Hemolytic Anemia Flashcards

1
Q

what is released upon RBC hemolysis?

A

lactate dehydrogenase (LDH) & hemoglobin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

haptoglobin

A

binds to free hemoglobin in circulation. decreased free levels in intravascular hemolysis.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

bilirubin conjugated vs unconjugated

A

breakdown of heme. conjugated form when released from liver, **unconjugated when released by hemolysis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

clinical manifestations specific to hemolysis

A

jaundice, hemoglobinuria (specifically intravascular hemolysis), pigmented gallstone disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

notable causes of intravascular hemolysis

A

microangiopathy (MAHA), paroxysmal nocturnal hemoglobinuria (PNH), infections

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

causes of extravascular hemolysis

A

herediatry spherocytosis, G6PD deficiency, autoimmune, drug induced, infections

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

proteins defective in hereditary spherocytosis

A

band 3, spectrin, or ankyrin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

diagnostic test for HS?

A

osmotic fragility test (HS cells lyse earlier)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

treatment for HS?

A

splenectomy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

G6PD deficiency

A

renders RBCs more susceptible to free radical damage due to inability to synthesize glutathione

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

morphological consequence of G6PD deficiency?

A

Heinz bodies (denatured hemoglobin aggregates in these within RBCs) with bite cells (macrophages nibble on the cells)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

G6PD A-

A

decreased G6PD activity in aged RBCs (African Americans)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

G6PD B

A

decreased G6PD activity in all RBCs (Mediterranean & Asians)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

clinical manifestations of G6PD deficiency

A

precipitated by oxidative stress (acute illness, drugs, fava beans)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

diagnosis of G6PD deficiency

A

G6PD biochemical assay (months after event)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

treatment of G6PD deficiency

A

avoid triggers, RBC transfusion

17
Q

AutoImmune Hemolytic Anemia (AIHA) types

A

IgG mediated (warm) & IgM mediated (cold)

18
Q

Warm AIHA

A

IgG binds to RBC at body temp, splenic macrophages activated by Fc receptor binding. Nibbling results in microspherocytes

19
Q

where is clearance of warm AIHA cells?

A

spleen

20
Q

Cold AIHA

A

IgM binds to antigens below body temp, attracts and binds complement to RBC, after which the cells are cleared by Kuppfer cells.

21
Q

where is clearance of cold AIHA cells?

A

liver

22
Q

what happens to cold AIHA RBCs at room temp?

A

agglutination

23
Q

diagnostic test for AIHA

A

Direct Coombs test (incubate RBCs with reagent that binds complement or IgG)

24
Q

common causes of warm AIHA

A

autoimmune disorders, lymphoproliferative diseases

25
Q

common causes of cold AIHA

A

infection (EBV), lymphoproliferative diseases

26
Q

treatment for warm AIHA

A

corticosteroids (downregulate Fc receptors on macrophages), splenectomy

27
Q

treatment for cold AIHA

A

avoid cold, treat underlying infection, rituximab, plasma exchange

28
Q

drug induced immune hemolytic anemia

A

clinical presentation like warm AIHA but history of drug exposure. treat via drug withdrawal

29
Q

three potential mechanisms of drug induced hemolytic anemia

A

hapten mediated (drug serves as bound antigen), neo-antigen mediated (drug causes conformational change in cell that become antigenic), alteration of antigen without binding

30
Q

microangiopathy (MAHA)

A

some sort of issue in vessel that causes destruction of RBCs.

31
Q

hallmark finding of microangiopathy

A

schistocytes in blood smear

32
Q

causes of microangiopathy

A

TTP, HUS, super hypertension, vasculitis, DIC

33
Q

paroxysmal nocturnal hemoglobinuria (PNH)

A

acquired somatic mutation in PIG-A, which causes deficiency of CD55 & CD59 GPI anchoring proteins. RBC becomes very susceptible to complement mediated intravascular hemolysis

34
Q

diagnosis of PNH

A

flow cytometry of CD55/59 in neutrophils or monocytes (NOT IN RBCs)

35
Q

treatment of PNH

A

iron/folate supplementation, blood transfusion, eculizumab (C5 inhibitor)

36
Q

clinical manifestations of PNH

A

increased risk of venous thrombosis and aplastic anemia

37
Q

hemolysis due to infection

A

toxins destroy membrane, infestation causes RES clearance, antigen mimicry