hemaped

Question 1

causes of microcytic anemia

Answer 1

(TAILS) Thalassemia; Anemia of chronic dse; Iron deficiency; Lead poisoning; sideroblastic anemia

Question 2

causes of normocytic anemia

Answer 2

anemia of chronic dse; uremia; hypothyroidism; bone marrow failure(aplastic anemia)

Question 3

causes of macrocytic anemia

Answer 3

folate deficiency; vit B12 deficiency; drug and alcohol-induced anemia

Question 4

anemia caused by failure or autoimmune destruction of myeloid stem cells

Answer 4

aplastic anemia

Question 5

viruses that cause aplastic anemia

Answer 5

parvovirus B19; hepatitis viruses; HIV; Epstein-Barr virus

Question 6

causes of aplastic anemia

Answer 6

idiopathic; viruses; alklylating and antimetabolite agents; chloramphenicol; insecticides; benzene; radiation

Question 7

typical peripheral blodd smear findings in aplastic anemia

Answer 7

hypocellularity and pancytopenia

Question 8

aplastic anemia with microcephaly; microphthalmia; hearing loss; limb anomalies(absent radii and thumbs)

Answer 8

Fanconi’s anemia

Question 9

aka Diamond-Blackfan syndrome or congenital hypoplastic anemia

Answer 9

pure red cell aplasia

Question 10

absence of erythrocyte precursor cells on bone marrow aspirate

Answer 10

pure red cell aplasia

Question 11

most important sign in iron deficiency anemia

Answer 11

pallor

Question 12

clinical manifestations in iron deficiency anemia

Answer 12

asymptomatic; lethargic; irritable; pale; easily fatigued; high output cardiac failure; koilonychia(spoon nails); pica

Question 13

low retic count; microcytic; hypochromic RBC’s; high RDW; low serum iron and ferritin; high TIBC

Answer 13

Iron deficiency anemia

Question 14

due to decrease in alpha-globulin chain synthesis; dec retic count; microcytic; hypochromic; normal RDW; taget cells; Heinz bodies on PBS

Answer 14

alpha thalassemia

Question 15

no beta-globin production; anemia at 6mos; splenomegaly;extramedullary hematopoiesis

Answer 15

Beta-thalassemia major(Cooley’s anemia)

Question 16

asymptomatic carriers of beta-thalassemia gene; mild or no anemia

Answer 16

Beta-thalassemia minor

Question 17

definitive diagnosis of thalassemias

Answer 17

Hb electrophoresis

Question 18

etiologies of hemolytic anemias

Answer 18

intrinsic RBC defects; autoimmune destruction; intravascular destruction

Question 19

defect in membrane spectrin or ankyrin

Answer 19

spherocytosis

Question 20

confirmatory test for spherocytosis

Answer 20

osmotic fragility test

Question 21

curative treatment to spherocytosis

Answer 21

splenectomy

Question 22

lack of enzyme needed by RBC to defend against oxidative stress leading to RBC destruction

Answer 22

G6PD deficiency

Question 23

typical PBS finding in G6PD deficiency

Answer 23

Heinz cells; blister cells; bite cells; ghost cells (implying intravascular hemolytic anemia)

Question 24

triggers of G6PD deficiency

Answer 24

FPAINNS Fava beans; Primaquine; ASA; Isoniazid; NSAIDS; Nitrofurantoin; Sulfa drugs

Question 25

episodes of painful crisis due to hypoxic tissue injury from microvascular occlusions

Answer 25

sickle cell dse

Question 26

radiologic findings in sickle cell dse

Answer 26

new bone formation on the external aspect of the skull (Crew cut or Hair on end appearanceon the skull radiographs)

Question 27

definitive diagnosis in sickle cell dse

Answer 27

HbS on Hb electrophoresis

Question 28

autoimmune platelet destruction; most common cause of thrombocytopenic purpura in children

Answer 28

ITP

Question 29

five cardinal symptoms of TTP

Answer 29

FAT RN Fever; Anemia; Thrombocytopenia; Renal dysfunction; Nervous system damages

Question 30

due to an acquired deficiency of a metaloproteinase (ADAMTS 13) that is responsible for cleaving the high molecular weight multimers of vWf

Answer 30

TTP

Question 31

X-linked recessive deficiencies of Factor VIII or Factor IX

Answer 31

Hemophilia

Question 32

Dx of hemophilia

Answer 32

normal BT PC and PT; prolonged PTT; decreased factor levels

Question 33

most common inherited bleeding disorder

Answer 33

von Willebrand disease

Question 34

treatment of mild bleeding in type I vWd

Answer 34

Desmopressin(causes release of vWf from endothelial stores)

Question 35

Tx for severe vWd

Answer 35

Factor VIII concentrates which contain high vWf Ag