QUANTITATIVE and QUALITATIVE PLATELET DISORDERS

Question 1

Generalized bone marrow suppression leads to a decrease in all cell types

Answer 1

Pancytopenia

Question 2

Absent or decreased and abnormal bone marrow megakaryocytes, congenital deformities of the arm

Answer 2

Thrombocytopenia with absent radius (TAR)

Question 3

A space-occupying lesion in the bone marrow such as a metastatic tumor, fibrosis, or leukemia; bone marrow tissue replacement/substitution from a normal cell into an abnormal cell (e.g. tumor)

Answer 3

Myelophthisic process

Question 4

Thrombocytopenia caused by an extensive blood transfusion; the degree of which is directly proportional to the number of units transfused

Answer 4

Dilutional Loss (platelets are diluted due to extensive blood transfusion)

Question 5

Uncontrolled proliferation of platelets, a characteristic of myeloproliferative disorders, PV, essential thrombocythemia, Chronic Granulomatous Leukemia (CGL)

Answer 5

Primary thrombocytosis

Question 6

Which is more sever, Primary or Secondary thrombocytosis

Answer 6

Primary thrombocytosis

Question 7

Enumerate ADHESION DEFECTS

Answer 7

• Bernard-Soulier Syndrome
• Von Willebrand’s disease

Question 8

Platelet lacks GpIb which is necessary for binding vWF; characterized by abnormal bleeding time and giant platelets

Answer 8

Bernard-Soulier Syndrome

Question 9

lack of vWF that also affects factor VIII:C

Answer 9

Von Willebrand’s Disease

Question 10

Enumerate Aggregation defects

Answer 10

• Glanzmann’s thrombasthenia
• Afibrinogenemia

Question 11

lack of GpIIbIIIa

Answer 11

Glanzmann’s thrombasthenia

Question 12

complete absence of fibrinogen

Answer 12

Afibrinogenemia

Question 13

Enumerate storage pool defects

Answer 13

1. Gray platelet syndrome
2. Wiskott-Aldrich Syndrome
3. Hermansky-Pudlak Syndrome
4. Chediak-Higashi Syndrome

Question 14

Characterized by Albinism, Recurrent Infections, Giant Lysosomes and Beta-granule deficiency

Answer 14

Chediak-Higashi Syndrome

Question 15

Characterized by a triad of tyrosinase-positive oculocutaneous albinism, accumulation of ceroid-like pigment in macrophages (fat) and a bleeding tendency; lacks Beta (dense) granules

Answer 15

Hermansly-Pudlak Syndrome

Question 16

Characterized by a triad of thrombocytopenia, recurrent infections, eczema; platelets lack DENSE & ALPHA granules; platelets are SMALL (microthrombocytopenia)

Answer 16

Wiskott-Aldrich Syndrome

Question 17

Alpha granules are missing, platelets are LARGER than normal and appear gray or blue-gray in wright stain

Answer 17

Gray platelet syndrome

Question 18

A very rare autosomal recessive disorder of calcium-induced membrane phospholipid scrambling/phospholipid flip and thrombin generation on platelets.

Answer 18

SCOTT SYNDROME

Question 19

what drug that can inhibit the enzyme cyclooxygenase that causes a decrease of TxA2 which results to decreased platelet aggregation

Answer 19

ASPIRIN

Question 20

the most common drug-induced thrombocytopenia

Answer 20

Heparin-Induced Thrombocytopenia (HIT)

Question 21

In HIT, IgG antibodies are directed to?

Answer 21

Platelet factor 4

Question 22

In APS (antiphospholipid syndrome), IgG antibodies are directed to?

Answer 22

b2 glycoprotein (GP I)

Question 23

Absorption of viral antigen on the platelet surface followed by antibody binding or by formation of an immune complex o the surface of platelets via the platelet Fc (Immunoglobulin) receptors; usually occur in children

Answer 23

Acute Immune Thrombocytopenia Purpura

Question 24

Majority of platelet autoantigens are present on either GPIIb/IIIa or GPIb/IX complex; usually occur in adults

Answer 24

Chronic Immune Thrombocytopenia Purpura