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Boards.CP.Heme > Diseases of RBCs > Flashcards

Diseases of RBCs Flashcards

1
Q

Characteristics of Pathologic Cold Agglutinins.

A
  • Broad thermal range
  • Spontaneous autoagglutination at RT
  • Titer >1,000 at 4 degrees
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2
Q

Cryoglobulinemia:

-Blood smears

A

Pale purple, cloudy aggregates of protein

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3
Q

Stomatcoytic type of HE (SAO) facts (2).

A
  • Band 3 protein defect

- Protection against P. vivax malaria

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4
Q

At what % of total Hi does cyanosis occur?

A

10% of total Hb (or 1.5 G/dL)

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5
Q

Thalassemic indices: RDW

A

Normal to slight increase

*Markedly INCREASED in IDA

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6
Q

What is the % HbF at 6 months; 1 year?

A
  • <10%

- <5%

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7
Q

What causes PCH?

A

-IgG biphasic hemolysin (Donath-Lansteiner Ab)

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8
Q

Hereditary persistence of HbF (HPFH) is caused by what?

A

Delayed switch from gamma (y) to beta or delta chains

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9
Q

What and where are Type I Cryoglobulins found?

A
  • Monoclonal Immunoglobulins

- MM or Waldenstrom

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10
Q

MC etiology in Hereditary Stomatocytosis.

A

Abnormal Na+/K+ permeability

*Ass w/ Rh null phenotype

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11
Q

Hereditary Spherocytosis (HS) gene mutation.

A

ANK1 (ankyrin)

Autosomal Dominant

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12
Q

T/F: Single gene deletions are asymptomatic with a normal CBC and normal electrophoresis.

A

True

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13
Q

Lab findings in G6PD.

A
  • Poikilocytosis, bite cells, blister cells
  • Heinz bodies (w/ supravital dyes)
  • Extravascular hemolysis (Jaundice, Increased LD)
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14
Q

Explain the Donath-Landsteiner Test.

A

2 Vials of blood

  • Vial 1 at 37; Vial 2 at 4 for 30 minutes
  • both at 37 for 30 minutes
  • Positive test - Hemolysis in Vial 2
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15
Q

What are the findings in Hb Bart?

A
  • Severe Hemolytic anemia

- Hb Bart on electrophoresis

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16
Q

Secondary CAIHA is a transient condition associated with infection. Which organisms is associated with:

  • IgM anti-I
  • IgM anti-i
A 
Mycoplasma pneumonia (anti-I)
EBV (anti-i)
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17
Q

What are Type II Cryoglobulins made up of?

A

Mixture of 2 polyclonal Igs

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18
Q

Thalassemic indices: P. Smear

A
  • Microcytosis
  • Target cells
  • Basophilic stippling
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19
Q

The Donath-Landsteiner Ab has specificity for what?

A

Anti-P

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20
Q

HbC disease (homozygous CC) Hb ratios

A

HbC - 90%
HbF - 7%
HbA2 - 3%
HbA - 0%

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21
Q

Hemoglobin Lepore runs with what on alkaline gel?

A

HbS, usually only 15% of total Hb

*Actual HbS is rarely present at this quantity, so suspect Lepore in this scenario

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22
Q

3 alpha gene deletions result in ___ disease.

A

HbH

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23
Q

How many gene deletions are result in alpha-thalasemia TRAIT; and what are the findings?

A
  • 2 gene deletions
    FINDINGS:
    -thalassemic indices
    -Normal HbA2 percentage
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24
Q

4 alpha gene deletions result in ___ disease.

A

Hb Bart

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25
Q

T/F: HbF exerts an inhibitory effect on HbS polymerizaiton.

A

True

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26
Q

What causes Cold Autoagglutinin disease (CAD)/CAIHA?

A

-IgM antibodies to anti-I (most commonly)

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27
Q

Average lifespan of HbSS RBC

A

17 days

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28
Q

HbG and HbD run with what on alkaline gel?

A

HbS (do sickle cell screen or citrate gel)

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29
Q

What are causes of secondary WAIHA? (70% of cases)

A
  • Hematolymphoid (CLL/SLL)
  • Inherited immunodeficiencies (CVID,IgA,etc.)
  • Collagen vascular disease
  • Thymoma
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30
Q

The membrane defects in PNH is caused by what?

A

Decreased Glycosyl Phosphatidyl Inositol (GPI) anchors

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31
Q

Cryoglobulinemia is a systemic immune complex disease characterized by a distinctive clinical syndrome, what are the symptoms? (7)

A
  • Palpable purpura (leukocytoclastic vasculitis)
  • Arthralgia
  • Hepatosplenomegaly
  • Lymphadenopathy
  • Anemia
  • Sensorineural deficits
  • Glomerulonephritis
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32
Q

Beta-Thalasemia trait (Heterozygotes) has what changes in Hb ratios? (what if also Iron Deficient)

A

HbA2 - >3.5%
HbF - Normal to INcreased

*If iron deficient the HbA2 may be normal

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33
Q

alpha or beta Thalassemia??

Manifestations are present at birth.

A
  • Alpha

* Beta become evident >6 months

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34
Q

What causes a warm autoimmune hemolytic anemia (WAIHA)?

A

warm reacting IgG autoantibody

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35
Q

3 types of Hereditary Elliptocytosis

A
  • Common type (HPP; African Americans)
  • Spherocytic type
  • Stomatocytic type (Southeast Asian Ovalocytosis)
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36
Q

Sickle Cell Disease (genotype SS) Hb ratios.

A

HbS - >80%
HbA - 0%
HbA2 - 1-4%
HbF - 1-20%

37
Q

Positive or Negative:

What is the DAT in WAIHA?

A

Positive (nearly ALL cases)

*usually polyspecific and anti IgG reagents

38
Q

What is the presentation of PCH?

A

Paroxysmal episodes of hemoglobinuria associated with COLD Exposure

39
Q

Which type of Cryoglobunimia is most commonly seen?

A

Type 2

40
Q

HbSC disease Hb ratios

A

HbS - 50%

HbC - 50%

41
Q

Beta-Thalasemia

There is a total of # copy of the __- gene on each chromosome __, for a total of __ productive genes.

A

1, Beta, 11, 2

*There i a total of on copy of the B gene (HBB) on chromosome 11, for a total of 2 productive genes.

42
Q

G6PD inheritance

A

X-linked recessive

43
Q

Sickle Cell Trait (genotype SA) Hb ratios.

A

HbS - 30-45%
HbA - 50-65%
HbA2 - <3%

44
Q

Homozygotes (Beta-Thal) hs what Hb ratios?

A

HbF - 50-85%
HbA - little to NONE
HbA2 - Normal to INcreased

45
Q

Hereditary Elliptocytosis/Ovalocytosis gene mutation.

A

Spectrin a-chain

Autosomal Dominant

46
Q

Mutation in Hemoblobin Constant Spring

A

mutation in the alpha globin STOP codon; producing abnrmally long transcript

47
Q

Which population is is most common in Paraxysmal Cold Hemoglobinuria (PCH) and what is it associated with?

A
  • Children
  • Post-Viral (Measles, Mumps, VZV, Mono)

*originally described in syphilis

48
Q

Abnormality in Methemoglobin (Hi, hemiglobin)

A

Iron is in the Oxidized ferric (Fe3+) state instead of usual ferrous (Fe2+) state.

Incapable of combining with Oxygen

49
Q

HbSC disease manifestations

A

Between SS and SA

50
Q

In alpha-thalassemia 1 (a0 thalassemia), chromosome 16 has __ deleted alpha genes. What race is this prevalent in?

A
  • 2

- Asians

51
Q

What are Type II Cryoglobulin made up of?

A

Mixture of Monoclonal IgM and Polyclonal IgG

52
Q

alpha-thalasemia:

There are __ copies of the __ gene on each chromosome __, for a total of __ genes.

A

2, a, 16, 4

*There are 2 copies of the alpha gene on each chromosome 16, for a total of 4 genes.

53
Q

What characteristic finding of PCH may be seen on P. smear?

A

Intraneutrophilic hemophagocytosis

54
Q

What clinical condition is the MCC of Type II cryoglobinemia?

A

HCV

55
Q

What is seen in renal biopsies of cryoglobulinemia?

A
  • Membranoproliferative Glomerulonephritis (MPGN)

* Deposits may produce the appearance of thrombotic microangiopathy

56
Q

How are Beta-Thal Major and Intermedia distinguished ?

A

Transfusion dependence (MAJOR)

57
Q

Characteristics of Nonpathologic Cold Agglutinins.

A
  • React most strongly at 4, up to 22 degrees

- Titer <64 at 4 degrees

58
Q

Thalassemic indices: MCV/RBC count ratio

A

<13 favors thalassemia

*>15 favors IDA

59
Q

What antigens does the pathogenic antibody in WAIHA usually have broad reactivity with?

A

Rh Antigens

60
Q

HbC disease manifestations.

A
  • Mild hemolytic anemia
  • Splenomegaly
  • Target cells (numerous)
  • Hexagonal or Rod-shaped CRYSTALS
61
Q

What is the abnormality in Hb Lepore?

A

fusion between Delta and Beta genes

62
Q

Intravascular or Extravascular Hemolysis in WAIHA?

A

Extravascular

63
Q

Hemoglobin S alteration.

A

B6 glu->val

64
Q

HbG is a defect in what chain?

A

Alpha

65
Q

What are the findings in HbH?

A
  • Hemolytic anemia

- Fast moving HbH on electrophoresis

66
Q

Thalassemic indices: RBC count

A

Normal to Increased RBC count

67
Q

What is the % of HbF at term?

A

30-40%

68
Q

Most alleles with thalassemic phenotype result from what type of a mutation?

A

Point

69
Q

HbD is a defect in what chain?

A

Beta

70
Q

T/F: Paroxysmal Nocturnal Hemoglobinuria (PNH) is an acquired clonal red cell disorder.

A

True

71
Q

Incidence of Sickle Cell Trait in AA.

A

10%

72
Q

What is the mutation/gene associated with PNH?

A

-PIG-A on X chromosome

73
Q

What is the 2nd MC abnormal Hemoglobin worldwide (after S)?

A

HbE (B26 glu->lys)

*Southeast Asia common

74
Q

What is the treatment for Methemoglobin?

A

Methylene blue, reduces Hi to Hb

75
Q

The leukocyte alkaline phosphatase (LAP) score is (increased/decreased) in PHN.

A

Decreased

76
Q

Pyruvate Kinase (PK) deficiency findings & Inheritance

A
  • Chronic hemolysis (varied severity)
  • Echinocytes (dessicocytes)
  • Extravascular Hemolysis
  • Audosomal Recessive
77
Q

What is the % of HbF in Combine HbSS/HPFH and what are symptoms?

A
  • 25%

- MILD symptoms (No anemia or vasoocclusive episodes)

78
Q

HS lab values.

A
  • MCHC Increased
  • Reticulocyte count Increased
  • MCV & MCH normal
79
Q

Fluorescent aerolysin (FLAER), obtained from what, binds specifically to GPI?

A

Aeromonas hydrophila

80
Q

Flow cytometry markers for PNH:

  • RBCs
  • Granulocytes/monocytes
A
  • CD55,59

- CD14,15,16,33

81
Q

Combined Sickle cell (HbSS) and HPFH is found in what percentage of homozygous HbSS?

A

1 percent (~1 in 100)

82
Q

Definition of cryoglobulins.

A

Immunoglobulins that precipitate reversibly at Low Temperatures

83
Q

How do you detect cyroglobulins?

A
  • Blood drawn at 37 and kept till clotted
  • Centrifuge at 37
  • Serum stored at 4 for >3 days
  • Centrifuge at 4
  • Any precipitate that forms is cryoprecipitate
  • Electrophoresis for characterization
84
Q

In alpha-thalassemia 2 (a+ thalassemia), chromosome 16 has __ normal and __ deleted alpha gene. It is the most common genotype in what race?

A
  • 1, 1

- African Americans

85
Q

After age 2 what is the normal % of HbF?

A

<1%

86
Q

In all tissues, what is the basic pathologic lesion in cryoglobulinemia?
-what is seen on EM?

A

Vasculitis

*EM - Subendothelial immune complex deposits with a fibrillary or tubular structure in a fingerprint like pattern

87
Q

MOA of hydroxyurea in SS.

A

Increase HbF and lessen severity of SS.

88
Q

PNH may evolve into what?

A
  • Aplastic Anemia (and or)

- AML

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