and hyperpigmentation exacerbated by alcohol and has tea-colored urine - what is the heme precursor that accumulates?

A

Uroporphyrin

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140
Q

What is the deficient enzyme in porphyria cutanea tarda?

A

Uroporphyrinogen decarboxylase

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141
Q

What is the inheritance pattern of porphyria cutanea tarda?

A

Autosomal dominant

142
Q

What is the rate-limiting enzyme in the heme synthesis pathway?

A

ALA synthase

143
Q

How does increased heme affect ALA synthase?

A

Decreases activity

144
Q

How does decreased heme affect ALA synthase?

A

Increases activity

145
Q

X-lined sideroblastic anemia has a defect in which heme synthesis enzyme?

A

ALA-synthase

146
Q

What enzymes are inhibited in lead poisoning?

A

Ferrochelatase and ALA dehydratase

147
Q

Inhibition of ferrochelatase leads to an increase in which heme precursor?

A

Protoporphyrin

148
Q

Inhibition of ALA dehydratase leads to an increase in which heme precursor?

A

ALA

149
Q

How does glucose affect heme synthesis?

A

Decreases ALA synthase activity

150
Q

What is the mechanism cell death in iron poisoning?

A

Peroxidation of membrane lipids

151
Q

What is the treatment for iron poisoning?

A

Chelation and dialysis

152
Q

Which chelators are used in iron poisoning?

A

IV deferoxamine or oral deferasirox

153
Q

What is the most common test used to follow patients on warfarin?

A

INR

154
Q

What lab test looks at function of the common and extrinsic pathway?

A

PT

155
Q

What factors are analyzed in PT test?

A

I, II, V, X, VII

156
Q

What lab test looks at function of the common and intrinsic pathway?

A

PTT

157
Q

What factors are analyzed in PTT test?

A

All factors except VII and XIII

158
Q

How are coagulation disorders diagnosed?

A

With mixing study

159
Q

A mixing study will have what results with clotting factor deficiencies?

A

Correction of PT and PTT

160
Q

A mixing study will have what results with acquired inhibitors?

A

No correction

161
Q

Hemophilia A will increase which coagulation test, PT or PTT or both?

A

Increased PTT

162
Q

Hemophilia B will increase which coagulation test, PT or PTT or both?

A

Increased PTT

163
Q

Hemophilia B will increase which coagulation test, PT or PTT or both?

A

Increased PTT

164
Q

Christmas disease is a defect in which coagulation factor?

A

Factor IX

165
Q

Hemophilia B is also called what?

A

Christmas disease

166
Q

What is the treatment for hemophilia?

A

Missing factor plus desmopressin

167
Q

Liver disease will lead to a deficiency of all coagulation factors except which?

A

Factor VIII

168
Q

What does a defect in platelet plug formation cause?

A

Increased bleeding time (BT)

169
Q

Coagulation disorders, like hemophilia, lead to what type of hemorrhage?

A

Macrohemorrhage

170
Q

Platelet disorders, like Bernard-Soulier, lead to what type of hemorrhage?

A

Microhemorrhage

171
Q

Bleeding into joints, easy bruising, or bleeding after trauma or surgery are examples of macrohemorrhage seen in what disorders?

A

Coagulation disorders like hemophilia

172
Q

Bleeding into mucus membranes, epistaxis, petechiae and purpura are examples of microhemorrhage seen in what disorders?

A

Platelet disorders like Bernard-Soulier syndrome

173
Q

What fails to adhere in Bernard-Soulier syndrome?

A

Platelet-to-vWF adhesion

174
Q

What is defective in Bernard-Soulier syndrome?

A

GpIb

175
Q

Defect in platelet plug formation caused by decreased GpIb

A

Bernard-Soulier syndrome

176
Q

What type of platelets are seen in Bernard-Soulier syndrome?

A

Large platelets

177
Q

What fails to adhere in Glanzmann thrombasthenia?

A

Platelet-to-platelet adhesion

178
Q

What is seen on blood smear in Glanzmann thrombasthenia?

A

No platelet clumping

179
Q

What is defective in Glanzmann thrombasthenia?

A

GpIIb/IIIa integrin

180
Q

What is increased in vitamin K deficiency, PT, PTT or both?

A

PT and PTT are both increased

181
Q

What is the bleeding time (BT) in vitamin K deficiency?

A

BT is normal

182
Q

What disease is characterized by thrombocytopenia, microangiopathic hemolytic anemia, and acute renal failure?

A

Hemolytic uremic syndrome (HUS)

183
Q

Common cause of HUS in children

A

EHEC E coli O157:H7

184
Q

What is an additional finding in children affected with HUS that is not seen in adults?

A

Bloody diarrhea

185
Q

What is the PT/PTT in HUS?

A

Normal PT/PTT

186
Q

What type of RBCs are seen in HUS?

A

Schistocytes

187
Q

What is the most common cause of thrombocytopenia in kids and adults?

A

Immune thrombocytopenia

188
Q

What is the cause of immune thrombocytopenia

A

Anti-GpIIb/IIIa antibodies

189
Q

What is common cause of immune thrombocytopenia in kids?

A

Post-viral infection

190
Q

What is common cause of immune thrombocytopenia in adults?

A

Women mainly 2ndry to lupus

191
Q

What is the mechanism of immune thrombocytopenia?

A

Anti-GpIIb/IIIa antibodies cause splenic macrophage consumption of platelet-antibody complex

192
Q

What are common lab findings in immune thrombocytopenia?

A

Increased megakaryocytes on bone marrow bx

193
Q

What is the treatment for immune thrombocytopenia?

A

Steroids, IVIG

194
Q

When is splenectomy required for immune thrombocytopenia?

A

In refractory cases

195
Q

Presents with neurologic and renal symptoms, fever, thrombocytopenia, and microscopic hemolytic anemia with schistocytes on blood smear in a female.

A

Thrombotic thrombocytopenic purpura (TTP)

196
Q

What is the cause of TTP?

A

ADAMTS-13 deficiency

197
Q

What are common lab findings in TTP?

A

Schistocytes, increased LDH, and normal coagulation parameters

198
Q

What is the treatment for TTP?

A

Plasmapheresis and steroids

199
Q

What coagulation defect is seen in von Willebrand disease?

A

Increased PTT and BT

200
Q

What coagulation factor is deficient in von Willebrand disease?

A

Factor VIII

201
Q

What fails to adhere in von Willebrand disease?

A

Platelet-to-vWF adhesion

202
Q

What is the inheritance pattern in von Willebrand disease?

A

Autosomal dominant

203
Q

Most common inherited bleeding disorder?

A

von Willebrand disease

204
Q

What is the treatment for von Willebrand disease?

A

Desmopressin

205
Q

How does desmopressin treat von Willebrand disease?

A

Releases vWF stored in endothelium

206
Q

Most common cause of inherited hypercoagulability inherited by Caucasians

A

Factor V Leiden

207
Q

G to A DNA point mutation causing an Arg506Gln mutation near the cleavage site

A

Factor V Leiden

208
Q

Inherited mutation that causes recurrent pregnancy losses

A

Factor V Leiden

209
Q

Increases risk of thrombotic skin necrosis with hemorrhage after administration of warfarin due to decreased ability to inactivate factors Va and VIIIa

A

Protein C and S deficiency

210
Q

Mutation in 3’ untranslated region leading to increased production of prothrombin causing increased plasma levels and venous clots

A

Prothrombin gene mutation

211
Q

What type of blood products are indicated for acute blood loss or severe anemia?

A

Packed RBCs

212
Q

What type of blood products are indicated for stopping significant bleeding?

A

Platelets

213
Q

What type of blood products are indicated for DIC, cirrhosis, or immediate anticoagulation reversal?

A

Fresh frozen plasma/prothrombin complex concentrate

214
Q

What type of blood products are indicated for coagulation factor deficiencies involving fibrinogen and factor VIII?

A

Cryoprecipitate

215
Q

What is the risk of infection transmission in blood transfusions?

A

Low

216
Q

What is the cause of hyperkalemia in blood transfusions?

A

Lyse of RBCs in old blood units

217
Q

What causes hypocalcemia in blood transfusions?

A

Citrate in blood chelates calcium

218
Q

What is the strongest predictor of prognosis in Hodgkin lymphoma?

A

Stage

219
Q

Which has a better prognosis, Hodgkin lymphoma or non-Hodgkin lymphoma?

A

Hodgkin lymphoma

220
Q

What type of cells that are specific for Hodgkin lymphoma are seen on histology?

A

Reed-Sternberg cells

221
Q

What virus is associated with Hodgkin lymphoma?

A

EBV

222
Q

Which gender is more commonly affected by Hodgkin lymphoma?

A

Men (except nodular sclerosing type)

223
Q

What age group is commonly affected by Hodgkin lymphoma?

A

Bimodal: Men > 55 years and young adults

224
Q

How are lymph nodes affected in Hodgkin lymphoma?

A

Localized, single group of nodes with contiguous spread

225
Q

How are lymph nodes affected in non-Hodgkin lymphoma?

A

Multiple lymph nodes involved, extranodal involvement common, noncontiguous spread

226
Q

What cell types are affected in non-Hodgkin lymphoma?

A

Mostly B-cells; a few are T-cell types

227
Q

What age group is affected by non-Hodgkin lymphoma?

A

Both children and adults

228
Q

What diseases are associated with non-Hodgkin lymphoma?

A

Autoimmune diseases and HIV

229
Q

Which type of Hodgkin lymphoma commonly affects young women?

A

Nodular sclerosis type

230
Q

Which type of Hodgkin lymphoma has the best prognosis?

A

Lymphocyte rich

231
Q

Which type of Hodgkin lymphoma has eosinophilia in immunocompromised patients?

A

Mixed cellularity

232
Q

What causes eosinophilia in Hodgkin lymphoma mixed cellularity type?

A

Increased IL-5

233
Q

Which type of Hodgkin lymphoma is more commonly seen in immunocompromised patients?

A

Lymphocyte depleted

234
Q

Which type of Hodgkin lymphoma has the worst prognosis?

A

Lymphocyte depleted

235
Q

What age group is commonly affected by Burkitt lymphoma?

A

Adolescents or young adults

236
Q

What chromosomal translocation is seen in Burkitt lymphoma?

A

t(8;14) of c-myc and heavy-chain Ig

237
Q

Which chromosome has the c-myc gene located on it?

A

Chromosome 8

238
Q

Which chromosome has the heavy-chain Ig gene located on it?

A

Chromosome 14

239
Q

Non-Hodgkin lymphoma with “starry sky” appearing sheets of lymphocytes with interspersed “tingible body” macrophages

A

Burkitt lymphoma

240
Q

What virus is associated with Burkitt lymphoma?

A

EBV

241
Q

In which group of people is the endemic form of Burkitt lymphoma with typical jaw lesions common?

A

Africans

242
Q

What area of the body is affected by the sporadic form of Burkitt lymphoma?

A

Pelvis or abdomen

243
Q

On what chromosome is the BCL-2 gene located?

A

Chromosome 18

244
Q

What age group is affected by diffuse large B-cell lymphoma?

A

Typically adults, some children

245
Q

What chromosomal translocation is seen in diffuse large B-cell lymphoma?

A

Alterations to BCL-2 and BCL-6

246
Q

What does BCL-2 inhibit?

A

Apoptosis

247
Q

Non-Hodgkin lymphoma that presents with painless “waxing and waning” lymphadenopathy with follicle-like nodules throughout lymph node

A

Follicular lymphoma

248
Q

What is the chromosomal translocation seen in follicular lymphoma?

A

t(14;18) heavy-chain Ig and BCL-2

249
Q

What is the most common type of non-Hodgkin lymphoma in adults?

A

Diffuse large B-cell lymphoma

250
Q

What is the histology of grade 1 follicular lymphoma?

A

Small cleaved cells

251
Q

What is the histology of grade 2 follicular lymphoma?

A

Mixture of large and small cells

252
Q

What is the histology of grade 3 follicular lymphoma?

A

Large cells

253
Q

What is the treatment for follicular lymphoma?

A

Low-dose Rituximab

254
Q

What is the target of Rituximab

A

CD20

255
Q

What CD marker is seen on small B-cells of follicular lymphoma?

A

CD20+ B-cells

256
Q

Very aggressive type of non-Hodgkin lymphoma where patients present with late-stage disease

A

Mantle cell lymphoma

257
Q

What age group is affected by mantle cell lymphoma?

A

Adult males

258
Q

What is the chromosomal translocation in mantle cell lymphoma?

A

t(11;14) cyclin D1 and heavy-chain Ig

259
Q

What cell surface marker is seen in mantle cell lymphoma?

A

CD5+

260
Q

What age group is affected by marginal zone lymphoma?

A

Adults

261
Q

What is the chromosomal translocation in marginal zone lymphoma?

A

t(11;18)

262
Q

What diseases are associated with marginal zone lymphoma?

A

Sjogren syndrome, chronic gastritis

263
Q

What is the typical presentation of marginal zone lymphoma?

A

Unilateral parotid gland enlargement

264
Q

What age group is affected by primary central nervous system lymphoma?

A

Adults

265
Q

Which non-Hodgkin lymphoma is considered an AIDS-defining illness?

A

Primary central nervous system lymphoma

266
Q

What is commonly seen on MRI in primary central nervous system lymphoma?

A

Mass lesions on MRI

267
Q

Primary central nervous system lymphoma needs to be distinguished from what other disease that also presents with mass lesions on MRI?

A

Toxoplasmosis

268
Q

The pathogenesis of primary central nervous system lymphoma involves what type of infection?

A

EBV infection

269
Q

Which lymphoma is associated with IV drug use, caused by HTLV and presents with cutaneous lesions?

A

Adult T-cell lymphoma

270
Q

Lytic bone lesions and hypercalcemia in a person of Japanese descent with a history of IV drug use has what diagnosis?

A

Adult T-cell lymphoma

271
Q

What countries have a higher incidence of adult T-cell lymphoma?

A

Japan, Caribbean, and West Africa

272
Q

Adult with skin patches characterized by atypical CD4+ cells with “cerebriform” nuclei and intraepidermal neoplastic cell aggregates has what condition?

A

Mycosis fungoides

273
Q

What can mycosis fungoides progress to?

A

Sezary syndrome

274
Q

What can follicular lymphoma progress to?

A

Diffuse large B-cell lymphoma

275
Q

What is the cause of hypercalcemia in multiple myeloma?

A

Increased osteoclast activity

276
Q

What is the cause of renal failure in multiple myeloma?

A

Light chains deposited in kidney

277
Q

What is the cause of Rouleaux formation seen in RBCs

A

Loss of charge between RBCs

278
Q

What are common findings in multiple myeloma?

A 
CRAB:
HyperCalcemia
Renal involvement
Anemia
Bone lytic lesions/Back pain
279
Q

What are the Ig light chains in urine ?

A

Bence Jones protein

280
Q

What are the X-ray findings in multiple myeloma?

A

Punched out lytic bone lesions

281
Q

What is the most common primary tumor arising within bone?

A

Multiple myeloma

282
Q

What percentage of monoclonal plasma cells in bone marrow must be present in multiple myeloma?

A

> 10%

283
Q

What malignancy presents with M-spike on serum protein electrophoresis, “fried-egg” appearing plasma cells with “clock-face” chromatin and intracytoplasmic inclusions?

A

Multiple myeloma

284
Q

What Ig globulin make up the M-spike in multiple myeloma?

A

IgG > IgA

285
Q

B-cell lymphoma with hyperviscosity syndrome and no CRAB findings or lytic lesions on X-ray and IgM M-spike

A

Waldestrom macroglobulinemia

286
Q

What is the treatment for Waldestrom macroglobulinemia?

A

Plasmapheresis

287
Q

Presents with bone marrow < 10% monoclonal plasma cells, is asymptomatic and no CRAB findings

A

MGUS

288
Q

What is the rate of MGUS developing into multiple myeloma?

A

1-2% per year

289
Q

What is the most common cause of death in multiple myeloma?

A

Infections

290
Q

Neutrophils with bilobed nuclei typically seen after chemotherapy

A

Pseudo-Pelger-Huet anomaly

291
Q

What percentage of blasts are seen in acute leukemia?

A

> 20%

292
Q

Cause of anemia in leukemia?

A

Decreased RBCs

293
Q

Cause of infections in leukemia?

A

Decreased WBCs

294
Q

Cause of hemorrhage in leukemia?

A

Decreased platelets

295
Q

Leukemia in the skin?

A

Leukemia cutis

296
Q

What trisomy is associated with T-ALL?

A

Down syndrome

297
Q

What type of markers are seen in T-ALL T-cells?

A

CD2+ and CD8+ (TdT+ cells)

298
Q

T-ALL commonly affects what age group?

A

Teenagers

299
Q

What common finding is seen in T-ALL?

A

Thymic mass (presents as SVC-like syndrome)

300
Q

Which chromosomal translocation is associated with a better prognosis in Acute Lymphoblastic Leukemia/Lymphoma?

A

t(12;21) seen in kids

301
Q

Which type of Acute Lymphoblastic Leukemia/Lymphoma is more common and has a better prognosis, B-ALL or T-ALL?

A

B-ALL

302
Q

Acute Lymphoblastic Leukemia/Lymphoma may spread to what parts of the body?

A

CNS and testes

303
Q

What translocation is considered the Philadelphia chromosome?

A

t(9;22)

304
Q

What cell surface marker is seen on B-ALL B-cells?

A

CD10+ marker

305
Q

Which age group affected by Acute Lymphoblastic Leukemia/Lymphoma has the worst prognosis?

A

Adults ( usually have Philadelphia chromosome)

306
Q

What is the most common adult leukemia?

A

Chronic lymphocytic leukemia (CLL)

307
Q

What is the age range for Chronic lymphocytic leukemia?

A

> 60 years

308
Q

What cell surface markers are associated with Chronic lymphocytic leukemia?

A

CD20, CD23, CD5

309
Q

What type of cells are seen on a peripheral blood smear?

A

Smudge cells

310
Q

What type of anemia is associated with CLL?

A

Autoimmune hemolytic anemia

311
Q

What is the danger of CLL?

A

Richter transformation

312
Q

What is Richter transformation?

A

CLL transforming into an aggressive lymphoma like diffuse large B-cell lymphoma

313
Q

Lymphoid neoplasm with cells that have filamentous, hair-like projections on LM that stains with tartrate-resistant acid phosphatase

A

Hairy cell leukemia

314
Q

What age group is affected by hairy cell leukemia?

A

Adult males

315
Q

A mature B-cell tumor that has a dry tap on aspiration and patients present with splenomegaly and pancytopenia

A

Hairy cell leukemia

316
Q

What is the treatment for hairy cell leukemia?

A

Cladribine or Pentostatin

317
Q

What is the median age of onset for acute myelogenous leukemia (AML)?

A

65 years

318
Q

What are typical myeloperoxidase positive inclusions seen in AML?

A

Auer rods

319
Q

What risk factors are associated with AML?

A

Exposure to alkylating chemotherapy, radiation, and myeloproliferative disorders

320
Q

What trisomy is associated with AML?

A

Down syndrome

321
Q

APL responds well to what treatment?

A

All-trans-retinoic acid (vitamin A)

322
Q

What is the effect of all-trans-retinoic acid (vitamin A) in APL?

A

Induces differentiation of promyelocytes

323
Q

What is the chromosomal translocation seen in APL?

A

t(15;17)

324
Q

Which myeloid neoplasm is defined by the Philadelphia chromosome, t(9;22) and myeloid stem cell proliferation?

A

Chronic myelogenous leukemia

325
Q

What age group gets CML?

A

All age groups; peaks at 45-85 years; median age 65 years

326
Q

Myeloid neoplasm that presents with dysregulated production of mature and maturing granulocytes like neutrophils, metamyelocytes and basophils

A

Chronic myelogenous leukemia

327
Q

What is a complication of CML?

A

May accelerate and transform to AML, ALL - considered a “blast crisis”

328
Q

What is a complication if cell with Auer rod ruptures?

A

DIC

329
Q

What is the treatment for CML?

A

Imatinib

330
Q

What is the mechanism of Imatinib?

A

Bcr-Abl tyrosine kinase inhibitor

331
Q

What is the value of leukocyte alkaline phosphatase in CML?

A

Low due to low activity of malignant neutrophils

332
Q

Which myeloproliferative disorder is associated with the Philadelphia chromosome?

A

CML

333
Q

What is the treatment for Polycythemia vera?

A

Phlebotomy, Hydroxyurea, Ruxolitinib

334
Q

What is the target of Ruxolitinib?

A

JAK1/2 inhibitor

335
Q

Presents with intense itching after hot shower, severe burning pain and red-blue discoloration due to episodic blood clots in vessels of the extremities

A

Polycythemia vera

336
Q

Lab findings in polycythemia vera

A

Increased: RBCs, WBCs, Platelets
Positive: JAK2 mutations

337
Q

Lab findings in essential thrombocythemia

A

Increased: Platelets
Positive: JAK2 mutations (30-50%)

338
Q

Characterized by massive proliferation of megakaryocytes and platelets with blood smear showing markedly increased number of platelets that are large or abnormally formed

A

Essential thrombocythemia

339
Q

Is erythromelalgia more common in Polycythemia Vera or Essential Thrombocythemia?

A

PV > ET

340
Q

Lab findings in myelofibrosis

A

Decreased: RBCs
Variable: WBCs, Platelets
Positive: JAK2 mutations (30-50%)

341
Q

What causes obliteration of bone marrow in myelofibrosis?

A

Increased fibroblast activity

342
Q

Teardrop RBCs and massive splenomegaly with fibrosis of bone marrow is seen in what myeloproliferative disorder?

A

Myelofibrosis

343
Q

Lab findings in CML

A

Increased: WBCs, Platelets
Decreased: RBCs
Positive: Philadelphia chromosome: t(9;22)

344
Q

Translocation associated with Burkitt lymphoma (c-myc activation)

A

t(8;14)

345
Q

Translocation associated with CML (Bcr-Abl hybrid), ALL (less common and poor prognosis)

A

t(9;22) - Philadelphia chromosome

346
Q

Translocation associated with mantle cell lymphoma (cyclin D1 activation)

A

t(11;14)

347
Q

Translocation associated with follicular lymphoma (Bcl2-activation)

A

t(14;18)

348
Q

Translocation associated with APL (M3 type of AML)

A

t(15;17)

349
Q

Translocation associated with marginal zone lymphoma

A

t(11;18)

350
Q

Which chromosome causes the overexpression of the other genes seen in Burkitt lymphoma, mantle cell lymphoma, and follicular lymphoma?

A

Chromosome 14

351
Q

EM shows Birbeck granules that look like tennis rackets or rod-shaped and cells express S-100 and CD1a; also seen are lytic bone lesions in a child - what is the disease?

A

Langerhans cells histiocytosis

Hematology/Oncology-First Aid 2017 (5 decks)

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