Systemic Lupus Erythematosus Presenting as Thrombotic Thrombocytopenia Purpura

Filipa Silva, Mariana Brandao, Afonso Esteves


Thrombotic thrombocytopenic purpura (TTP) is an uncommon disorder characterized by the classic pentad of microangiopathic hemolytic anemia, thrombocytopenia, renal dysfunction, fever and neurologic abnormalities. It is associated with infections, malignancy, drugs and autoimmune diseases. TTP and systemic lupus erythematosus (SLE) rarely present simultaneously and generally constitute a diagnostic dilemma. We present a case of a 42-year-old patient with overlapping features of SLE and TTP, who was admitted to our hospital with complaints of generalized fatigue, epistaxis and bruises. The workup revealed microangiopathic hemolytic anemia (negative Coombs test with schistocytes), and thrombocytopenia. The presence of clinical and immunological criteria made the diagnosis of SLE possible. Recognition of TTP in the setting of SLE is important, because the combination of both conditions carries a worse prognosis than SLE alone. Prompt treatment with plasmapheresis and aggressive immunosuppressive therapy is life saving.

J Med Cases. 2017;8(1):5-7


Systemic lupus erythematosus; Thrombotic thrombocytopenic purpura; ADAMTS-13; Plasmapheresis; Immunosuppressive therapy

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