AIHA Flashcards

1
Q

What are the 4 causes of a positive DAT?

A
  • Txn rxns
  • HDFN
  • Autoimmune hemolytic anemias (warm and cold)
  • Drugs
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2
Q

Transfusion reactions

- Alloimmune? Autoimmune?

A

Alloimmune

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3
Q

Transfusion reactions

- Appearance of DAT

A

?

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4
Q

HDFN

- Alloimmune? Autoimmune?

A

Alloimmune

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5
Q

HDFN

- Appearance of DAT

A

?

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6
Q

Autoimmune hemolytic anemia

- Alloimmune? Autoimmune?

A

Autoimmune

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7
Q

Autoimmune hemolytic anemia

- Appearance of DAT

A

Positive

- Autocontrol is positive

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8
Q

Drugs

- Alloimmune? Autoimmune?

A

?

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9
Q

Drugs

- Appearance of DAT

A

Positive (common)

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10
Q

Typical lab findings of a patient w/ AIHA

A
  • Macrocytosis
  • Spherocytosis
  • ↑ retics, unconjugated bili, LDH
  • ↓ haptoglobin
  • Intravascular hemolysis may lead to hemoglobinemia and hemoglobinurea
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11
Q

Diagnostic test for AIHA

A

Positive DAT (positive AC and Rh control also)

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12
Q

Categories of AIHA

A
  • Cold AIHA → CHD, M. pneumoniae, IM
  • PCH
  • Warm AIHA
  • Drug-induced hemolytic anemia
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13
Q

Autoanti-I

- Who makes the autoAbs?

A

Everyone?

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14
Q

Autoanti-I

- Expected test rxns w/ adult vs. cord cells

A
  • Reacts w/ adult cells

- Non-reactive w/ cord cells

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15
Q

Autoanti-H

- Who makes the autoAbs?

A

Seen in group A1 and A1B patients, whose cells have most of the “H” Ag

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16
Q

Autoanti-H

- Expected test rxns w/ adult vs. cord cells

A

Strongly reactive w/ group O cells and non-reactive w/ A1 or A1B

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17
Q

Procedure that removes Abs (usually autoAbs or Ab to high frequency Ags) from aliquot of serum in order to see if there are underlying Abs in the serum aliquot

A

Adsorption (DAT, elution w/ EGA/CDP treated patient cells, IAT)

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18
Q

Procedure for adsorption

A

Performed by incubating serum w/ cells having corresponding Ag under optimal conditions so Ab will attach to cells (Ag), thus removing Ab from serum upon centrifugation
- Underlying Abs will be detected upon testing eluate adsorbed serum

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19
Q

Interpretation of adsorption results

A

?

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20
Q

This is performed when IgG Ab is coating the cells in order to ID the Ab

A

Elution

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21
Q

Procedure for elution

A

Cells that are coated w/ Abs are treated (w/ acid) to disrupt bonds b/w Ag and Ab
- Abs released into supernate (eluate), which can then be tested for identification of Ab

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22
Q

Interpretation of eluate results

A

?

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23
Q

Demonstrates anti-P specificity and is diagnostic test used for PCH

A

Donath-Lndsteiner test

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24
Q

Procedure for Donath-Landsteiner test

A

2 red top (serum) tubes drawn from patient and kept at 37°C; control tube remains at 37°C; the “test tube” is incubated at 4’C and then back to 37°C, centrifuge and look for hemolysis in both tubes

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25
Q

Interpretation of Donath-Landsteiner test

A
  • Pos: hemolysis

- Neg: no hemolysis

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26
Q

Purpose is to remove the Ab coating the cell when patient ccells can’t be AHG-Ag typed by routine methods (such as in WAIHA)

A

EGA or CDP treatment of patient cells

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27
Q

Procedure for EGA or CDP treatment of patient cells

A

?

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28
Q

Interpretation of EGA or CDP treatment of patient cells

A

?

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29
Q

?

A

Pre-warm testing

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30
Q

Procedure for pre-warm testing

A

?

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31
Q

Interpretation of pre-warm testing

A

?

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32
Q

List the 3 different types of adsorption

A
  • Autoadsorption
  • Homologous adsorption
  • Differential adsorption or “triple” adsorption
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33
Q

Adsorption where patient can’t have been transfused in the past 3 months

A

Autoadsorption using patient’s own cells

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34
Q

Adsorption where patient has been recently transfused and therefore must use donor cells that have patient’s same phenotype

A

Homologous adsorption

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35
Q

Adsorption most common in reference labs using 3 donors w/ known phenotypes for all other blood groups

A

Differential adsorption or “triple” adsorption

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36
Q

Abs being directed against an individual’s own RBCs

A

AutoAbs

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37
Q

IgG Ab that binds to patient’s cells at cold temps, fixes C’, causes intravascular hemolysis at 37°C (IgG elutes off cells at 37°C)

A

Biphasic autohemolysin

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38
Q

?

A

Selective allogeneic adsorption

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39
Q

?

A

ZZAP treatment

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40
Q

?

A

Panreactive

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41
Q

?

A

Polyagglutinable

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42
Q

?

A

Least incompatible

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43
Q

Shortened red cell survival due to immune response (Ab production)

A

IHA

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44
Q

What is the autoAb production theory?

A

Autoantibody production is usually prevented by feedback mechanism. Suppressor T cells induce tolerance to “self” Ags by inhibiting B cell activity. If T-suppressor cells lose function then autoantibody production results

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45
Q

Appearance of positive DATs for autoAbs

A

?

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46
Q

Appearance of positive DATs for alloAbs due to txn rxns

A

Recipient Ab attaches to transfused donor cells w/ corresponding Ag

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47
Q

Appearance of alloAbs due to HDFN

A

Mother’s IgG Ab crosses placenta and attaches to baby’s paternally-derived Ags

48
Q

Purpose is to remove the cold autoAb from serum so underlying alloAbs can be detected

A

Cold autoadsorption

49
Q

Procedure for cold autoadsorption

A

Aliquots of patient’s own cells are used to remove autoantibody from patient’s serum at 4’C, leaving alloab in absorbed serum; only performed if no recent transfusions in the last 3 months; test adsorbed serum at 37°C-AHG

50
Q

Effect of a positive DAT on weak D or other AHG-Ag typings

A

Since the patient cells are already coated w/ Ab the AHG/weak D will always be positive unless treated

51
Q

AIHA is problematic for BB testing, b/c patients destroy their own cells as well as ____ cells, and all XM may be ____

A

Donor; incompatible

52
Q

Which Rh Ab is the most common?

A

Anti-e

53
Q

List 4 mechanisms of drug-induced IHA

A
  • Drug independent
  • Immune complex (innocent bystander)
  • Drug-adsorption (Hapten)
  • Membrane modification
54
Q

Immune complex/innocent bystander mechanism of drug-induced IHA
- Theory

A

Drug binds w/ anti-drug and forms complex that accidentally “bumps” into RBCs and cause positive DAT by binding C’ onto cell

55
Q

Immune complex/innocent bystander mechanism of drug-induced IHA
- What is DAT coated w/?

A

C’ only

56
Q

Immune complex/innocent bystander mechanism of drug-induced IHA
- Eluate pattern

A

Non-reactive

57
Q

Drug-adsorption mechanism of drug-induced IHA

- Theory

A

Drug binds firmly to cell membrane and anti-drug then binds drug → DAT +

58
Q

Drug-adsorption mechanism of drug-induced IHA

- What is DAT coated w/?

A

IgG

59
Q

Drug-adsorption mechanism of drug-induced IHA

- Eluate pattern

A

Non-reactive

60
Q

Membrane modification mechanism of drug-induced IHA

- Theory

A

Drug and plasma proteins adsorb onto memebrane and cause positive DAT

61
Q

Membrane modification mechanism of drug-induced IHA

- What is DAT coated w/?

A

Variable (IgA, IgM, IgG may bind C’ so DAT varies as to which component is positive)

62
Q

Membrane modification mechanism of drug-induced IHA

- Eluate pattern

A

Non-reactive

63
Q

Drug independent mechanism of drug-induced IHA

- Theory

A

T-suppressor cells altered causing a production of autoAbs →DAT +

64
Q

Drug independent mechanism of drug-induced IHA

- What is DAT coated w/?

A

IgG

65
Q

Drug independent mechanism of drug-induced IHA

- Eluate pattern

A

Panreactive (IAT +)

66
Q

Hemolytic Txn Rxn (HTR)

- Ig type

A

IgM or IgG

67
Q

HTR

- DAT + due to…

A

IgG and/or C’ (DAT mf)

68
Q

HTR

- IAT pos/neg?

A

Positive for specific alloAb

69
Q

HTR

- Eluate pattern

A

Specific alloAb

70
Q

HTR

- Txn requirements

A

Ag negative blood

71
Q

CHD

- Patient population

A

Eldelry or middle-aged

72
Q

CHD

- Pathogenesis

A

Idiopathic

- Secondary to M. pneumoniae or infectious mononucleosis

73
Q

CHD

- Clinical features

A
  • Acrocyanosis
  • Numbness in extremities
  • Raynaud’s syndrome
  • Hemogloinurea (in some)
  • Autoagglutination of blood at RT
74
Q

CHD

- Severity of hemolysis

A

Chronic and rarely severe

75
Q

CHD

- Site of hemolysis

A

Extra/invascular

76
Q

CHD

- Thermal range

A

High (up to 31°C)

77
Q

CHD

- Titer

A

High ( > 1000)

78
Q

CHD

- Donath-Landsteiner test

A

Negative

79
Q

CHD

- Treatment

A

Keep warm/avoid the cold

- If needs txn, transfuse blood through a blood warmer

80
Q

CHD

- Cause

A

Anti-I (very high titers and greater thermal amplitude)

81
Q

CHD

- Reactivity temperature

A

4°C

82
Q

Paroxysmal Cold Hemoglobinurea (PCH)

- Patient population

A

Kids and young adults

83
Q

PCH

- Pathogenesis

A

Following infection (measles, chicken pox, flu, infectious mono)

84
Q

PCH

- Clinical features

A
  • Fever
  • Shaking, chills
  • Malaise
  • Abdominal cramps
  • Back pain
85
Q

PCH

- Severity of hemolysis

A

Acute and rapid

86
Q

PCH

- Site of hemolysis

A

Intravascular

87
Q

PCH

- Thermal range

A

Moderate ( < 20°C)

88
Q

PCH

- Titer

A

Moderate (< 64)

89
Q

PCH

- Donath-Landsteiner test

A

Positive

90
Q

PCH

- Treatment

A

Avoid cold exposure (supportive)

91
Q

CHD

- Lab findings

A
  • Hemoglobinurea

- High titer anti-I (?)

92
Q

PCH

- Lab findings

A
  • Hemoglobinurea

- Hemoglobinemia

93
Q

CHD

- Ig type

A

IgM (anti-I/i)

94
Q

PCH

- Ig type

A

IgG (anti-P, biphasic hemolysin)

95
Q

CAIHA

- DAT + due to…

A

C’ only

96
Q

CAIHA

- IAT pos/neg?

A

Positive

- anti-I, anti-H, anti-IH, DL Ab

97
Q

CAIHA

- Eluate pattern

A

Nonreactive

98
Q

CAIHA

- Txn requirements

A

Blood warmer

99
Q

WAIHA

- Ig type

A

IgG

100
Q

WAIHA

- DAT + due to…

A

IgG w/ or w/o C’

  • Patient cells can’t be AHG-Ag typed by routine methods
  • Must treat patient cells w/ EGA or Chloroquine diphosphate (CDP) to remove the Ab coating cells
101
Q

WAIHA

- IAT pos/neg?

A

Positive or negative

- ID positive adsorption must be performed to look for underlying alloAbs

102
Q

WAIHA

- Eluate pattern

A

Panreactive or Rh specificity

103
Q

WAIHA

- Txn rxn

A

Least incompatible; phenotypically similar blood

104
Q

Drug-induced IHA

- Txn rxn

A

XM compatible

105
Q

WAIHA

- Treatment

A
  • Cortosteroids
  • Splenectomy
  • Immunosuppressive drugs
  • Txn as a last resort
106
Q

Drug-induced IHA

- Treatment

A

Discontinue drug

107
Q

WAIHA

- What temperature does it react?

A

37°C

108
Q

WAIHA

- Majority of patients will have ____ and need ____

A

Anemia; txns

109
Q

WAIHA

- Symptoms

A

Pallor, weakness, dizziness, dyspnea, jaundice, unexplained fever

110
Q

WAIHA

- Onset is usually ____

A

Chronic

111
Q

WAIHA

- The MOST important part of a workup is ____

A

To identify any underlying alloAbs

- Adsorption procedures often needed

112
Q

WAIHA will have ____ autocontrol,

WAIHA will have ____ autocontrol

A

Positive; negative

113
Q

WAIHA is ____ w/ ficin/PEG/gell than LISS; HTLA are ____ w/ the media

A

Enhanced; variable

114
Q

WAIHA is ____ w/ all cells; HTLAs are ____ if tested against Ag negative cells

A

Positive; negative

115
Q

Which adsorption technique would you recommend for an untransfused patient diagnosed w/ WAIHA and whose RBCs are coated w/ Ab (DAT +)?

A

Autoadsorption

116
Q

Describe the process of autoadsorption

A
  1. Separate cells from serum so cells can be treated
  2. ZZAP treat patient cell s(removes Ab coating cells and enhances binding sites)
  3. Wash ZZAP completely and leave packed cell aliquotes w/ no saline in them
  4. Add an aliquot of patient’s serum to ZZAP-treated patient cells in ratio 1:1
  5. Incubate 30 min @ 37°C → spin → remove supernate and put on next aliquot of ZZAP-treated cells
  6. Repeat steps 4 and 5 for all aliquots (max of 4x)
  7. Test supernate adsorbed serum w/ panel of selected cells to ID underlying alloAbs
117
Q

It’s generally NOT possible to find compatible RBCs for WAIHA patients, but if the clinican determines that the patient’s hgb does indicate the need for txn, what is the procedss for selection of RBCs in this situation?

A
  • Phenotype patient (may need to EGA and cell separate) for Rh and K Ags
  • Provide donor cells that are Ag negative for any Rh or K-Ag that patient is negative for
  • Perform full XM on Rh/K-negative donor cells → incompatible @ AHG
  • Transfuse patient w/ least compatible blood → must have Dr’s consent to give least compatible