Management of Thrombotic Thrombocytopenic Purpura

In this article, we will discuss the Management of Thrombotic Thrombocytopenic Purpura. So, let’s get started.

Management

Plasmapheresis coupled with fresh frozen plasma infusion: Until recently, the disease was considered to be universally fatal but now with the availability of plasmapheresis, more than 90% of cases can survive if therapy is immediately instituted. The plasmapheresis can be done daily or even twice-daily with plasma replacement. The response is judged by an increase in platelet count, fall in LDH levels and fragmented red cells. If response is obtained, plasmapheresis is continued but less frequently for several weeks to months.

Role of corticosteroids and antiplatelet agents: The efficacy of this treatment is not known.

Immunomodulatory therapies with rituximab, immunosuppression by vincristine or cyclophosphamide and splenectomy: In case of relapse following initial treatment, plasmapheresis should be reinstituted. If ineffective, or in case with primary refractoriness, second line treatment may be considered including rituximab, steroids, IVIG, vincristine, cyclophosphamide and splenectomy.

Platelet transfusions should not be given because they will act as ‘fuel to fire’ and can precipitate thrombotic events.

Care of comatose patient

General supportive measures with I.V. fluids and oxygen must be given to treat hypoxia.

Hemodialysis should be considered for patient with significant renal impairment.

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