Platelet Diseases

Question 1

Pinpoint (<3 mm) hemorrhagic spots (purple, red)

Answer 1

Petechiae

Question 2

Hemorrhage od blood into small areas of skin.

Answer 2

Purpura

Appear first as Red; Later turns Purple; Finally to Brown

Question 3

Form of purpura which blood escapes into large areas of skin, not affecting deep tissues.

Answer 3

Ecchymosis

Question 4

Swelling in tissues with blood

Answer 4

Hematoma

Question 5

A stool with dark red or black blood

Answer 5

Melena

Question 6

Ehler’s Danlos syndrome

“EH Di PaSa”

Answer 6

Increased vascular fragility

Question 7

Scurvy

Answer 7

defect in Collagen synthesis due to vitamin c defeciency

Question 8

Hereditary hemorrhagic telangiectasia

(Rendu-Osler-Weber syndrome)

Answer 8

Most common Inherited Vascular Disorder

Vascular malformations and surface skin lesions

Question 9

Hemangioma-thrombocytopenia syndrome

(Kasabach-Merritt syndrome)

Answer 9

Associated with tumors composed of blood vessels that swell in the surface

Question 10

Henoch’s-Schonlein Syndrome

Allergic purpura

Answer 10

Purpura in the GIT and Joint

Henoch’s - GIT; Schonlein - Joint (knees & ankles)

Question 11

von Willebrand’s Disease

Answer 11

lack vWF

Question 12

Bernard-Soulier Syndrome

Giant Platelet Syndrome

Answer 12

lacks GPIb

LARGEST platelet

Question 13

Glanzmann thrombasthenia

Answer 13

lacks GPIIbIIIa

Question 14

Afibrinogenemia

Answer 14

Abesence of fibrinogen

Question 15

Giant Platelets

Answer 15

Bernard-Soulier Syndrome
May-Heglin anomaly
Gray Platelet
Alport Syndrome

Question 16

Gray Platelet

Answer 16

Alpha granule deficiency

Question 17

Dense granule deficiency

Answer 17

Wiskott-Aldrich Syndrome
Hermansky-Pudlak Syndrome
Chediak-higashi Syndrome

Question 18

Wiskott-Aldrich Syndrome

Answer 18

Smallest platelet with dense granule deficiency

Triad: Thrombocytopenia, Recurrent infections, Eczemia

Question 19

Triad - Hermansky-Pudlak Syndrome

Answer 19

Albinsm
Ceroid-like pigment in macrophages
Bleeding

Question 20

Chediak-Higashi Syndrome

Answer 20

Giant lysosomal granules

Triad: Albinism, Recurrent infections, Giant lysosomes

Question 21

Disorders of Thrombosis

Answer 21

Disorders of Thrombosis
Primary
* Anti-thrombin III deficiency
* Protein S, and C deficiency
* Fibrinolytic System Disorders
* Dystfibrinogenemia
* Homocystinuria
Secondary
* Lupus anticoagulant

Question 22

Associated to thrombosis due to prostacyclin inhibition

Answer 22

Lupus anticoagulant

due to anti-phospholipid antibody

Question 23

Lupus anticoagulant

Answer 23

Most common cause of prolonged aPTT

Question 24

Common coagulopathy in Askenazi Jews

Answer 24

Hemophilia C

Factor XI deficiency

Question 25

**Hemophilia C** * Other name: * Factor deficiency: * Common: * Inheritance:

Answer 25

Hemaphilia C * Other name: Rosenthal Disease * Factor deficiency: Fcator XI * Common: Ashkenazi jews * Inheritance: Autosomal recessive

Question 26

**Hemophilia B** * Other name: * Factor deficiency: * Inheritance:

Answer 26

**Hemophilia B** * Other name: Christmas Disease * Factor deficiency: Factor IX * Inheritance: X linked recessive

Question 27

**Hemophilia A** * Other name: * Factor deficiency: * Inheritance:

Answer 27

**Hemophilia A** * Other name: Classical hemophilia/Royal's Disease * Factor deficiency: Factor VIII:C * Inheritance: X-linked recissive

Question 28

Most Frequently encountered Hereditary Coagulopathy

Answer 28

Von Willebrand's Disease

Question 29

Initial workup - VWD

Answer 29

CBC aPTT PT

Question 30

Owren's Diseases | a.k.a Parahemophilia

Answer 30

Factor V deficiency

Question 31

Consumptive Coagulation

Answer 31

Disseminated Intravascular Coagulation | Excess consumption of fibrinogen, thrombin and platelet consumption

Question 32

Lab Findings - DIC * Increased: * Decreased: * Prolonged:

Answer 32

Lab Findings - DIC * Increased: **D-dimer** * Decreased: Platelet count, **Fibrinogen (<220 mg/dL)** * Prolonged: **APTT, PT, TT**

Question 33

Primary Fibrinolysis

Answer 33

Degradation of Fibrinogen (Fibrenogenolysis)

Question 34

Secondary Fibrinolysis

Answer 34

Degredation of Fibrin (Fibrinolysis)

Question 35

**Primary Fibrinolysis** * Fibrin monomer: * Stable clot: * Protamine Sulfate test: * Euglobulin Lysis test: * D-dimer:

Answer 35

**Primary Fibrinolysis** * Fibrin monomer: **None** * Stable clot: None * Protamine Sulfate test: **Negative** * Euglobulin Lysis test: **Shortened** * D-dimer: Negative

Question 36

**Secondary Fibrinolysis** * Fibrin monomer: * Stable clot: * Protamine Sulfate test: * Euglobulin Lysis test: * D-dimer:

Answer 36

**Secondary Fibrinolysis** * Fibrin monomer: **Present** * Stable clot: Present * Protamine Sulfate test: **Positive** * Euglobulin Lysis test: Normal/S. decreased * D-dimer: **Positive**

Question 37

Congenital Bleeding disorders

Answer 37

VWD Hemophilia A Hemoohilia B Qualitative platelet disorders