Abstract
Four cases of malignant histiocytosis of the intestine (MHI) are described in detail, with emphasis on the clinical features and laboratory findings that may lead to a diagnosis. MHI is a rapidly progressive, usually fatal condition, which may occur in a setting of adult celiac disease or apparently spontaneously; all patients, however, have villous atrophy and crypt hyperplasia of the jejunum. Characteristic symptoms are malaise, weight loss, abdominal pain, and diarrhea followed, after a period of weeks or months, by acute small intestinal obstruction, perforation, or bleeding. Because of the high mortality associated with MHI, suggestive symptomatology and investigations should, in the absence of a definitive diagnosis, lead to an urgent laparotomy with a view to chemotherapy if the diagnosis is made.
Similar content being viewed by others
References
Gough KR, Read AE, Naish JM: Intestinal reticuloses as a complication of idiopathic steatorrhoea. Gut 3:232–239, 1962
Austad WI, Comes JS, Gough KR, McCarthy CF, Read AE: Steatorrhoea and malignant lymphoma. Am J Dig Dis 12:475–490, 1967
Harris OD, Cooke WT, Thompson H, Waterhouse JAH: Malignancy in adult coeliac disease and idiopathic steatorrhoea. Am J Med 42:899–912, 1967
Freeman HJ, Weinstein WM, Shnitka TK, Piercey JR, Wensel RH: Primary abdominal lymphoma: Presenting manifestation of celiac sprue or complicating dermatitis herpetiformis. Am J Med 63:585–594, 1977
Isaacson P, Wright DH: Intestinal lymphoma associated with malabsorption. Lancet 1:67–70, 1978
Isaacson P, Wright DH: Malignant histiocytosis of the intestine: its relationship to malabsorption and ulcerative jejunitis. Hum Pathol 9:661–677, 1978
Byrne GE, Rappaport H: Malignant histiocytosis. Malignant Diseases of the Haematopoietic System. K. Akazaki (ed). Tokyo, University Park Press, 1973, pp 145–162
Thompson H: Necropsy studies in adult coeliac disease. J Clin Pathol 27:710–712, 1974
Rappaport H, Ramot B, Hulu N, Park JK: The pathology of so-called Mediterranean abdominal lymphoma with malabsorption. Cancer 29:1502–1511, 1972
Glees JP, Thomas M, Redding WH, Hefney M, Gazet JC: Liver biopsy at lymphoma laparotomy. Lancet 1:210–211, 1978
Isaacson P, Wright DH, Judd MA, Mepham PL: Primary gastrointestinal lymphoma: A classification of 66 cases. Cancer 43:1805–1819, 1979
Gordon S, Todd J, Cohn ZA:In vitro synthesis and secretion of lysozyme by mononuclear phagocytes. J Exp Med 139:1228–1248, 1974
Hodges JR, Isaacson P, Eade OE, Wright R: Serum lysozyme levels in malignant histiocytosis of the intestine. Gut 19:A993, 1978
Warnke RA, Kim H, Dorman RF: Malignant histiocytosis (histiocytic medullary reticulosis). Cancer 35:215–230, 1975
Stein RS, Moran EM, Byrne GE: Malignant histiocytosis complete remission with combination therapy. Cancer 38:1083–1086, 1975
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Hodges, J.R., Isaacson, P., Smith, C.L. et al. Malignant histiocytosis of the intestine. Digest Dis Sci 24, 631–638 (1979). https://doi.org/10.1007/BF01333708
Issue Date:
DOI: https://doi.org/10.1007/BF01333708