Background: Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic microangiopathy characterized by
• Microangiopathic hemolytic anemia,
• Thrombocytopenia, often with purpura but not usually severe bleeding
• Acute renal insufficiency
• Neurologic abnormalities, usually fluctuating
• Fever
Only 20-25% of patients present with the above classical pentad
Recently developed, high probability PLASMIC score supports treatment with immediate plasma exchange where as low PLASMIC score suggests withholding plasma exchange and search for alternative diagnosis.

Aims: Series of Thrombotic thrombocytopenic purpura presentation and course of disease.

Methods: This is a retrospective study conducted in department of clinical haematology in a tertiary hospital in india. we are reporting five cases of TTP , data were retrieved from MRD department, two cases were pregnancy related and other three were sporadic TTP. One case presented with expressive aphasia.

Results: All 5 cases were evaluated, for all cases PLASMIC score was done, all 5 received plasma exchange, 3 cases were successfully treated only with single episode but two cases had recurrence. These reccurences were post rituximab therapy which were successfully treated with ciclosporin . All five cases had ADAMTS13 level estimation, No mortality was seen in our series.

Conclusions: TTP is uncommon and potentially fatal disease if not having high index of suspicion and early plasma exchange, classical Pentad may not be useful for diagnosing. PLASMIC score help us to early initiate the plasma exchange.

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ISTH Congress Abstracts - https://abstracts.isth.org/abstract/varied-presentations-of-ttp/