Hemolytic Anemias

Question 1

What is haptoglobin and what happens to it in hemolytic anemias

Answer 1

A protein that binds hemoglobin, so its level decreases in hemolytic anemias

Question 2

What happens to LDH levels in hemolytic anemia

Answer 2

Increases due to LDH release from lysed RBCs

Question 3

Genetics of sickle cell

Answer 3

autosomal recessive

Question 4

What conditions cause sickle crisis

Answer 4

acidosis, low temp, hypoxia, dehydration, infection

Question 5

Which infectious agent common causes aplastic crisis in sickle cell patients

Answer 5

B19 parvovirus

Question 6

What bone infection is common in asplenia sickle cell patients

Answer 6

Salmonella osteomylelitis

Question 7

What is treatment for sickle cell crises?

Answer 7

Hydration
Morphine
keep patient warm
O2
(hydroxyurea can also be used to increase HbF)

Question 8

Genetics of Hereditary Spherocytosis

Answer 8

autosomal dominant

Question 9

Test to Dx hereditary spherocytosis

Answer 9

Osmotic Fragility test in hypotonic saline

Question 10

Treatment for Hereditary Spherocytosis

Answer 10

Splenectomy (prophylaxis)

Question 11

Genetics of G6PD def.

Answer 11

X-linked recessive

Question 12

Precipitating drugs of G6PD def.

Answer 12

Sulfonamides
nitrofurantoin
primaquine
fava beans
infection

Question 13

Dx of G6PDH def.

Answer 13

Heinz bodies on smear or “Bite Cells” on smear

Question 14

Tx for G6PD def.

Answer 14

avoid drugs that cause it
hydration
RBC transfusion when necessary

Question 15

What Abs are present in warm autoimmune hemolytic anemia (AIHA) vs. cold AIHA?

Answer 15

Warm: both IgG and IgM
Cold: only IgM

Question 16

What is the pathophys of warm vs cold AIHA?

Answer 16

Warm: mainly IgG binds surface RBC proteins causing lysis in the spleen (extravascular)

Cold: IgM mainly binds surface RBC proteins at colder temps (like fingers and toes) causes intravascular hemolysis in the liver

Question 17

How can the direct coombs test differ the two AIHAs?

Answer 17

Warm: test shows IgG binding
Cold: test shows only complement binding

Question 18

Treatment for AIHA

Answer 18

usually self limiting and no treatment necessary

-can do glucocorticoids or splenectomy in warm if needed

Question 19

Pathology of Paroxysmal Nocturnal Hemoglobinuria (PNH)

Answer 19

deficiency of anchor proteins preventing complement from reacting to RBCs, WBCs, and platelets

result can be a pancytopenia

Question 20

Treatment for PNH

Answer 20

glucocorticoids

in an aplastic crisis a bone marrow transport may be needed