immunoproliferative small intestinal disease（malignant phase）の1例

Immunoproliferative Small Intestinal Disease, Report of a Case

要旨　immunoproliferative small intestinal diseaseと考えられる症例を経験した．患者は54歳，女性．1年前より下痢，体重減少が出現し，低蛋白血症を指摘され入院．著明な低蛋白血症を認め，蛋白漏出性胃腸症を証明した．消化管X線，内視鏡検査では小腸皺襞の肥厚と胃から上部小腸にかけてびまん性に微細顆粒状粘膜を認めた．生検で大細胞型悪性リンパ腫と診断された．CT上腹腔内リンパ節の腫大をみた．血中，腸液中にα鎖蛋白は認められなかった。化学療法を施行したところ一時的に下痢，低蛋白血症は軽快し，X線，内視鏡像の改善をみた．

　A 54-year-old woman was admitted to our hospital because of diarrhea and hypoproteinemia. On admission, she was thin. Ascites and peripheral edema were evident. Lymphadenopathy or clubbed finger was not found. Laboratory data revealed marked hypoproteinemia (3.3 g/dl). ¹³¹I-RISA excretion test and α₁-antitrypsin clearance suggested protein-losing gastroenter-opathy. α-heavy chain protein was not detected in her serum or jejunal juice.

　A barium meal study showed thickening of Kerckring's folds in the duodenum and jejunum. Double contrast studies of the stomach, duodenum, and jejunum revealed that the mucosa had a fine granular pattern diffusely. Endoscopic examinations also revealed innumerable small elevations and several erosions in the stomach, duodenum, and jejunum. Endoscopic biopsy specimens obtained from the jejunum, duodenum, and stomach showed dense infiltration of lymphoma cells, diagnosed as malignant lymphoma of diffuse large cell type. Enlargement of the mesenteric lymph nodes was detected by ultrasonography and computed tomography. She was treated with a course of chemotherapy, which was effective temporarily.

　These findings suggest that our case should be regarded as immunoproliferative small intestinal disease in the malignant phase. This is the fifth case with immunoproliferative small intestinal disease reported in Japan where this disorder seems to be extremely rare.