Abstract :
Background: TTP is a rare multisystem disease among children due to dysfunction of ADAMTS 13 enzyme in plasma causing defective vWF cleavage, thus giving rise to platelet aggregation, micro thrombi formation, platelet consumption, capillary blockage and finally organ dysfunction leading to a severe life-threatening condition with mortality around 90% if untreated. It can be congenital (5%) or acquired (95%) and Plasmapheresis works wonder for both the subtypes.
Clinical Description : Here we have described a case of acquired TTP masquerading TIA in an 8-year-old girl child who had attacks of sudden onset aphasia and upper limb weakness lasting for few hours with spontaneous recovery. On detailed examination we found pallor and few petechial spots on face and upper limbs with a peripheral blood picture suggestive of thrombocytopenia with microangiopathic hemolytic anemia but a normal renal function. We sent blood for ADAMTS 13 activity which reported a critically low level of ADAMTS 13 enzymatic activity with presence of inhibitors and absent genetic mutation.
Management: Immediate Plasmapheresis was started along with oral prednisolone therapy for a span of one month which resulted both clinical and hematological recovery. Platelet count acted as a good indicator of clinical remission.
Conclusion: A high degree of clinical suspicion is required to make a prompt diagnosis of this life threatening disease (TTP) since this carries a very high mortality rate and can mimic a more common disease HUS.
Keyword :
Plasmapheresis, Petechial, Rituximab