Malignant histiocytosis of the intestine

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.