Iriuchishima Hirono, Ogawa Yoshiyuki, Hoshino Takumi, Yoshida Takatomo, Sato Ken, Takagi Hitoshi, Toyama Kotaro, Tsukamoto Norifumi, Jinbo Takahiro
Department of Internal Medicine, Fujioka Public Hospital.
Rinsho Ketsueki. 2007 Jun;48(6):505-9.
A 68-year-old woman was admitted to our hospital with severe ascites, hepatomegaly and hypereosinophilia. We initially suspected Budd-Chiari Syndrome (BCS), but that was ruled out after confirming the presence of no obstruction in the major veins. A molecular biologic examination proved the clonality of the eosinophils and she was therefore diagnosed as having chronic eosinophilic leukemia (CEL). The pathologic findings of a liver biopsy showed dilation of the sinusoids with infiltration of eosinophils, portal eosinophilic infiltrations with fibrosis, and biliary damage. These findings thus suggested infiltration of the liver by the CEL. A relationship between myeloproliferative disorders and BCS has been commonly reported, however there have so far been very few reports which describe the pathology of CEL liver infiltrates. As a result, the present case in which CEL occurred while demonstrating symptoms and findings similar to BCS is therefore considered to be extremely rare. Further accumulation of such cases should therefore be carried out in the future.
一名68岁女性因严重腹水、肝肿大和嗜酸性粒细胞增多症入住我院。我们最初怀疑是布加综合征（BCS），但在确认主要静脉无梗阻后排除了该诊断。分子生物学检查证实嗜酸性粒细胞具有克隆性，因此她被诊断为慢性嗜酸性粒细胞白血病（CEL）。肝活检的病理结果显示肝血窦扩张，伴有嗜酸性粒细胞浸润、门静脉嗜酸性粒细胞浸润伴纤维化以及胆管损伤。这些结果提示CEL浸润肝脏。骨髓增殖性疾病与BCS之间的关系已有普遍报道，然而，迄今为止，很少有报告描述CEL肝脏浸润的病理学情况。因此，本病例中CEL出现时伴有与BCS相似的症状和表现，被认为极为罕见。今后应进一步积累此类病例。
