Fax +41 61 306 12 34 E-Mail karger@karger.ch www.karger.com Original Paper Acta Haematol 2006;116:108–113 DOI: 10.1159/000093640 Hemophagocytic Syndrome in Kidney Transplant Recipients: Report of Four Cases from a Single Center Alihan Gurkan Ulkem Yakupoglu Asuman Yavuz Hilmi Dikici Yarkin Kamil Yakupoglu Murat Tuncer Alper Demirbas Fevzi Ersoy Akdeniz University Organ Transplantation Center, Antalya, Turkey Introduction Hemophagocytic syndrome (HPS) is characterized by the inappropriate activation of macrophages or histio- cytes with significant hemophagocytosis both in bone marrow and the reticuloendothelial system [1]. It was first reported as a neoplastic disorder, showing hemophagocy- tosing histiocytes and termed as histiocytic medullary re- ticulosis in 1939, by Scott and Rob-Smith, and then pro- posed by Rappaport as malignant histiocytosis. After the introduction of the term familial hemophagocytic lym- phohistiocytosis (primary HPS) by Farquhar and Claireaux in 1952, Risdall et al. reported 19 patients with HPS secondary to viral infection, thus used the term vi- rus-associated hemophagocytic syndrome (VAHS) (sec- ondary or reactive form of HPS). Soon, it was understood that any kind of microorganism could cause HPS, so the term infection-associated hemophagocytic syndrome (IAHS) was preferred. Reactive HPS can also be accom- panied by lymphoma (lymphoma-associated hemophago- cytic syndrome – LAHS) or autoimmune diseases (auto- immune-associated hemophagocytic syndrome – AAHS) [1–3] . In the light of this information, HPS can be classi- fied as four different types: (a) sporadic; (b) familial form seen in children; (c) associated with acute infection, and (d) associated with malignant disorders, immunodefi- ciency or defective leukocyte functions. This syndrome Key Words Hemophagocytic syndrome  Hemophagocytic syndrome, prognosis  Kidney transplantation Abstract Background: The prognosis of hemophagocytic syn- drome (HPS) in kidney transplant recipients is reported to be poor, however the optimal therapeutic approach is still unclear. Patients and Methods: The clinical and fol- low-up data of the 4 patients with HPS (3 male, 1 female; age 39.7 8 11.3 years) among 368 kidney transplant re- cipients during a 5-year period were retrospectively ana- lyzed. Results: HPS developed 35–61 days in the post- transplant period. All 4 patients presented with fever. Hepatosplenomegaly and lymphadenopathy were ob- served only in the first patient. Laboratory tests revealed pancytopenia and hyperferritinemia in all patients, but elevated liver enzymes were observed in 3. Two patients had cytomegalovirus infection, and 1 had Epstein-Barr virus infection. Three patients died despite aggressive supportive therapy, however the fourth case survived after graft nephrectomy. Conclusion: HPS pathogenesis in kidney transplants appears to be related with the graft itself. Graft nephrectomy may be the preferable thera- peutic approach for kidney transplant recipients with HPS resistant to standard supportive therapy. Copyright © 2006 S. Karger AG, Basel Received: October 19, 2004 Accepted after revision: November 8, 2005 Ulkem Yakupoglu, MD Göztepe Mah Fahrettin Kerim Gökay Cad, Coskun Sok Mazharbey Apt 7/20 34730, Kadıkoy Istanbul (Turkey) Tel. + 90 216 567 3578, Fax + 90 216 469 3796, E-Mail ulkemyakupoglu@yahoo.com © 2006 S. Karger AG, Basel 0001–5792/06/1162–0108$23.50/0 Accessible online at: www.karger.com/aha Downloaded by: Ondokuz Mayis Universitesi 193.140.28.132 - 1/28/2015 1:11:46 PM