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.

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The Donath-Landsteiner (DL) hemolytic anemia or paroxysmal cold hemoglobinuria (PCH) is a rare subtype of autoimmune hemolytic anemia (AIHA). We report here a case of a 22-year-old young female who presented with acute onset shortness of breath. Routine hematological investigation revealed erythrophagocytosis and platelet phagocytosis by monocytes in peripheral blood smear, which are rarely reported in AIHA, especially the DL. The unusual blood smear findings directed the pathologists to initiate workup for hemolytic anemia that yielded positive results in direct Coombs test C3d (Complement) and direct DL test. The findings confirmed the case as DL hemolytic anemia. To the best of our knowledge, this is the first report of both erythrophagocytosis and platelet phagocytosis in peripheral blood smear in PCH.

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