Platelet Diseases Flashcards
Pinpoint (<3 mm) hemorrhagic spots (purple, red)
Petechiae
Hemorrhage od blood into small areas of skin.
Purpura
Appear first as Red; Later turns Purple; Finally to Brown
Form of purpura which blood escapes into large areas of skin, not affecting deep tissues.
Ecchymosis
Swelling in tissues with blood
Hematoma
A stool with dark red or black blood
Melena
Ehler’s Danlos syndrome
“EH Di PaSa”
Increased vascular fragility
Scurvy
defect in Collagen synthesis due to vitamin c defeciency
Hereditary hemorrhagic telangiectasia
(Rendu-Osler-Weber syndrome)
Most common Inherited Vascular Disorder
Vascular malformations and surface skin lesions
Hemangioma-thrombocytopenia syndrome
(Kasabach-Merritt syndrome)
Associated with tumors composed of blood vessels that swell in the surface
Henoch’s-Schonlein Syndrome
Allergic purpura
Purpura in the GIT and Joint
Henoch’s - GIT; Schonlein - Joint (knees & ankles)
von Willebrand’s Disease
lack vWF
Bernard-Soulier Syndrome
Giant Platelet Syndrome
lacks GPIb
LARGEST platelet
Glanzmann thrombasthenia
lacks GPIIbIIIa
Afibrinogenemia
Abesence of fibrinogen
Giant Platelets
Bernard-Soulier Syndrome
May-Heglin anomaly
Gray Platelet
Alport Syndrome
Gray Platelet
Alpha granule deficiency
Dense granule deficiency
Wiskott-Aldrich Syndrome
Hermansky-Pudlak Syndrome
Chediak-higashi Syndrome
Wiskott-Aldrich Syndrome
Smallest platelet with dense granule deficiency
Triad: Thrombocytopenia, Recurrent infections, Eczemia
Triad - Hermansky-Pudlak Syndrome
Albinsm
Ceroid-like pigment in macrophages
Bleeding
Chediak-Higashi Syndrome
Giant lysosomal granules
Triad: Albinism, Recurrent infections, Giant lysosomes
Disorders of Thrombosis
Disorders of Thrombosis
Primary
* Anti-thrombin III deficiency
* Protein S, and C deficiency
* Fibrinolytic System Disorders
* Dystfibrinogenemia
* Homocystinuria
Secondary
* Lupus anticoagulant
Associated to thrombosis due to prostacyclin inhibition
Lupus anticoagulant
due to anti-phospholipid antibody
Lupus anticoagulant
Most common cause of prolonged aPTT
Common coagulopathy in Askenazi Jews
Hemophilia C
Factor XI deficiency
Hemophilia C
* Other name:
* Factor deficiency:
* Common:
* Inheritance:
Hemaphilia C
* Other name: Rosenthal Disease
* Factor deficiency: Fcator XI
* Common: Ashkenazi jews
* Inheritance: Autosomal recessive
Hemophilia B
* Other name:
* Factor deficiency:
* Inheritance:
Hemophilia B
* Other name: Christmas Disease
* Factor deficiency: Factor IX
* Inheritance: X linked recessive
Hemophilia A
* Other name:
* Factor deficiency:
* Inheritance:
Hemophilia A
* Other name: Classical hemophilia/Royal’s Disease
* Factor deficiency: Factor VIII:C
* Inheritance: X-linked recissive
Most Frequently encountered Hereditary Coagulopathy
Von Willebrand’s Disease
Initial workup - VWD
CBC
aPTT
PT
Owren’s Diseases
a.k.a Parahemophilia
Factor V deficiency
Consumptive Coagulation
Disseminated Intravascular Coagulation
Excess consumption of fibrinogen, thrombin and platelet consumption
Lab Findings - DIC
* Increased:
* Decreased:
* Prolonged:
Lab Findings - DIC
* Increased: D-dimer
* Decreased: Platelet count, Fibrinogen (<220 mg/dL)
* Prolonged: APTT, PT, TT
Primary Fibrinolysis
Degradation of Fibrinogen (Fibrenogenolysis)
Secondary Fibrinolysis
Degredation of Fibrin (Fibrinolysis)
Primary Fibrinolysis
* Fibrin monomer:
* Stable clot:
* Protamine Sulfate test:
* Euglobulin Lysis test:
* D-dimer:
Primary Fibrinolysis
* Fibrin monomer: None
* Stable clot: None
* Protamine Sulfate test: Negative
* Euglobulin Lysis test: Shortened
* D-dimer: Negative
Secondary Fibrinolysis
* Fibrin monomer:
* Stable clot:
* Protamine Sulfate test:
* Euglobulin Lysis test:
* D-dimer:
Secondary Fibrinolysis
* Fibrin monomer: Present
* Stable clot: Present
* Protamine Sulfate test: Positive
* Euglobulin Lysis test: Normal/S. decreased
* D-dimer: Positive
Congenital Bleeding disorders
VWD
Hemophilia A
Hemoohilia B
Qualitative platelet disorders
