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Blood Bank - JD > Autoimmune Hemolytic Anemias > Flashcards

Autoimmune Hemolytic Anemias Flashcards

1
Q

What is defined as shortened RBC survival mediated through the immune response, specifically by humoral antibody?

A

immune hemolytic anemia

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2
Q

Name the 3 broad categories of immune hemolytic anemias.

A
  1. Alloimmune
  2. Autoimmune (AIHA)
  3. Drug-induced (DIIHA)
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3
Q

In an intravascular hemolytic event, what substance rises, peaks, and falls in urine within 2 days?

A

Hemoglobin

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4
Q

In an intravascular hemolytic event, what substance decreases drastically within 1 day?

A

serum haptoglobin

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5
Q

In an intravascular hemolytic event, what substance in urine rises in 2-3 days, stays broadly peaked at 5-6 days, and slowly decreases after day 6?

A

hemosiderin

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6
Q

What is hemosiderin?

A

An iron containing pigment derived from hemoglobin when RBCs are destroyed. Serves as a packager of iron so that it can be reused.

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7
Q

Antibodies that are directed against an individual’s own RBCs are termed:

A

autoantibodies (autoantiagglutinins)

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8
Q

Studies have suggested that autoantibodies occur because of a …

A

failure of the immune regulatory responses (T regulator cells)

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9
Q

Autoantibodies cause…

A

shortened RBC survival in vivo

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10
Q

TRUE OR FALSE: A positive DAT, positive autocontrol, or serum autoantibody confers the diagnosis of AIHA.

A

FALSE

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11
Q

Serologic ABO discrepancies, a positive Rh control, or a positive DAT may suggest the presence of…

A

autoantibodies

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12
Q

If a patient who does not have autoantibodies, but has a positive DAT can be transfused with which blood?

A

least incompatible blood that the doctor signed for

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13
Q

Type of anemia where the rate of RBC production will nearly equal the rate of RBC destruction.

A

compensated anemia

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14
Q

The type of anemia where the rate of RBC destruction exceeds the rate of RBC production.

A

uncompensated anemia

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15
Q

How will compensated anemia affect:
Hgb
Hct
Retics

A

Hgb: decrease
Hct: decrease
Retics: increase

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16
Q

How will uncompensated anemia affect:
bilirubin
haptoglobin
LDH

A

bilirubin: increase
haptoglobin: decrease
LDH: increase

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17
Q

Macrocytosis and spherocytosis are seen in which anemia?

A

uncompensated anemia

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18
Q

AIHA can be diagnosed and classified as what 3 classifications?

A

cold reactive, warm reactive, or drug-induced

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19
Q

Autoantibodies can be characterized by their…

A

optimal temperature of reactivity.

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20
Q

What percentage of reported cases of AIHA are:
warm reactive
cold reactive
drug induced

A

warm reactive: 70%
cold reactive:18%
drug induced:12%

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21
Q

Cold reactive autoantibodies are most frequently encountered where?

A

serologic testing

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22
Q

TRUE OR FALSE: Cold reactive autoantibodies are not clinically significant.

A

FALSE. Most typically aren’t but occasionally they are clinically significant and cause immune hemolytic anemia.

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23
Q

When testing is performed at 4 degrees C, which type of autoantibody is most commonly encountered?

A

cold reactive autoantibody

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24
Q

A typical cold autoantibody will have a low or high titer?

A

low titer (<64 at 4 degrees C)

25
Q

TRUE OR FALSE: cold agglutinins do not interfere with serologic testing at room temperature.

A

FALSE. They can interfere with ABO/Rh typing, DAT, antibody detection/identification, and compatibility testing.

26
Q

Most cold autoantibodies have what specificity?

A

anti-I

27
Q

TRUE OR FALSE: anti-I is a relatively uncommon autoantibody

A

TRUE.

28
Q

Which Ii antigen is most commonly expressed on adult cells, but very weakly, if at all, on cord cells?

A

I

29
Q

Anti-H specificity may be found in individuals of what ABO type(s)?

A

A1 and A1B (occasionally B)

30
Q

Which ABO types react best with anti-H and why?

A

O and A because they have the most H antigen expressed on the cells

31
Q

Cold agglutinins in group A1 and A1B individuals may have what specificity?

A

anti-H

32
Q

What makes a cold agglutinin pathogenic?

A

reacting at body temp in vivo

33
Q

What disease is often associated with an infectious disease, such as Mycoplasma pneumoniae or Infectious Mono?

A

Cold AIHA

34
Q

Cold autoantibodies are typically of which Ig class, which means they are good at what?

A

IgM, which are good at activating complement

35
Q

Chronic hemagglutinin disease occurs predominantly in what population?

A

> 50 years old

36
Q

Antibody specificity in CHD is typically ….. and rarely ….

A

typically anti-I and rarely anti-Pr

37
Q

A positive DAT due to complement only and reticulocytosis are common laboratory findings of what disease?

A

chronic hemagglutinin disease (CHD)

38
Q

If an individual is diagnosed with CHD, what does the doctor usually recommend?

A

no treatment needed, but avoid the cold; stay in warm areas

39
Q

What is a least common type of AIHA that is most often seen in children in association to viral illnesses?

A

Paroxysmal Cold Hemoglobinuria (PCH)

40
Q

The Donath-Landsteiner antibody is an autoantibody with which specificity?

A

anti-P specificity

41
Q

The Donath-Landsteiner antibody is a classic antibody produced in which condition?

A

Paroxysmal Cold Hemoglobinuria (PCH)

42
Q

TRUE OR FALSE: harmless warm autoantibodies are serologically indistinguishable from the harmful ones.

A

TRUE

43
Q

TRUE OR FALSE: A significant number of warm autoantibody cases suffer severe anemia sufficient enough to require a transfusion.

A

TRUE

44
Q

What percentage of Warm autoantibody cases have:
IgG only
Complement only
IgG and complement

A

IgG only: 20%
Complement only: 13%
IgG and complement: 67%

45
Q

IgG reacted best by which antiglobulin technique?

A

indirect antiglobulin

46
Q

What type of RBC destruction occurs in WAIHA?

A

Extravascular immune RBC destruction

47
Q

When there is no other reasons for certain serologic and hematological findings, what should be suspected?

A

drug-induced hemolytic anemia

48
Q

What is the term that describes a possible causative mechanism when more than one drug-associated antibody specificity is present?

A

unifying hypothesis

49
Q

What are 3 mechanisms in which drugs cause immune hemolytic anemias?

A
  1. Drug binds to RBC membrane and antibody formed is directed at the drug.
  2. Drug doesn’t covalently bind RBC membrane, but complexes with drug antibody
  3. Drug induces an autoimmune response, but the antibody Is directed toward the RBC membrane
50
Q

Drugs operating through the drug-adsorption mechanism bind firmly to…

A

proteins

51
Q

Which drug is most commonly associated with the drug-adsorption (hapten) mechanism?

A

penicillin

52
Q

Most penicillin antibodies are of what Ig class?

A

IgM

53
Q

The penicillin antibody responsible for a positive DAT is most often of which class?

A

IgG

54
Q

What is the drug-adsorption (hapten) mechanism?

A

Drug bind to the RBC membrane and an antibody is formed against the drug bound to the RBC

55
Q

What is the drug-dependent or immune complex (innocent bystander) mechanism?

A

The drug reacts in a non-covalent manner with a specific membrane component and forms a new antigen (neoantigen) determinant with both drug and membrane components

56
Q

The concept of “neoantigen” formations has been proposed to explain which DIIHA mechanism?

A

Drug-dependent or immune complex (innocent bystander)

57
Q

How does membrane modification (Nonimmunologic protein adsorption) mechanism cause DIIHA?

A

Drugs adsorbed to the RBC membrane modify the RBCs so that plasma proteins can bind to the membrane

58
Q

Which drugs are known to modify RBC membranes when bound?

A

cephalosporins

59
Q

3% of patients taking which cephalosporin may exhibit a positive DAT with polyspecific and monospecific reagents?

A

Keflin (cephalothin

Blood Bank - JD (16 decks)

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