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红细胞增多症的分类与诊断

The classification and diagnosis of erythrocytosis.

作者信息

McMullin M F

机构信息

Department of Haematology, The Queen's University, Belfast, UK.

出版信息

Int J Lab Hematol. 2008 Dec;30(6):447-59. doi: 10.1111/j.1751-553X.2008.01102.x. Epub 2008 Sep 23.

DOI:10.1111/j.1751-553X.2008.01102.x
PMID:18823397
Abstract

An absolute erythrocytosis is present when the red cell mass is raised and the haematocrit is elevated above prescribed limits. Causes of an absolute erythrocytosis can be primary where there is an intrinsic problem in the bone marrow and secondary where there an event outside the bone marrow driving erythropoiesis. This can further be divided into congenital and acquired causes. There remain an unexplained group idiopathic erythrocytosis. Investigation commencing with thorough history taking and examination and then investigation depending on initial features is required. Clear simple criteria for polycythaemia vera are now defined. Those who do not fulfil these criteria require further investigation depending on the clinical scenario and initial results. The erythropoietin level provides some guidance as to the direction in which to proceed and the order and extent of investigation necessary in an individual patient. It should thus be possible to make an accurate diagnosis in the majority of patients.

摘要

当红细胞容量增加且血细胞比容高于规定限度时,即存在绝对红细胞增多症。绝对红细胞增多症的病因可分为原发性(骨髓存在内在问题)和继发性(骨髓外有促使红细胞生成的因素)。这又可进一步分为先天性和后天性病因。还有一组不明原因的特发性红细胞增多症。需要先进行全面的病史采集和体格检查,然后根据初步特征进行进一步检查。目前已明确了真性红细胞增多症的清晰简单标准。不符合这些标准的患者需要根据临床情况和初步检查结果进行进一步检查。促红细胞生成素水平可为后续检查的方向、顺序及个体患者所需检查的范围提供一些指导。因此,大多数患者应该能够得到准确的诊断。

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