Dubin-Johnson症候群に特発性門脈圧亢進症を合併した1例―特徴的な色素顆粒の分布について―

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  • A CASE OF DUBIN-JOHNSON SYNDROME ASSOCIATED WITH IDIOPATHIC PORTAL HYPERTENSION -ITS CHARACTERISTIC CHANGES IN BOTH LAPAROSCOPIC AND HISTOLOGICAL APPEARANCES

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A case of Dubin-Johnson Syndrome associated with Idiopathic Portal Hypertension was reported, emphasizing charcteristic changes of both laparoscopic and histological appearances. A 43 year old woman was admitted because of general lassitude. Her serum transaminase values were within normal range, but hyperbilirubinemia and the characteristic pigmentation of the liver were present, and an extended bromsulphalein (BSP) test for 120 minutes showed a late re-rise curve. Besides, hepatosplenomegaly, pancytopenia, and portal hypertension without any cirrhotic patterns were recognized. So, she was diagnosed as a case of Dubin-Johnson Syndrome and Idiopathic Portal Hypertension, which was considered remarkably different from the ordinal Dubin-Johnson Syndrome in the following findings. Laparoscopically, the liver surface showed light black color and big rippling wavelike appearance with scattered small depressions. Histologically abundant Dubin-Johnson granules were observed in liver cells not only centrilobular but also in periportal area having dense fibers.

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