Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome and adult onset Still's disease: case report and review of the literature

Modern Rheumatology
Mehmet SayarliogluMehmet Ali Ucar

Abstract

Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP/HUS) is a multisystem disorder characterized by consumptive thrombocytopenia, microangiopathic hemolytic anemia, neurologic symptoms, renal function abnormalities, and fever. Coexistence of TTP/HUS and adult onset Still's disease (ASD) is extremely rare. We report the case of a 46-year-old woman who presented with fever, arthritis, myalgias, petechia on skin and confusion five years after the onset of ASD. Thrombocytopenia, renal failure, marked elevation lactate dehydrogenase, and red cell fragmentation on peripheral blood smear were observed. We made a diagnosis of TTP/HUS associated with ASD, according to physical examination and characteristic laboratory data. She recovered from the TTP/HUS following daily sessions of therapeutic plasma exchange with fresh frozen plasma replacement and glucocorticoid therapy. Awareness of the possible development of TTP/HUS in ASD is important for early diagnosis and treatment.

References

Aug 1, 1987·Kidney International·G Remuzzi
Feb 1, 1987·Arthritis and Rheumatism·J J CushL A Cooperstein
Jul 1, 1997·Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association·J PortolésJ Blanco
Aug 23, 2002·The New England Journal of Medicine·Joel L Moake
Feb 27, 2003·Southern Medical Journal·Martin G V Perez, Francis R Rodwig
Jun 14, 2003·Arquivos de neuro-psiquiatria·Renan B DominguesMaria Carmen S Santos
May 5, 2006·The New England Journal of Medicine·James N George

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Citations

Jan 18, 2014·Clinical Rheumatology·Petros EfthimiouBella Mehta
Nov 6, 2009·Wiener klinische Wochenschrift·Lea SalamonJadranka Morovic-Vergles
Sep 15, 2011·Case Reports in Medicine·João Tadeu Damian Souto FilhoGustavo Fernandes Ribas

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