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[白塞病先于慢性髓系白血病出现发病]

[Behçet's disease with an onset prior to the appearance of chronic myeloid leukemia].

作者信息

Tassies D, Cervantes F, Feliu E, Cabal G, Martínez Orozco F, Rozman C

机构信息

Escuela de Hematología Farreras Valentí, Hospital Clínic i Provincial, Barcelona.

出版信息

Med Clin (Barc). 1992 Jun 6;99(2):67-8.

PMID:1630184
Abstract

The case of a Ph-positive female patient with chronic myeloid leukemia (CML) is reported. The patient presented a cutaneous-mucous picture prior to the appearance of the hemopathy consisting of genitals ulcers, buccal aphthae and nodular cutaneous lesions the study of which demonstrated panniculitis. The lesions improved with the administration of low doses of prednisone and colchicine. The CML evolved to a blastic crisis of a monocytic phenotype at 14 months of diagnosis leading to death of the patient. The cutaneous-mucous picture was catalogued as Becçet disease (BD) according to the criteria of the International Study Group for Behçet Disease. Given the lack of serologic tests or pathognomonic histologic lesions the difficulty in the diagnosis of BD is commented upon and the differential diagnosis of this disease, particularly with respect to the Sweet syndrome, is discussed.

摘要

报告了1例Ph阳性慢性髓性白血病(CML)女性患者的病例。该患者在血液病出现之前呈现出皮肤黏膜症状,包括生殖器溃疡、口腔阿弗他溃疡和结节性皮肤病变,对这些病变的研究显示为脂膜炎。给予低剂量泼尼松和秋水仙碱后病变有所改善。CML在诊断后14个月演变为单核细胞表型的原始细胞危象,导致患者死亡。根据国际白塞病研究组的标准,该皮肤黏膜症状被归类为白塞病(BD)。鉴于缺乏血清学检查或特征性组织学病变,讨论了BD诊断的困难以及该疾病的鉴别诊断,特别是与Sweet综合征的鉴别。

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