SYSTEMIC MASTOCYTOSIS - A RARE CAUSE OF NONCIRRHOTIC PORTAL-HYPERTENSION SIMULATING AUTOIMMUNE CHOLANGITIS - REPORT OF 4 CASES

Four patients with systemic mastocytosis, two men and two women, are p resented. Three of them (patients 1, 2, and 4) developed portal hypert ension and ascites without histological evidence of cirrhosis in liver biopsy, The remaining patient (patient 3) had severe bone lesions wit h multiple vertebral fractures, None of the patients had skin or lymph node involvement, Two patients (patients 1 and 2) died 12 and 9 month s after diagnosis with acute nonlymphocytic leukemia and overt mastocy tic leukemia, respectively, while time other true (patients 3 and 4) a re alive 58 and 14 months after diagnosis, Treatment with hydroxyurea or cytosine arabinoside had not any beneficial effect in two patients, while a substantial amelioration of back pain had been obtained by lo cal irradiation and recombinant human interferon-alpha-2b administrati on in one patient (patient 3), All patients had laboratory findings co mpatible with autoimmune cholangitis, We concluded that systemic masto cytosis is a rare cause of noncirrhotic portal hypertention often simu lating autoimmune cholangitis and leading tea the erroneous diagnosis of liver cirrhosis. Diagnosis is based on the presence of mast cells i n Giemsa-stained lives histological sections, and it may be confirmed by immunohistochemical detection of tryptase in the cytoplasm of these abnormally proliferating cells, (C) 1998 by Am. Coll. of Gastroentero logy.