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[未分类的慢性骨髓增殖性Ph阴性;i(17q);+8综合征合并混合性髓系-巨核系母细胞危象——病例报告]

[Unclassified chronic myeloproliferative Ph(-); i(17q); +8 syndrome with mixed myelo-megakaryoblastic crisis--case report].

作者信息

Mariańska B, Apel D, Seferyńska I, Maj S

机构信息

Kliniki Hematologicznej Instytutu Hematologii i Transfuzjologii w Warszawie.

出版信息

Acta Haematol Pol. 1996;27(1):85-8.

PMID:8629449
Abstract

We present a case of a 17-year old patient with extreme hepatosplenomegaly, hyperthrombocytosis, hyperleucocytosis and the presence of myelo- and megakaryoblasts in the peripheral blood film. Numerous complications that occurred in the course of the disease made cytostatic treatment difficult. Since Ph chromosome and hybrid gene bcr/abl were absent, the diagnosis of unclassified chronic myeloproliferative syndrome in the phase of blast crisis was established. Immunophenotyping confirmed a mixed myelo- megakaryoblastic character of the crisis. In the differential diagnosis other myeloproliferative syndromes were taken into account including i(17q) syndrome. The patient died after a 13-month observation due to neoplasm progression and sepsis.

摘要

我们报告一例17岁患者,有极度肝脾肿大、血小板增多、白细胞增多,且外周血涂片中有成髓细胞和原巨核细胞。疾病过程中出现的众多并发症使细胞抑制治疗变得困难。由于不存在费城染色体和bcr/abl融合基因,确诊为处于原始细胞危象期的未分类慢性骨髓增殖性综合征。免疫表型分析证实危象具有混合性成髓-原巨核细胞特征。鉴别诊断时考虑了其他骨髓增殖性综合征,包括i(17q)综合征。患者在13个月的观察期后因肿瘤进展和败血症死亡。

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Acta Haematol Pol. 1996;27(1):85-8.
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