RBC and Bleeding Disorders

Question 1

Autosomal dominant
Defects in membrane skeleton
Extravascular
Hemolysis
Spherical cells without central pallor
Splenectomy

Answer 1

Hereditary spherocytosis

Question 2

Hereditary spherocytosis is thorugh a defect in membrane skeleton such as

Answer 2

Spectrin

Ankyrin

Question 3

X linked recessive
Decreased NADPH for glutathione reduction
Intravascular and extravascular hemolysis
Heinz bodies
Bite cells

Answer 3

G6PD Deficiency

Question 4

Autosomal recessive
Mutation on the 6th codon of B globin gene
Howell Jolly bodies- nuclear remnants
Target cells
Protective against malaria
Increased susceptibility to encapsulated org.
Treatment: hydroxyurea

Answer 4

Sickle cell anemia

Question 5

What is the mutation of the 6th codon in the B globin gene?

Answer 5

Glutamate ➡️Valine

Question 6

Protective against malaria
Decreased synthesis of glibin chains
Intrinsic extravascular hemolysis
Symptoms at 5-6 months of age
Shift from HbF to HbA synthesis

Answer 6

Beta thalassemia

Question 7

Protective against malaria
Decreased synthesis of glibin chains
Intrinsic extravascular hemolysis
Symptoms at birth
Hemoglobin Barts
More stable

Answer 7

A- thalassemia

Question 8

Most common nutritional disorder in the world

Answer 8

Iron Deficiency Anemia

Question 9

Principal site of iron absorption

Answer 9

Proximal duodenum

Question 10

Serum Fe- dec
Transferrin- inc
Ferritin- dec

IDA or chronic disease?

Answer 10

IDA

Question 11

Serum Fe- dec
Transferrin- dec
Ferritin- inc

IDA or chronic disease?

Answer 11

Anemia of chronic disease

Question 12

Chronic primary hematopoietic failure with pancytopenia
Most common known cause is drugs
Normocytic, normochromic anemia
Hypocellular marrow

Answer 12

Aplastic anemia

Question 13

Prothrombin time measures what pathway of coagulation?

Answer 13

Extrinsic

Question 14

Activated partial thromboplastin time measures what pathway of coagulation?

Answer 14

Intrinsic

Question 15

Childhood
Due to postviral illness
Antibodies against gpIIb-IIIa and gbIb-IX
Increased megakaryocytes
Splenic congestion, large platelets
Self limited

Answer 15

Acute Idiopathic Thrombocytopenic Purpura

Question 16

40 years old
Due to SLE, HIV, CLL
Antibodies against gpIIb-IIIa and gbIb-IX
Increased megakaryocytes
Splenic congestion, large platelets
Glucocorticoid treatment
Splenectomy

Answer 16

Chronic Idiopathic Thrombocytopenic Purpura

Question 17

Microangiopathic hemolytic anemia
ADAMTS13 level is decreased
Thrombocytopenia 
Renal failure 
Neurologic manifestations

Answer 17

Thrombotic thrombocytopenic purpura

Question 18

HUS is caused by

Answer 18

E coli O157:H7

Question 19

Deficiency of gp1b-IX
No platelet adhesion
Thrombocytopenia

Answer 19

Bernard Soulier

Question 20

gpIIb-IIIa deficiency
Prevent platelet aggregation
Normal platelet count

Answer 20

Glanzmann Thrombasthenia

Question 21

Most common inherited bleeding disorder

Answer 21

Von Willebrand disease

Question 22

Autosomal dominant coagulation disorder
Normal platelet count
Impaired platelet function

Answer 22

von Willebrand disease

Question 23

Most common hereditary syndrome associated with life threatening bleeding

Answer 23

Hemophilia

Question 24

X linked recessive coagulation disorder

Platelet count and function normal

Answer 24

Hemophilia

Question 25

Excessive activation of coagulation and the formation of thrombi in the microvasculature leading to consumption of platelets and coagulation factors

Answer 25

DIC