Hemostasis

Question 1

Acute ITP

Answer 1

arises in children after a viral infection or immunization

Self limited

Question 2

Bernard-Soulier syndrome is

Answer 2

Genetic GP-1b deficiency impairing platelet adhesion

Question 3

Blood smear in Bernard-Soulier shows

Answer 3

Enlarged platelets

Question 4

Children with E coli think

Answer 4

HUS

Question 5

Chronic ITP

Answer 5

arises in adults, usually women of childbearing age

May cause short-lived thrombocytopenia in offsprings

Question 6

Clinical features of disorder of primary hemostasis

Answer 6

1. Mucosal and skin bleeding
2. Epistaxis
3. Hemoptysis
4. GI beeding
5. Intracranial bleedin occurs with severe thrombocytopenia

Question 7

Clinical findings in HUS and TTP

Answer 7

Microangiopathic hemolytic anemia

Renal insufficiency (HUS)

CNS abnormalities (TTP)

Question 8

DIC is secondary to

Answer 8

1. Obsteric complications: Tissue thromboplastin in Amniotic fluid
2. Sepsis
3. Adenocarcinoma
4. Acute promyelocytic leukemia
5. Rattlesnake bite

Question 9

Ecchymoses is

Answer 9

>3 mm of skin bleeding

Question 10

Glanzmann thrombasthenia is due to

Answer 10

A genetic GP-2b/3a deficiency impairing platelet aggregation

Question 11

Hemarthrosis

Answer 11

Deep tissue bleeding into muscles and joints

Question 12

Hemophilia A is due to

Answer 12

X-linked ressive deficiency in Factor 8

Question 13

Hemophilia B a.k.a.

Answer 13

Christmas disease

Question 14

Hemophilia B is a deficiency of

Answer 14

Factor 9

Question 15

How do you test vWF deficiency

Answer 15

Ristocetin test that induces platelet aggregation by causing vWF to bind platelet GP-1b

Question 16

How do you treat vWF deficiency

Answer 16

Desmopressin

Question 17

Immune thrombocytopenia purpura (ITP) is

Answer 17

Autoimmune production of IgG against GP-2b/3a

Question 18

Lab finding in Hemophilia A

Answer 18

Increase PTT

Decrease F8

Normal platelet count and BT

Question 19

Lab findings in DIC

Answer 19

1. Decrease platelet count
2. Increase PT and PTT
3. Decrease fibrinogen
4. Microangiopathic hemolytic anemia
5. Elevated D-dimer

Question 20

Lab findings in HUS and TTP

Answer 20

1. Thrombocytopenia with increase BT
2. Normal PT/PTT
3. Anemia with schistocytes
4. Increase megakaryocytes on BM biopsy

Question 21

Lab findings in ITP

Answer 21

1. Decrease platelet count (Destroyed by speen)
2. Norman PT/PTT
3. Increase megakaryocytes on BM biopsy

Question 22

Lab findings in vWF deficiency

Answer 22

Increase BT

Increas PTT

Question 23

Microangiopathic Hemolytic Anemia (MAHA) is

Answer 23

A pathologic formation of platelet microthrombi in small vessles

RBCs are sheared when they cross microthrombi resulting in hemolytic anemia with schistocytes

Question 24

Petechiae is

Answer 24

1-2 mm of skin bleeding

Signs of thryombocytopenia and not usually seen with qualitative disorders

Question 25

Platelets bind vWF using

Answer 25

GP-1b

Question 26

Purpura is

Answer 26

\> 1 cm skin bleeding

Question 27

Thrombocytopenic purpura (**TTP**) is due to

Answer 27

Decreased **ADAMTS13** an enzyme that cleaves vWF for degradation

Question 28

Von Willebrand disease is

Answer 28

AD Genetic vWF deficiency Causes quantitative and qualitative defects

Question 29

What are thormbi characterized by

Answer 29

**Line of Zahn**: alternating layers of platelets/fibrin and RBC

Question 30

What inactivates plasmin

Answer 30

α2-antiplasmin

Question 31

What promotes exposure of GP-2b/3a

Answer 31

ADP released from platelet dense granules