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蕈样肉芽肿与慢性髓性白血病合并症。附病例报告

[A combination of mycosis fungoides and chronic myeloid leukemia. Apropos of a case].

作者信息

Sigal Nahum M, Rostoker G, Gaulier A, Urbajtel M, Raphael M, Bouzamondo A, Sigal S

机构信息

Service de Dermatologie, Hôpital d'Argenteuil.

出版信息

Ann Dermatol Venereol. 1988;115(2):159-66.

PMID:3260764
Abstract

The coexistence of a T-cell lymphoma with a myelodysplatic syndrome seems to be exceptional. In the case reported here the diagnostic problems raised by the appearance of cutaneous nodules in a patient with chronic myeloid leukaemia (CML) were solved by histo-immunological examinations. A 70-year old male patient had been presenting since 1976 with a psoriasis-like skin disease. He was first seen at the Argenteuil hospital in 1984. Physical examination showed psoriasiform finger-like erythemato-squamous lesions, infiltrated plaques and an ulcerated tumoral swelling of the right elbow. A diagnosis of mycosis fungoides was made on histological and immunological examination results. At histology, this epidermotropic lymphoma was peculiar in that the atypical infiltrate was clearly centred on vessels. Electron microscopy confirmed that the vascular walls were invaded by the mycosis cells. Additional examinations showed hyperleucocytosis and myelaemia which were rapidly attributed to a chronic myelocytic leukaemia since the Philadelphia chromosome was present and the leucocytes had a low alkaline phosphatase score. Bone marrow biopsy disclosed a myeloproliferative syndrome of the CML type. Biopsy of a right axillary lymph node showed myelocytic infiltration associated with dermopathic lymphadenitis. There were no circulating Sezary cells, and a search for extension proved negative. From May, 1984 to June, 1985 the patient's CML was treated with busulfan which produced blood and bone marrow remission. The skin lesions were treated first with mechlorethamine, then with topical corticosteroids. Superficial electron therapy was applied to the tumoral lesions.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

T 细胞淋巴瘤与骨髓增生异常综合征并存似乎很罕见。在本文报道的病例中,组织免疫检查解决了一名慢性髓性白血病(CML)患者出现皮肤结节所引发的诊断问题。一名 70 岁男性患者自 1976 年起患有银屑病样皮肤病。1984 年他首次在阿让特伊医院就诊。体格检查显示有银屑病样指状红斑鳞屑性病变、浸润性斑块以及右肘部溃疡肿瘤性肿胀。根据组织学和免疫检查结果诊断为蕈样肉芽肿。组织学上,这种亲表皮性淋巴瘤的特殊之处在于非典型浸润明显以血管为中心。电子显微镜证实蕈样细胞侵犯血管壁。进一步检查显示白细胞增多和髓细胞血症,由于存在费城染色体且白细胞碱性磷酸酶评分低,很快归因于慢性髓细胞白血病。骨髓活检显示为 CML 型骨髓增殖综合征。右腋窝淋巴结活检显示髓细胞浸润伴皮肤型淋巴结炎。无循环 Sezary 细胞,检查有无扩散结果为阴性。1984 年 5 月至 1985 年 6 月,患者的 CML 用白消安治疗,使血液和骨髓缓解。皮肤病变先用氮芥治疗,然后用局部皮质类固醇治疗。对肿瘤病变进行了浅层电子治疗。(摘要截短于 250 字)

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