All of the following are inherited platelet function disorders Except:
Answer is D (Weber-Christian disease) Weber Christian Disease is not an inherited disorder of platelet function. Weber Christian Disease is a relapsing febrile nodular non suppurative panniculitis characterized by multiple recurrent subcutaneous nodules, with accompanying fever. The laboratory abnormalities include elevated ESR, Anemia, Leucopenia or leucocvtosis, depression of complements and evidence of circulating immune complexes. Classification of congenital disorder of platelet function: 1. Defects in platelet-vessel wall interaction (disorders of adhesion) (a) von Willebrand disease (deficiency or defect in plasma vWF) (b) Bernard-Soulier syndrome (deficiency or defect in GPIb) 2. Defects in platelet-platelet interaction (disorders of aggregation) (a) Congenital alibrinogenemia (deficiency of plasma fibrinogen) (b) Glanzmann thrombosthenia (deficiency or defect in GPIlb-Ilia) 3. Disorders of platelet secretion and signal transduction (a) Storage pool deficiency (b) Quebec platelet disorder (c) Chediak Higashi syndrome (d) Gray Platelet syndrome (e) Wiskott-Aldrich syndrome 4. Disorders of platelet coagulation - protein interaction (a) Defect in facor Va-Xa interaction on platelets (Scott syndrome)
Bernard Soulier syndrome
Glanzman Thrombasthenia
Wiskott Aldrich Syndrome
Weber-Christian disease