Auto Immune Haemolytic Anaemia Part 1 Flashcards

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1
Q

Define Auto Immune Haemolytic Anaemia

A

Premature destruction of RBCs by an autoantibody directed against individual’s own red cells

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2
Q

Define Elution

A

removal of antibodies from the sensitised red cells to identify the antibody

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3
Q

Describe the pathophysiology of AIHA

A
  1. Abnormality in suppressor T cell tolerance to self-antigens
  2. Production of antibodies against own blood group antigens
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4
Q

In what type of individuals is AIHA mostly common in?

A
  1. Haematological malignancy
  2. Individuals with other auto-immune disorders
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5
Q

State the two types of AIHA classifications and state what consists in each

A
  1. Classification I
    a. Warm AIHA
    b. Cold AIHA
  2. Classification II
    a. Drug-induced AIHA
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6
Q

At what temperatures do antibodies in the WAIHA and CAIHA activate in?

A
  1. WAIHA - 37 degrees
  2. CAIHA - 4 to 24 degrees or 30 degrees
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7
Q

what type of AIHA accounts for 18% AIHA

A

Cold AIHA

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8
Q

what type of AIHA accounts for 12% of AIHA

A

drug induced

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9
Q

what type of AIHA accounts for 70% of AIHA

A

Warm AIHA

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10
Q

What are the differences in pathophysiology of WAIHA, CAIHA, and Drug induced?

A

WAIHA - Extravascular, IgG, non complement-activating antibody
CAIHA - Intravascular, IgM, complement-activating antibody
Drug Induced - Intravascular or extravascular

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11
Q

Clinical symptoms of WAIHA

A
  1. Weakness, pallor
  2. Weight loss
  3. Fever
  4. Jaundice
  5. Common in children and adults
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12
Q

Clinical symptoms of Cold Haemolytic Disease

A
  1. Weakness, pallor
  2. Weight loss
  3. Cyanosis in extremities
  4. Numbness in hands and feet
  5. Haemoglobinuria
  6. Common in >50 years old
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13
Q

Haemotology Laboratory results of AIHA

A
  1. Anaemia
  2. Decreased haemoglobin and haematocrit
  3. Increased reticulocyte
  4. Macrocytosis
  5. Spherocytosis
  6. Nucleated RBC
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14
Q

Clinical Chemistry results of AIHA

A
  1. Increased bilirubin
  2. Increased lactate dehydrogenase
  3. low haptoglobin level
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15
Q

Blood bank laboratory results of AIHA

A

Positive DAT

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16
Q

What antibodies are responsible for WAIHA?

A
  1. auto anti-Rh protein
  2. auto anti-e,c,D,C,E,f early in disease
  3. auto antibodies to high incidence antigens like U in MNS
17
Q

what antibodies are responsible for Cold Haemolytic Disease?

A
  1. auto anti-I
  2. auto anti-i
18
Q

what drugs cause AIHA?

A
  1. Quinidine
  2. Phenacetin
  3. Piperacillin
  4. Cephalosporins
  5. Streptomycin
  6. Methyldopa
19
Q

What difficulties arise by working with Warm AIHA?

A
  1. Antibody screen and panel cells are pangglutinins due to auto-antibodies hiding allo-antibodies thus allo-antibody exclusion unavailable
  2. DAT positive cells will immediately agglutinate via IAT
20
Q

What reagent is added to DAT positive cells to make them DAT negative?

A

EDTA glycine acid

21
Q

List the testing procedures of RBCs in WAIHA

A
  1. ABO and Rh(D)
  2. DAT with anti-IgG and anti-C3d separately
  3. Elution
  4. screen and panel of eluate plasma for identification of auto-antibody
  5. Phenotype EGA treated cells
22
Q

List the test procedures of plasma in WAIHA

A
  1. ABO grouping
  2. Antibody screen
  3. Antibody panel
  4. Auto adsorption or allo-adsorption if patient transfused in last 3 months
  5. Screen and panel of adsorbed plasma for detection of allo-antibody
  6. IAT crossmatch on adsorbed plasma
23
Q

Define Auto Adsorption

A

Incubation of patient RBCs against own plasma to remove autoantibodies without removing all antibodies

24
Q

List the treatments for WAIHA

A
  1. High dose of corticosteroids
  2. Intravenous intragram
  3. Splenectomy
  4. Drugs to block antibody production
25
Q

List the criteria of Red cells to transfuse to WAIHA

A
  1. ABO and Rh(D) compatible
  2. Negative for antigens that patient has antibodies to
  3. Phenotype Antigen Matched
  4. IAT crossmatch with adsorbed plasma
  5. “least incompatible”
26
Q

What is the specification of PAM blood in AIHA patients?

A

Transfuse red cells with similar antigen phenotype in Rh, Kell, Kidd, Duffy, and Ss system