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[与骨髓发育异常和急性嗜酸性粒细胞白血病相关的复发性多软骨炎]

[Relapsing polychondritis associated with myelodysplasia and acute eosinophilic leukemia].

作者信息

Várkonyi J, Jakab L, Szombathy T, Vámos R, Zalatnai A, Nagy P

机构信息

Semmelweis Orvostudományi Egyetem, III.sz.Belgyógyászati Klinika, Budapest.

出版信息

Orv Hetil. 1997 Apr 13;138(15):945-8.

PMID:9173385
Abstract

Authors report a case in which relapsing polychondritis had been diagnosed two years before myelodysplastic syndrome developed and terminated in eosinophilic leukemia. The observation that relapsing polychondritis may precede myelodysplasia is not in concordance with some of the previous reports regarding relapsing polychondritis as a paraneoplastic phenomenon of myelodysplastic syndrome. The terminally developed eosinophilic leukemia is not supposed to be a blastic phase of the underlying myelodysplasia, much rather a second malignant process. This opinion may be confirmed by the early presence of blast cells in the myelodysplastic process without eosinophilia. It seems interesting to note that both our patient and his daughter suffered from diseases of autoimmune origin: acquired vitiligo and subacute cutan lupus erythematodes, respectively.

摘要

作者报告了一例病例,该患者在骨髓增生异常综合征发生并发展为嗜酸性粒细胞白血病之前两年就已被诊断为复发性多软骨炎。复发性多软骨炎可能先于骨髓发育异常出现,这一观察结果与之前一些将复发性多软骨炎视为骨髓增生异常综合征副肿瘤现象的报告不一致。最终发展的嗜酸性粒细胞白血病不应被认为是潜在骨髓发育异常的原始细胞阶段,而更应是第二个恶性过程。骨髓发育异常过程中早期存在原始细胞而无嗜酸性粒细胞增多这一情况可能证实了这一观点。值得注意的是,我们的患者及其女儿分别患有自身免疫性疾病:获得性白癜风和亚急性皮肤型红斑狼疮。

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