Hematology

Question 1

Results frim failure or autoimmune deatruction of myeloid stem cells–> PANCYTOPENI

Answer 1

Aplastic Anemia

Question 2

Aplastic anemia with microcephaly, microphthalmia, hearing loss and limb anomalies

Answer 2

Fanconi’s Anemia

Question 3

Congenital hypoplastic anemia in neonates or infants having renal congenital anomalies, macrocytic anemia and reticulocytopenia?

Answer 3

Pure Red Cell Aplasia or Diamond-Blackfab Syndrome

Question 4

Most common hematologic disease of infancy and childhood?

Answer 4

Iron Deficiency Anemia

Question 5

What period of growing infant do we start seeing IDA?

Answer 5

Start of complementary feeding

Question 6

For how long should iron supplementation be given after Hemoglobin normalizes?

Answer 6

4-5 months

Question 7

2 differentials for microcytic, hypochromic anemia in children?

Answer 7

Iron Deficiency Anmeia

Thalassemias

Question 8

Target cells and Heinz bodies on PBS?

Answer 8

Alpha Thalassemia

Question 9

Presents with anemia at 6months, splenomegaly and extrapulmonary hematopoiesis?

Answer 9

Beta-thalassemia major/ Cooley’s Anemia

Question 10

Test to detect presence of Thalassemia?

Answer 10

Hgb Electrophoresis

Question 11

Bacteria to look out for during course of Thalassemia that can eat up free iron?

Answer 11

Yersinia enterocolitica

Question 12

Normocytic, normochromic RBC’s with inc retic ct is characteristic of?

Answer 12

Hemolytic Anemia

Question 13

Defect in membrane spectrin or ankyrin decreasing membrane-volume ratio causing loss of biconcave shape and being more fragile?

Answer 13

Spherocytosis

Question 14

Dse that lacks enzyme needed by RBC to defend itself from oxidative stress?

Answer 14

G6PD Deficiency prevents conversion of NADP to NADPH

Question 15

(+) Ghost cells

Answer 15

G6PD deficiency

Question 16

Radiologic findings in sickle cell disease?

Answer 16

Crew-cut or hair on the end appearance on the skull

Question 17

Presents of episodes of painful crisis due to hypoxic tissue injuryfrom micro vascular lesions?

Answer 17

Sickle Cell Anemia

Question 18

Most common cause of thrombocytopenic purpura in childhood?

Answer 18

Idiopathic Thrombocytopenic Purpur

Question 19

3 year old px came in with history of resolved viral infection 2-3 weeks ago came in with thrombocytopenia and increased megakaryocytes and PE showed brusing and petechial rashes; what condition?

Answer 19

ITP

Question 20

Patient came in with fever, anemia, thrombocytopenia, renal dysfunction and changes jn CNS symptoms?

Answer 20

Thrombotic Thrombocytopenic Purpura

Due to def in metallopreoteinaae ADAMTS-13 that is resp for cleaving vWF promting clot formation

Question 21

Treatment for:
ITP?
TTP?

Answer 21

Platelet transfusion and Prednisone for severe bleeding cases
Plasmapheresis

Question 22

X-linked bleeding disorders with severe hemarthrosis, hematoma and blood exam presenting with:
Normal BT, PC and PT
Prolonged PTT
Dec factor levels

Answer 22

Hemophilia (VIII and IX)

Question 23

Most common inherited bleeding disorder?

Prolonged BT and PTT; Normal PT

Answer 23

vWF Disorder (carrier for F VIII for platelet adhesion)

Question 24

Given in mild cases of vWF disease?

Answer 24

Desmopressin to release vWF from endothelial stores