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骨髓增殖性疾病的巨核母细胞终末阶段

Megakaryoblastic termination of myeloproliferative disorders.

作者信息

Amin M B, Maeda K, Carey J L, Babu R V, Raman B K

机构信息

Department of Pathology, Henry Ford Hospital.

出版信息

Henry Ford Hosp Med J. 1992;40(1-2):122-6.

PMID:1428963
Abstract

Megakaryoblastic termination of myeloproliferative disorders is rare. The morphology of megakaryoblastic transformation can be subtle and is often mistaken for myeloid or lymphoid proliferations. Previously reported observations suggest a relatively poor prognosis for this category of patients, making precise diagnosis imperative. A multifaceted approach using morphology, ultrastructure, cytochemistry, and immunological membrane analysis may be helpful. We present two cases of myeloproliferative disorder with aggressive megakaryoblastic phases (myelofibrosis with agnogenic myeloid metaplasia and chronic myeloid leukemia with blast crisis). The clinical course is described and the results of the morphological, cytochemical, ultrastructural, and cytogenetic studies of both cases are presented. In addition, immunochemical studies (flow cytometry) and platelet function studies (aggregation, beta-thromboglobulin, and platelet factor IV release) were done for one of these patients.

摘要

骨髓增殖性疾病的巨核母细胞终末期较为罕见。巨核母细胞转化的形态可能不明显,常被误诊为髓系或淋巴系增殖。先前报道的观察结果表明,这类患者的预后相对较差,因此精确诊断至关重要。采用形态学、超微结构、细胞化学和免疫膜分析的多方面方法可能会有所帮助。我们报告了两例具有侵袭性巨核母细胞期的骨髓增殖性疾病(原发性骨髓纤维化和慢性粒细胞白血病急变期)。描述了临床病程,并展示了两例病例的形态学、细胞化学、超微结构和细胞遗传学研究结果。此外,对其中一名患者进行了免疫化学研究(流式细胞术)和血小板功能研究(聚集、β-血小板球蛋白和血小板第IV因子释放)。

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