TTP Without Fragmented Erythrocytes

9/13/00 (Doyle / Obideen)

Group: Wednesday Residents

Presenting Resident (Followup on 9/20/00): M.K. Obideen

RE: A 27 year old Mexican immigrant with 9 days of fever and 1 day of right quadrant pain.

Question: Can TTP occur without observable fragmented red cells in the peripheral smear? 

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Unique Identifier: 96185182

Authors: Brilliant SE. Lester PA. Ohno AK. Carlon MJ. Davis BJ. Cushner HM.

Institution: Department of Medicine, Madigan Army Medical Center, Tacoma, WA 98431-5000, USA.

Abstract: We report the case of an 18-year old man with hemolytic-uremic syndrome (HUS) having a classic clinical presentation and diagnostic renal pathology without evidence of microangiopathic hemolytic anemia (MAHA) by peripheral blood smear. Indirect evidence of hemolysis was suggested by mild anemia, elevation of serum lactate dehydrogenase, and examination of the patient's bone marrow. We postulate that in this case the inability to detect schistocytes in the peripheral smear reflected a low degree of hemolysis. Review of the literature revealed that evidence of fragmented erythrocytes by peripheral smear is not always present in HUS, yet this observation has received little attention. Thus, the diagnosis of HUS need not include overt evidence of MAHA as is traditionally taught.

Link Directly to Fulltext article in Ovid

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[Link Directly to Fulltext Article in OVID]

Unique Identifier: 95252135

Authors: Fava S. Galizia AC.

Institution: Department of Medicine, St Luke's Hospital, G'Mangia, Malta.

Abstract: Thrombotic thrombocytopenic purpura is an uncommon disorder that if left untreated has a very high mortality. Schistocytes are generally considered essential for the diagnosis. A patient is presented with a thrombotic thrombocytopenic purpura-like syndrome in whom schistocytes were persistently absent and who responded to plasmapheresis.

Link Directly to Fulltext Article at Publisher

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Unique Identifier: 99043724

Authors: George JN. Gilcher RO. Smith JW. Chandler L. Duvall D. Ellis C.

Institution: Oklahoma Blood Institute, Department of Medicine, University of Oklahoma Health Sciences Center, Oklahoma City 73190, USA. Jim-George@OUHSC.edu

Abstract: Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) is a clinical syndrome defined by the presence of thrombocytopenia and microangiopathic hemolytic anemia without a clinically apparent etiology. Patients may also have multiple other symptoms and signs including neurologic and renal abnormalities and fever. In the era prior to effective therapy with plasma exchange, most patients developed multisystem abnormalities and the syndrome was more easily recognized. Now, since there is urgency to begin treatment, sufficient diagnostic criteria for TTP-HUS are only thrombocytopenia and microangiopathic hemolytic anemia without a clinically apparent cause; patients may have no neurologic symptoms, renal abnormalities, or fever. This has lead to an apparent increased incidence because of both the increased importance of early recognition and the decreased specificity of the diagnostic criteria. Effective treatment has also revealed new aspects of the clinical course of TTP-HUS following the initial response to plasma exchange treatment: prompt exacerbations, which are common when plasma exchange is diminished in frequency or discontinued, and later relapses, which may occur many years after the initial episode. This review describes the evolution of the syndrome of TTP-HUS in the current era of effective treatment, and describes the management and clinical outcomes among patients treated by the Oklahoma Blood Institute. [References: 33]