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Two patients with the congenital deficiency of antithrombin El (AT-III) assoiated with superior mesenteric venous thrombosis (SMVT) are described and a possible relevance of the AT-III deficiency to SMVT is discussed. Patient No. 1: A 34 year-old male without a history of thrombosis was found to have SMVT at operation, which was further confirmed histologically. Hematological studies disclosed abnormally low levels of progressive antithrombin activity, heparin-cofactor as well AT-ill antigen. The decreased AT-Ill was also found in his mother, sister and niece. Patient No. 2: The diagnosis of SMVT was established surgically as well as histologically in a 24 year-old male, whose father had died of recurrent SMVT. Abnormally decreased levels at about 50% of normal of progressive antithrombin. heparin-cofactor and AT-ill antigen were also found in this patient and his sister. The familial involvement in their family indicates that the decrease in AT-Ill is hereditary, most probably autosomally transmitted. Besides the decreased level of AT-ill, no abnormal laboratory findings were noted in both patients and their affected family members.

Two patients with the congenital deficiency of antithrombin III associated with superior mesenteric venous thrombosis