Takahashi K, Naito M, Matsuoka Y, Takatsuki K
Second Department of Pathology, Kumamoto University Medical School, Japan.
Pathol Res Pract. 1988 Nov;183(6):717-23. doi: 10.1016/S0344-0338(88)80057-8.
An autopsy case of alpha-chain disease (ACD) clinically manifesting generalized lymph node swelling, slight splenomegaly and long-standing ichthyosiform skin eruptions, was reported. Autopsy revealed systemic superficial and profound lymph node swelling and slight splenomegaly, but little or no tumorous lesion in any part of the alimentary tract or pulmonary tissue. The histologic picture of the lymph nodes showed a diffuse monomorphic plasmocytic lymphoma, and there was tumor cell infiltration in the spleen and bone marrow. Immunohistochemistry demonstrated that the tumor cells contained IgA devoid of light chains, i.e. ACD protein. Immunoelectron microscopy revealed that this abnormal immunoglobulin was localized in the rough endoplasmic reticulum and perinuclear space. Persistent chronic inflammation with infiltration mainly of helper-inducer T cells were found in the skin and dermatopathic lymphadenopathy was confirmed in the lymph node biopsies. From these peculiar clinicopathological features, this case is considered to be a previously unknown form of ACD.
报告了一例α链病（ACD）尸检病例，该病例临床表现为全身淋巴结肿大、轻度脾肿大和长期存在的鱼鳞病样皮疹。尸检发现全身浅表和深部淋巴结肿大以及轻度脾肿大，但在消化道或肺组织的任何部位几乎没有或没有肿瘤性病变。淋巴结的组织学表现为弥漫性单形性浆细胞淋巴瘤，脾脏和骨髓有肿瘤细胞浸润。免疫组织化学显示肿瘤细胞含有不含轻链的IgA，即ACD蛋白。免疫电子显微镜显示这种异常免疫球蛋白定位于粗面内质网和核周间隙。在皮肤中发现主要由辅助诱导性T细胞浸润的持续性慢性炎症，并在淋巴结活检中证实有皮肤性淋巴结病。根据这些独特的临床病理特征，该病例被认为是一种先前未知的ACD形式。
