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3例患者以血小板减少作为白血病前期的首发症状。

Thrombocytopenia as presenting symptom of preleukaemia in 3 patients.

作者信息

Tricot G, Criel A, Verwilghen R L

出版信息

Scand J Haematol. 1982 Mar;28(3):243-50. doi: 10.1111/j.1600-0609.1982.tb00522.x.

Abstract

3 patients with preleukaemia (idiopathic acquired myelodysplasia) presenting with isolated peripheral thrombocytopenia and an increased number of megakaryocytes are described. The diagnosis of preleukaemia, however, was strongly suspected on bone marrow morphology, revealing an abnormal megakaryocytic series. Megakaryoblasts with characteristic nuclei and basophilic pseudopodes, and immature megakaryocytes were frequent. These last cells had 1 or 2 nuclei and a basophilic poorly granulated cytoplasm, often containing large vacuoles, corresponding to dilated interconnecting demarcation membranes in electron microscopy. Megakaryocytes were not considered able to produce a normal amount of platelets, explaining the peripheral thrombocytopenia; platelet survival (51Cr) was indeed too long for ITP. Cytogenetic analysis confirmed the diagnosis of an abnormal haematopoietic clone. Ferrokinetics, in vitro agar cultures, bone marrow biopsy and platelet function tests were helpful additional examinations for establishing the correct diagnosis. It is important to differentiate preleukaemia from ITP. Prognosis is totally different and the treatment is not only ineffective in the great majority of the preleukaemic patients, but may be hazardous.

摘要

本文描述了3例白血病前期(特发性获得性骨髓发育异常)患者,这些患者表现为单纯外周血血小板减少且巨核细胞数量增多。然而,根据骨髓形态学强烈怀疑为白血病前期,可见异常的巨核细胞系列。具有特征性细胞核和嗜碱性伪足的原巨核细胞以及未成熟巨核细胞很常见。这些晚期细胞有1个或2个细胞核,细胞质嗜碱性且颗粒稀少,常含有大空泡,在电子显微镜下对应于扩张的相互连接的分界膜。巨核细胞被认为无法产生正常数量的血小板,这解释了外周血血小板减少的原因;血小板生存期(51Cr)对于特发性血小板减少性紫癜(ITP)来说确实过长。细胞遗传学分析证实了异常造血克隆的诊断。铁动力学、体外琼脂培养、骨髓活检和血小板功能测试是有助于确立正确诊断的其他检查。将白血病前期与ITP区分开来很重要。预后完全不同,并且治疗不仅对绝大多数白血病前期患者无效,而且可能有风险。

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