Patho-RBC and Bleeding Disorders

Question 1

Erythropoeitin level in hemolytic anemias

Answer 1

Increased

Question 2

Most common cause of IDA overall?

Answer 2

GI bleeding

Question 3

Intrinsic hemolysis is caused by

Answer 3

Intrinsic defects in rbc

Question 4

How do u differentiate extravascular from intravascular hemolysis?

Answer 4

Extravascular hemolysis has splenomegaly

Question 5

What is the expected morphology on peripheral blood smear of hemolytic anemias?

Answer 5

Normicytic normochromic

Question 6

Hereditary spherocytosis is brought about by what mutations

Answer 6

Ankyrin
Spectrin
Band 3
Band 4.2

Question 7

In a patient with hereditary spherocytosis, infection with parvovirus b19 can cause?

Infection with EBV?

Answer 7

Parvo - aplastic crisis

EBV- hemolytic crisis

Question 8

Small dark nuclear remnants present in RBCs of asplenic patients

Answer 8

Howell-jolly bodies

Question 9

G6pd is the rate limiting step in which biochemical pathway?

Answer 9

Pentose phosphate pathway

Question 10

What is the function of G6PD

Answer 10

Reduces NADP to NADPH; provides reducing equivalents

Question 11

Morphology G6PD deficiency

Answer 11

Heinz bodies

Bite cells

Question 12

What are heinz bodies?

Answer 12

Membrane bound precipitates on denatured globin chains

Question 13

Mutation in sickle cell anemia

Answer 13

Point mutation in 6th codon of beta globin

Leads to

Replacement of glutamate with VALINE

Question 14

Dehydrated RBCs with bulls eye apoearance seen in sickle cell disease

Answer 14

Codocytes

Question 15

Why are infants with sickle cell disease asymptomatic until 2-4 mos of age?

Answer 15

Due to high fetal hemoglobin F

Question 16

Crises in Sickle cell anemia

Answer 16

Vasoocclusive crisis
Sequestration crisis
Aplastic crisis

Question 17

Most common manifestation of vasoocclusive crisis in sickle cell anemia?

Answer 17

Hand foot syndrome

Inflammation of finger and toe

Question 18

Massive entrapment of sickle cells leads to rapid splenic enlargement, hypovolemia, and shock

Answer 18

Sequestration crisis

Question 19

Renal manifestation of sickle cell anemia

Answer 19

Hyposthenuria

Inability to concentrate urine

Question 20

Signs of extramedullary hematopoieisis in sickle cell

Answer 20

Crew cut skull

Prominent cheekbones

Question 21

What is the best initial diagnostic test for sickle cell anemia?

Answer 21

PBS

Not used in newborns

Question 22

What is the drug of choice for sickle cell anemia and what is its mechanism of action?

Answer 22

Hydroxyurea (increases HbF)

Question 23

Diagnostic gold standard for sickle cell anemia

Answer 23

Hemoglobin electrophoresis