Abstract
We report two cases of hemophagocytic syndrome (HPS), a rare but fatal complication after living-donor liver transplantation (LDLT). Despite their recovery from pancytopenia following treatment with steroid pulse therapy, granulocyte stimulating factor, and intravenous γ-globulin, both patients died. The outcomes reported in cases published in English are devastating, with only 4 survivors among the total 14 patients including ours. Pancytopenia is frequently recognized postoperatively in liver transplant recipients, although its cause is difficult to establish. When pancytopenia accompanying persistent high fever is recognized in LDLT recipients, HPS should be suspected and bone marrow aspiration performed as promptly as possible because of the poor prognosis of this syndrome. There is still no optimal treatment for HPS after liver transplantation.
Similar content being viewed by others
References
Henter JI, Elinder G, Ost A. Diagnostic guidelines for hemophagocytic lymphohistiocytosis. The FHL Study Group of the Histiocyte Society. Semin Oncol 1991;18:29–33.
Imashuku S. Advances in the management of hemophagocytic lymphohistiocytosis. Int J Hematol 2000;72:1–11.
Liver Cancer Study Group of Japan. General rules for the clinical and pathological study of primary liver cancer, 2nd English ed. Tokyo: Kanehara; 2003.
Egawa H, Teramukai S, Haga H, Tanabe M, Fukushima M, Shimazu M. Present status of ABO-incompatible living donor liver transplantation in Japan. Hepatology 2008;47:143–152.
Yoshimura N, Okajima H, Ushigome H, Sakamoto S, Fujiki M, Okamoto M. Current status of organ transplantation in Japan and worldwide. Surg Today 2010;40:514–525.
Chisuwa H, Hashikura Y, Nakazawa, Kamijo T, Nakazawa K, Nakayama J, et al. Fatal hemophagocytic syndrome after livingrelated liver transplantation: a report of two cases. Transplantation 2001;72:1843–1846.
George TI, Jeng M, Berquist W, Cherry AM, Link MP, et al. Epstein-Barr virus-associated peripheral T-cell lymphoma and hemophagocytic syndrome arising after liver transplantation: case report and review of the literature. Pediatr Blood Cancer 2005;44:270–276.
Karasu Z, Kilic M, Cagirgan S, Lebe E, Yilmaz F, Demirbas T, et al. Hemophagocytic syndrome after living-related liver transplantation. Transplant Proc 2003;35:1482–1484.
Taniai N, Akimaru K, Kawano Y, Mizuguchi Y, Shimizu T, Takahashi T, et al. Hemophagocytic syndrome after living-donor liver transplantation for fulminant liver failure: a case report. Hepatogastroenterology 2005;52:923–926.
Akamatsu N, Sugawara Y, Tamura S, Matsui Y, Hasegawa K, Imamura H, et al. Hemophagocytic syndrome after adult-to-adult living donor liver transplantation. Transplant Proc 2006;38:1425–1428.
Yoshizumi T, Taketomi A, Kayashima H, Harada N, Uchiyama H, Yamashita Y, et al. Successful treatment for a patient with hemophagocytic syndrome after a small-for-size graft liver transplantation. Hepatogastroenterology 2008;55:359–362.
Lladó L, Figueras J, Comí S, Torras J, Serrano T, Castellote J, et al. Haemophagocytic syndrome after liver transplantation in adults. Transpl Int 2004;17:221–223.
Hardikar W, Pang K, Al-Hebbi H, Curtis N, Couper R. Successful treatment of cytomegalovirus-associated haemophagocytic syndrome following paediatric orthotopic liver transplantation. J Paediatr Child Health 2006;42:389–391.
Dharancy S, Crombe V, Copin MC, Boleslawski E, Bocket L, Declerck N, et al. Fatal hemophagocytic syndrome related to human herpesvirus-6 reinfection following liver transplantation: a case report. Transplant Proc 2008;40:3791–3793.
Risdall RJ, McKenna RW, Nesbit ME, Krivit W, Balfour HH Jr, Simmons RL, et al. Virus-associated hemophagocytic syndrome: a benign histiocytic proliferation distinct from malignant histiocytosis. Cancer 1979;44:993–1002.
Sakai T, Shiraki K, Deguchi M, Itoh N, Konishi T, Takase K, et al. Hepatocellular carcinoma associated with hemophagocytic syndrome. Hepatogastroenterology 2001;48:1464–1466.
Hansmann ML, Rontogianni D, Jankaschaub GE, Wacker HH, Hinkeldey K, Radzun HJ. Familial hemophagocytic lymphohistiocytosis macrophages showing immunohistochemical properties of activated macrophages and T-accessory cells. Pediatr Hematol Oncol 1989;6:237–246.
Henter JI, Elinder G, Soder O, Hansson M Andersson B, Andersson U. Hypercytokinemia in familial hemophagocytic lymphohistiocytosis. Blood 1991;78:2918–2922.
Imashuku S. Differential diagnosis of hemophagocytic syndrome: underlying disorders and selection of the most effective treatment. Int J Hematol 1997;66:135–151.
Koduri PR, Carandang G, DeMarais P, Patel AR. Hyperferritinemia in reactive hemophagocytic syndrome report of four adult cases. Am J Hematol 1995;49:247–249.
Imashuku S, Hibi S, Sako M, Ishida Y, Mugishima H, Chen J, et al. Soluble interleukin-2 receptor: a useful prognostic factor for patients with hemophagocytic lymphohistiocytosis. Blood 1995;86;4706–4707.
Billiau AD, Roskams T, Van Damme-Lombaerts R, Matthys P, Wouters C. Macrophage activation syndrome: characteristic findings on liver biopsy illustrating the key role of activated, IFN-gamma-producing lymphocytes and IL-6- and TNF-alphaproducing macrophages. Blood 2005;105:1648–1651.
Hibi S, Ikushima S, Fujiwara F, Hashida T, Tsunamoto K, Todo S, et al. Serum and urine beta-2-microglobulin in hemophagocytic syndrome. Cancer 1995;75:1700–1705.
Henzan T, Nagafuji K, Tsukamoto H, Miyamoto T, Gondo H, Imashuku S, et al. Success with infliximab in treating refractory hemophagocytic lymphohistiocytosis. Am J Hematol 2006;81:59–61.
Azuma E, Tabata N, Shibata T, Komada Y, Ito M, Atsumi S, et al. G-CSF enhanced granulocyte production in a child with severe neutropenia in the setting of fatal virus-associated hemophagocytic syndrome. Am J Hematol 1990;35:266–268.
Gill DS, Spencer A, Cobcroft RG. High-dose gamma-globulin therapy in the reactive haemophagocytic syndrome. Br J Haematol 1994;88:204–206.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Soyama, A., Eguchi, S., Takatsuki, M. et al. Hemophagocytic syndrome after liver transplantation: Report of two cases. Surg Today 41, 1524–1530 (2011). https://doi.org/10.1007/s00595-010-4512-9
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00595-010-4512-9