Hematology

Question 1

acquired deficiency of metalloproteinase

Answer 1

Thrombotic thrombocytopenic purpura (TTP)

Question 2

Treatment of choice for TTP

Answer 2

Plasmapharesis

Question 3

Autoimmune platelet destruction; most common cause of thrombocytopenic purpura in childhood

Answer 3

Idiopathic thrombocytopenic purpura (ITP)

Question 4

Bruising and petechial rash 1-4 weeks after infection

Answer 4

Idiopathic thrombocytopenic purpura (ITP)

Question 5

Treatment for ITP

Answer 5

None (70% spontaneously resolve)

For severe thrombocytopenia: IVIg, platelet transfusion and prednisone

Question 6

X-linked recessive deficiency of factor VIII

Answer 6

Hemophilia A

Question 7

X-linked recessive deficiency of factor IX

Answer 7

Hemophilia B or Christmas Disease

Question 8

Normal bleeding time

Answer 8

5-8 mins

Question 9

Most common inherited bleeding disorder

Answer 9

Von Willebrand disease

Question 10

Treatment for Von Willebrand Disease

Answer 10

Desmopressin (mild);

Factor VIII concentrate (severe)

Question 11

Increased PTT

Normal PT, BT, plt ct

Answer 11

Hemophilia A and B

Question 12

Increased PT, PTT

Normal PT, plt ct

Answer 12

Von Willebrand’s Disease

Question 13

Increased PT, PTT, BT

Decreased plt ct

Answer 13

Disseminated Intravascular Coagulopathy (DIC)

Question 14

Increased PT, PTT

Normal BT, plt ct

Answer 14

Vitamin K deficiency

Question 15

Normal PT, PTT
Increased BT
Decreased plt ct

Answer 15

Idiopathic thrombocytopenic purpura (ITP)

Question 16

Fever, anemia, thrombocytopenia, renal dysfunction, nervous system changes

Answer 16

Thrombotic thrombocytopenic purpura (TTP)

Question 17

Low Hgb, 0 retic
Normal WBC and platelets
BM: absence of red cell precursors

Answer 17

Transient erythroblastopenia of childhood

Question 18

Normochromic, normocytic
Leucocytosis
Dec Serum Fe
Normal TIBC
Low to normal transferrin
Inc serrm Ferritin
Fe sequestration to macrophages
Blunted response to EPO

Answer 18

Anemia of Chronic Disease

Question 19

Two types of aplastic anemia

Answer 19

Fanconi (congenital) anemia

Acquired aplastic anemia

Question 20

rare autosomal recessive inherited BM failure assoc with cafe au lait, short stature, microcephaly, skeletal, renal & cardiac anomalies

Answer 20

Fanconi anemia

Question 21

Macrocytosis ff by thrombocytopenia
then progressive pancytopenia
terminating in myelodysplastic syndrome

Answer 21

Fanconi anemia

Question 22

Pancytopenia d/t destruction of stem cells by IFN gamma and TNF (also potent inhibitors of progenitor cells)

Answer 22

Acquired immune-mediated aplastic anemia

Question 23

Marked depression or absence of hematopoietic cells with replacement of fatty tissue-containing reticulin cells

Answer 23

Acquired aplastic anemia

Question 24

Granulocyte <500
Platelet <20,000
Retic ct <40,000

Answer 24

Severe aplastic anemia

Question 25

Storage diseases that cause BM infiltration

Answer 25

Gaucher’s
Newmann-Pick
Cystine storage disease

Question 26

Rare pure red cell aplasia in w/c erythroid progenitors and precursors are highly sensitive to death by apoptosis

Answer 26

Diamond-Blackfan syndrome

Question 27

Macrocytic anemia
Low retic
Normocellular BM with selective paucity of eryhteoid precursors
Inc Hgb F, inc eADA

(+) malignant potential
Normal karyotype, 50% had physical anomalies
median age: 2 mos

Answer 27

Diamond-blackfan syndrome

Question 28

2 alpha 2 gamma

Answer 28

Hgb F

Question 29

2 alpha 2 beta/delta

Answer 29

Adult Hgb

Question 30

4 betas

Answer 30

Hgb H

Question 31

4 gamma’s

Answer 31

Bart’s Hgb