Hemophagocytic syndrome and fulminant hepatic failure

Year 2005, Volume: 4 Issue: 3, 190 - 192, 01.12.2005

Osman Z. Şahin Fatih Tekin Zeki Karasu Deniz Nart Yücel Batur

Abstract

Hemophagocytic syndrome is a clinical manifestation of hemophagocytosis that is characterized by high fever, hepatosplenomegaly, and pancytopenia. Infections, hematological malignancies and connective tissue diseases are the main etiological factors of hemophagocytic syndrome. In this article, we present a case with fulminant hepatic failure associated with hemophagocytic syndrome.

Keywords

Hemophagocytic syndrome, hemophagocytosis, fulminant hepatic failure

Hemofagositik sendrom ve fulminan hepatit

Abstract

Hemofagositik sendrom; makrofajların kan hücrelerini fagosite etmesiyle karakterize ve ateş, hepatosplenomegali, pansitopeni gibi bulgularla kendini gösteren klinik bir tablodur. Hemofagositik sendromun etyolojisinde başlıca enfeksiyonlar, hematolojik maliniteler ve bağ doku hastalı kları yer almaktadır. Bu yazıda hemofagositik sendrom ve fulminan hepatit birlikteliği gösteren bir olgu sunulmaktadır.

Keywords

Hemofagositik sendrom, hemofagositoz, fulminan hepatit

References

• Henter JI, Elinder G, Ost A, abd FHL Study Group of Histiocytic Society. Diagnostic guidelines for hemophagocytic lymphohistiocytosis. Semin Oncol 1991; 18: 29-33.
• Oren H, Gulen H, Ucar C, et al. Successful treatment of infection associated hemophagocytic syndrome with intravenous immunglobulin. Turk J Haematol 2003; 20(2): 95-9.
• Parizhskaya M, Reyes J and Jaffe R. Hemophagocytic syndrome presenting as acute hepatic failure in two infants: Clinical overlap with neonatal hemochromatosis. Pediatric and Developmental Pathology 1999; 2: 360-6.
• Su IJ, Wang CH, Cheng AL, et al. Hemophagocytic syndrome in Ebstein-Barr virus associated T-lymphoproliferative disorders: Disease spectrum, pathogenesis, and management. Leuk Lymphoma 1995; 19: 401-6.
• Hisano C, Nakamura M, Mayumi T, et al. A case of hemophagocytic syndrome manifesting adult Still’s disease and acute hepatitis. Nihon Rinsho Meneki Gakkai Kaishi. 1995; 18(2): 256-64.
• King PD, Diaz-Arias AA, et al. Reactive hemophagocytic syndrome simulating acute hepatitis. A case due to hepatic peripheral T-cell lymphoma. J Clin Gastroenterol 1994; 19(3): 234-7.
• Faurschou M, Nielsen OJ, Hansen PB, et al. Fatal virus-associated hemophagocytic syndrome associated with coexistent chronic active hepatitis B and acute hepatitis C virus infection. American Journal of Hematology 1999; 61: 135-8.
• Wu CS, Chang KY, Dunn P, et al. Acute hepatitis A with coexistent hepatitis C virus infection presenting as a virus-associated hemophagocytic syndrome: a case report. Am J Gastroenterol 1998; 93(7): 1187-8.
• Watanabe M, Shibuya A, Okuno J, et al. Hepatitis A virus infection associated with hemophagocytic syndrome: report of two cases. Intern Med 2002; 41(12): 1188-92.
• 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.
• Dofoureg-Lagelouse R, Jabado N, Le Diest F, et al. Linkage of familial hemophagocytic lymphohistiocytosis to 10q21-22 and evidence for heterogeneity. Am J Hum Genet 1999; 64: 172-9.
• Z. Karasu, M. Kilic, S. Cagirgan, et al. Hemophagocytic syndrome after living-related liver transplantation. Transplantation Proceedings, 2003; 35: 1482-4.
• Gritta J, Shinsaku I, Goran EI, et al. Hematol Oncol Clin North Am 1998; 12: 435.
• Fujiwara F, Hibi S, Imashuku S, et al. Hypercytokinemia in hemophagocytic syndrome. Am J Pediatr Hematol Oncol 1993; 15: 92-8.
• Henter JI, Arico M, Elinder G, et al. Familial hemophagocytic lymphohistiocytosis. Primary hemophagocytic lymphohistiocytosis. Hematol Oncol Clin North Am 1998; 12: 417-33.