True about Thrombocytopenic pupura is?
Ans. is 'c' i.e., Thrombosis in cerebral vessels Thrombotic thromhocytopenic purpura (TTP) o TTP is included among group of diseases called thrombotic microangiopathic which are characterised by widespread thrombosis in microcirculation. o The other disorder which is included in this group include hemolytic uremic syndrome (HUS). o Small vessels like terminal aerioles and capillaries are involved. Pathogenesis of TTP o Patients with TTP are deficient in an enzyme called ADAMTSB.13 also known as vWF metalloprotease that degrades very high molecular weight multitimers of vWF. o In the absence of this enzyme, these multimers of vWF accumulate in plasma and promote platelet macroaggregate formation throughout the microcirculation leading to symptoms of TTP. Clinical features o TTP is characterized by a pentad of : ? 1.Microangiopathic hemolytic anemia --> Because red cells are forced to squeeze through abnormally narrowed small vessels because of thrombi. 2. Thrombocytopenia Due to consuption of platelets in formation of platelet thrombi. 3. Neurological symptoms Due to presence of thrombi in CNS microcirculation. 4. Renal dysfunction --> Due to presence of thrombi in renal microcirculation. 5. Fever
Haemolysis is extravasular
Normal renal function test
Thrombosis in cerebral blood vessels
Immediate cure following plasmapheresis