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[急性巨核细胞白血病:慢性髓性白血病的初发及转化]

[Acute megakarioblastic leukemia: de novo and transformation of chronic myeloid leukemia].

作者信息

Mihaljević B, Nedeljkov-Jancić R, Sretnović M, Janković S, Radosević I, Kraguljac N, Marisavljević D, Basara N, Jovanović V, Petrović M

出版信息

Srp Arh Celok Lek. 1995 May-Jun;123(5-6):164-6.

PMID:17974464
Abstract

Acute megakarioblastic leukemia (AMKL) is a rare myeloproliferative syndrome with a fulminant clinical course characterized by progressive pancytopenia, palior, weakness and severe haemorrhage. Two cases of AMKL are presented: a 18-year old male with pancytopenia and massive haemorrhage, lymphadenopathy, organomegaly and mediastinal tumour. The diagnosis of AMKL was established by cytological and immunocytochemical analyses of peripherial blood cells (blasts were GPIIIa and GPIb postitive), by histological analysis or the bone marrow and lymph node, and immunohistochemical analysis of lymph node. The second case had megakarioblastic transformation of HGL which was confirmed by cytomorphological and immunophenotypical analyses. In spite of therapy, the patients died soon after the first signs of the disease.

摘要

急性巨核细胞白血病(AMKL)是一种罕见的骨髓增殖综合征,临床病程凶险,特征为进行性全血细胞减少、面色苍白、虚弱及严重出血。本文报告2例AMKL:1例为18岁男性,有全血细胞减少、大量出血、淋巴结病、器官肿大及纵隔肿瘤。通过外周血细胞的细胞学和免疫细胞化学分析(原始细胞GPIIIa和GPIb阳性)、骨髓和淋巴结的组织学分析以及淋巴结的免疫组织化学分析确诊为AMKL。第2例为毛细胞白血病(HGL)巨核细胞转化,经细胞形态学和免疫表型分析得以证实。尽管进行了治疗,但患者在出现疾病的最初症状后不久便死亡。

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