Abstract
A case of hemophagocytic syndrome associated with ulcerative colitis is very rare. A 32-year-old man visited the hospital complaining of fever and severe abdominal pain for 7 days. He was diagnosed to have ulcerative colitis 2 years ago and had been treated with sulfasalazine. Three months ago, he had abdominal pain, weight loss, and hematochezia, so prednisolone and mercaptopurine were added to the treatment. On admission, the physical examination showed splenomegaly. Peripheral blood counts revealed pancytopenia, and bone marrow aspirate smears showed many histiocytes with active hemophagocytosis. There was no evidence of viral and bacterial infections and other neoplasms, which were commonly associated with hemophagocytic syndrome. He was successfully treated with high dose steroid. We report this case along with a review of the related literatures.
REFERENCES
1. Wong KF, Chan JK. Reactive hemophagocytic syndrome–a clinicopathologic study of 40 patients in an Oriental population. Am J Med. 1992; 93:177–180.
2. 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.
4. Kanaji S, Okuma K, Tokumitsu Y, Yoshizawa S, Nakamura M, Niho Y. Hemophagocytic syndrome associated with fulminant ulcerative colitis and presumed acute pancreatitis. Am J Gastroenterol. 1998; 93:1956–1959.
5. Koketsu S, Watanabe T, Hori N, Umetani N, Takazawa Y, Nagawa H. Hemophagocytic syndrome caused by fulminant ulcerative colitis and cytomegalovirus infection: report of a case. Dis Colon Rectum. 2004; 47:1250–1253.
6. James DG, Stone CD, Wang HL, Stenson WF. Reactive hemophagocytic syndrome complicating the treatment of inflammatory bowel disease. Inflamm Bowel Dis. 2006; 12:573–580.
7. Reiner AP, Spivak JL. Hematophagic histiocytosis. A report of 23 new patients and a review of the literature. Medicine (Baltimore). 1988; 67:369–388.
8. Scott H, Moynahan EJ, Risdon RA, Harvey BA, Soothill JF. Familial opsonization defect associated with fatal infantile dermatitis, infections, and histiocytosis. Arch Dis Child. 1975; 50:311–317.
9. Hsiao CH, Kao HL, Lin MC, Su IJ. Ulcerative colon T-cell lymphoma: an unusual entity mimicking Crohn's disease and may be associated with fulminant hemophagocytosis. Hepatogastroenterology. 2002; 49:950–954.
10. Dehner LP. Morphologic findings in the histiocytic syndromes. Semin Oncol. 1991; 18:8–17.
11. Drew WL. Laboratory diagnosis of cytomegalovirus infection and disease in immunocompromised patients. Curr Opin Infect Dis. 2007; 20:408–411.
12. Kumakura S, Ishikura H, Umegae N, Yamagata S, Kobayashi S. Autoimmune-associated hemophagocytic syndrome. Am J Med. 1997; 102:113–115.
13. Matsuura T, West GA, Youngman KR, Klein JS, Fiocchi C. Immune activation genes in inflammatory bowel disease. Gastroenterology. 1993; 104:448–458.