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系统性红斑狼疮伴发恶性组织细胞病

FIRST CASE OF SYSTEMIC LUPUS ERYTHEMATOSUS ASSOCIATED WITH MALIGNANT HISTIOCYTOSIS IN CHINA

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【作者】 钟月娴; 余斌杰; 胡媛芳;

【Author】 Zhong Yuexian et al

【机构】 中山医科大学第一附属医院内科; 中山医科大学病理解剖教研室;

【摘要】 <正> 系统性红斑狼疮为一自身免疫疾病,病因未明,可能为多因性,与病毒、遗传和环境因素等有关。文献报道,本病可与胸腺瘤、卵巢精原细胞瘤、骨髓纤维变性等伴发;而何杰金氏病、淋巴性白血病、急性粒细胞性白血病可在系

【Abstract】 The patient, a 17 year-old boy, was admitted in Sept. 20,1986. Laboratory data revealed anemia, leukocytopenia, thrombopenia, hypergammaglobulinemia and increased ESR. The blood examinations for LE cell, ANA and anti-ds-DNA antibody were positive. Sk(?)n lupus band test was positive. Bone marrow aspirate revealed histiocytosis with the presence of atypical histiocytes, macrophages and lymphoid cells: Lymph node biopsy revealed the destruction of normal architecture and histiocytosis. The clinical and laboratory data, bone marrow picture and lymph node biopsy were compatible with the diagnosis. Thus, the diagnosis was established clinically, There was no improvement after antibiotic and large dose of corticosteroid treatment. The patient died 34 days after admission, Autopy findings confirmed the diagnosis.

  • 【文献出处】 广东医学 ,Guangdong Medical Journal , 编辑部邮箱 ,1988年04期
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