Chronic Relapsing Thrombotic Thrombocytopenic Purpura in Adult Onset Still's Disease

Martin G.V. Perez, MD, Francis R. Rodwig Jr., MD


South Med J. 2003;96(1) 

In This Article

Abstract and Introduction

We report the first known case of chronic relapsing thrombotic thrombocytopenic purpura associated with adult-onset Still's disease. The patient presented with diffuse arthralgias, sore throat, and a maculopapular rash involving the trunk and extremities; she was hospitalized with fever and confusion. Thrombocytopenia, renal failure, and microangiopathic hemolytic anemia developed within several days. After a diagnosis of thrombotic thrombocytopenic purpura was made, she responded well to a series of plasma exchanges. Evaluation for infection, autoimmune disorders, and malignancy was negative. She was discharged to home in good condition, with normal renal function and normal platelet count. Two more episodes of TTP developed 7 and 9 months after the first hospitalization. The diagnosis of adult-onset Still's disease was then determined on the basis of clinical and laboratory criteria. She was successfully treated with plasma exchange, prednisone, and azathioprine. She later had splenectomy and has subsequently been wihout recurrence of thrombotic thrombocytopenic purpura for 2 years.

Adult onset Still's disease (AOSD) is a systemic disorder of uncertain etiology characterized by spiking fever, evanescent rash, arthritis, and involvement of various organs.[1] The diagnosis is one of clinical suspicion, requiring the exclusion of infection, malignancy, and systemic disease.[2] This disease entity is rarely associated with thrombotic thrombocytopenic purpura (TTP), a hematologic disease of many etiologies characterized by thrombocytopenia, microangiopathic hemolytic anemia, fever, neurologic abnormalities, and renal disease.


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