MUST READ HEMATOLOGY Flashcards
Most common inherited vascular disorder;
Walls of the affected blood vessels are thin and lacks smooth muscles
Hereditary hemorrhagic telangectasia or
osler-weber-rendu disease
Disorder associated with tumors composed of blood vessels that commonly swell and bleed at the surface
Congenital hemangiomata/
Kasabach-merritt syndrome
Increased vascular fragility, autosomal dominant disorder
Ehlers-Danlos syndrome/
Marfan’s syndrome
Connective tissue elastic fibers in small arteries are calcified and structurally abnormal
Pseudoxanthoma elasticum
LOSS of ELASTICITY OF THE SKIN;
Decreased production of collagen and elastin;
Bruised areas commonly found on the forearms of elderly persons
Senile purpura
Deficiency of ASCORBIC ACID;
Acquired defect in the SYNTHESIS OF COLLAGEN and HYALURONIC ACID
Scurvy
Immunologic damage to endothelial cells
Henoch-Schonlein purpura
Hermansky-Pudlak syndrome
A. Alpha granule deficiency
B. Dense granule deficiency
B. Dense granule deficiency
Chediak-Higashi Syndrome
A. Alpha granule deficiency
B. Dense granule deficiency
B. Dense granule deficiency
Gray platelet syndrome:
A. Alpha granule deficiency
B. Dense granule deficiency
A. Alpha granule deficiency
Wiskott- Aldrich syndrome
A. Alpha granule deficiency
B. Dense granule deficiency
B. Dense granule deficiency
Platelet aggregation studies:
Normal ECA decreased Ristocetin
Inherited as both an Autosomal dominant types (Ia, IIA and IIB) and an autosomal recessive trait (IIC and III)
A. Glanzmann’s thrombasthenia
B. Von Willebrand’s disease (vWD)
C. Bernard-Soulier Syndrome
B. Von Willebrand’s disease (vWD)
Giant platelets or largest platelet seen
A. Glanzmann’s thrombasthenia
B. Von Willebrand’s disease (vWD)
C. Bernard-Soulier Syndrome
C. Bernard-Soulier Syndrome
Platelet aggregation studies:
Normal ECA
Decreased Ristocetin
Platelet aggregation studies:
Defective ECA
Normal response with Ristocetin
Decrease or absence of gp2b3a complex
A. Glanzmann’s thrombasthenia
B. Von Willebrand’s disease (vWD)
C. Bernard-Soulier Syndrome
A. Glanzmann’s thrombasthenia
(Essential Arthrombia)
Major site of inhibition
Endothelium and platelet
