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华氏巨球蛋白血症。一例以肺部表现为首发症状的尸检病例报告。

Waldenström's macroglobulinemia. Report of an autopsy case presenting with a pulmonary manifestation.

作者信息

Tsuji M, Yoshii Y, Taka T, Min K, Mori H

机构信息

Department of Pathology, Osaka Medical College, Japan.

出版信息

Virchows Arch A Pathol Anat Histopathol. 1989;415(2):169-73. doi: 10.1007/BF00784355.

Abstract

An autopsy case of Waldenström's macroglobulinemia is reported, in whom an abnormal pulmonary shadow had already existed 2 years before the diagnosis of the disease and was proved to be pulmonary involvement. Immunoelectrophoresis demonstrated a monoclonal increase in immunoglobulin M with kappa light chain. A chest X-ray film showed a reticulo-nodular shadow in the right lower lobe of the lung. A bronchial biopsy specimen revealed a diffuse and dense lymphocytic infiltration. Bone marrow aspirate revealed no remarkable change except for a slight increase in plasma cells (1.7%) and an appearance of atypical lymphocytes (0.5%). At autopsy, more than half of the right lower lobe of the lung was occupied by a pale whitish, viscid and glossy tumour mass. Heptosplenomegaly and lymph node enlargement were not observed. Histological findings of the tumour tissue were similar to those of the biopsy specimen. Lymphocytic infiltration was observed also in the liver, kidneys, spleen, bone marrow and lymph nodes, but was of minor degree. Other reported cases of Waldenström's macroglobulinemia accompanied by pulmonary involvement are reviewed.

摘要

报告1例华氏巨球蛋白血症尸检病例,该患者在疾病诊断前2年就已存在异常肺部阴影,后被证实为肺部受累。免疫电泳显示免疫球蛋白M单克隆性升高,伴有κ轻链。胸部X线片显示右下肺有网状结节状阴影。支气管活检标本显示弥漫性致密淋巴细胞浸润。骨髓穿刺除浆细胞略有增多(1.7%)和出现非典型淋巴细胞(0.5%)外,未见明显变化。尸检时,右下肺叶一半以上被灰白色、黏稠且有光泽的肿瘤块占据。未观察到肝脾肿大和淋巴结肿大。肿瘤组织的组织学表现与活检标本相似。在肝脏、肾脏、脾脏、骨髓和淋巴结中也观察到淋巴细胞浸润,但程度较轻。对其他报道的伴有肺部受累的华氏巨球蛋白血症病例进行了综述。

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