Anemia 3

Question 1

What are the three classification schemes for hemolytic anemia?

Answer 1

Site of RBC destruction
Acquired vs congenital
MOA RBC desctruction

Question 2

What are the signs/symptoms of hemolytic anemia, apart from the usual anemia symptoms?

Answer 2

Jaundice
Urine discoloration
Gallstones
Splenomegaly

Question 3

What is the preferential site of RBC destruction in hemolytic anemia? What does this cause?

Answer 3

Spleen

Splenomegaly

Question 4

What happens to haptoglobin in hemolysis? Why? How does this differ between intra and extravascular causes?

Answer 4

Decreased–bound to Hgb

No difference

Question 5

What happens to LDH (lactate dehydrogenase) in hemolytic anemia? Why?How does this differ between intra and extravascular causes?

Answer 5

Elevated since this enzyme is found in RBCs, and is spilt out

No difference

Question 6

What happens to the reticulocyte count with hemoyltic anemia?

Answer 6

Elevated (>100,000 suggest a hyperproliferative bone marrow)

Question 7

What happens to the indirect bilirubin in hemolytic anemia?

Answer 7

Elevated

Question 8

What happens to urine hemosiderin levels in hemolysis? How does this differ between intra and extravascular causes?

Answer 8

In intravascular ONLY, increased

Question 9

Is LDH specific to hemolytic anemia?

Answer 9

Yes

Question 10

What is the Coombs test result in hemolytic anemias?

Answer 10

Positive

Question 11

What is the mode of inheritance for G6Pd deficiency?

Answer 11

X linked

Question 12

What is the most common enzymatic deficiency that leads to hemolytic anemia?

Answer 12

G6PD deficiency

Question 13

What are the findings on a PBS with G6PD deficiency?

Answer 13

Heinz bodies

Bite cells

Question 14

What are the Heinz bodies seen in G6PD deficiencies?

Answer 14

chunks of denatured Hgb

Question 15

What food should pts with G6PD deficiencies avoid at all costs?

Answer 15

Fava beans

Question 16

What is the MOA of hemolytic anemia d/t pyruvate kinase deficiency?

Answer 16

Loss of structural proteins since there is a lack of ATP

Question 17

What is the major difference between G6PD deficiency and pyruvate kinase deficiency?

Answer 17

Pyruvate kinase deficiency is not susceptible to increased oxidate stress

Question 18

Are there periphearl blood smear abnormalities with pyruvate kinase deficiency?

Answer 18

No

Question 19

What is the usual presentation of pyruvate kianse deficiency?

Answer 19

neonatal jaundice

Question 20

True or false: sickle cell syndrome/disease usually has some degree of hemolytic anemia

Answer 20

True

Question 21

Pts with sickle cell anemia are susceptible to what infections?

Answer 21

By encapsualted bacteria and salmonella

Question 22

What causes the recurrent renal problems in pts with HbS?

Answer 22

recurrent infarcts

Question 23

What are the symptoms of sickle cell trait?

Answer 23

Usually asymptomatic, but can have painless hematuria

Question 24

What is Hgb SC disease?

Answer 24

Both HbS and HbC, causing rigid (but not sickled) Hgb

Question 25

Why is a splenectomy used as a treatment for heriitary sphereocytosis?

Answer 25

Because spleen will capture any cells that are not correctly shaped

Question 26

What are the two major complications of heriditary spherocytosis?

Answer 26

Splenomegaly Cholelithiasis both from dsetruction of RBCs

Question 27

Is the spleen normally palpable?

Answer 27

No

Question 28

What is the cause of paroxysmal nocturnal hmoglobinuria?

Answer 28

Defective PIG-A gene

Question 29

What is the triad for paroxysmal nocturnal hemoglobinuria?

Answer 29

Hemolysis Pancytopenia Thromboses

Question 30

How do you diagnose PNH?

Answer 30

Specific flwo cytometry assays for CD55 and CD59

Question 31

What is the treatment for PNH?

Answer 31

corticosteroids | Anticoagulants

Question 32

What two antibody types usually cause hemolytic anemia?

Answer 32

IgG or IgM

Question 33

What is the test to check for autoimmune hemolytic anemia?

Answer 33

coombs test

Question 34

What is the treatment for autoimmune hemolytic anemia?

Answer 34

Corticosteroids

Question 35

Abs that react with RBC membrane proteins are of what type? What if they react to polysaccharides?

Answer 35

RBC proteins = IgG (warm) Poly = IgM (cold)

Question 36

What abx type is associated with hemolysis?

Answer 36

PCNs | Cephalosporins