Refractory thrombotic thrombocytopenic purpura treated successfully with monoclonal antibody (rituximab)

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a nonimmune, microangiopathic hemolytic anemia, associated with thrombocytopenia, fever, neurologic, or renal dysfunction. Plasma exchange (PEX) with or without steroids constitutes first-line therapy in TTP. However, a subset of the patients may be refractory to PEX. Rituximab appears to be an alternative effective therapy for refractory or relapsing TTP. Here, we report a case of TTP in a 43-year-old female presented with fever, generalized weakness, headache, vomiting, and ecchymotic patches over forearms and upper chest for 7 days along with one episode of seizure. The laboratory evaluation revealed severe thrombocytopenia, anemia, and indirect hyperbilirubinemia with peripheral blood smear showing schistocytes (fragmented red blood cells). Initial therapy with multiple PEXs along with parenteral corticosteroids resulted in only minimal improvement of platelet count. Subsequently, rituximab was administered which helped in normalization of platelet count and overall clinical improvement. This case highlights the importance of timely utilization of second-line drugs such as rituximab in refractory TTP.

Keywords

• adamts13 enzyme
• cryo-poor plasma exchange
• microangiopathic hemolytic anemia
• refractory thrombotic thrombocytopenic purpura
• rituximab