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血细胞计数低:免疫介导、特发性或骨髓增生异常。

Low blood counts: immune mediated, idiopathic, or myelodysplasia.

机构信息

Department of Internal Medicine I, Division of Hematology & Hemostaseology and Ludwig Boltzmann Cluster Oncology, Medical University of Vienna, Vienna, Austria.

出版信息

Hematology Am Soc Hematol Educ Program. 2012;2012:485-91. doi: 10.1182/asheducation-2012.1.485.

Abstract

Traditionally, cytopenias are classified as deficiency mediated, immune mediated, BM failure induced, renal, or idiopathic, with the latter including the so-called idiopathic cytopenias of undetermined significance (ICUS). Clinical findings, symptoms, blood counts, BM findings, and other laboratory parameters are usually sufficient to reveal the type and cause of a marked cytopenia. However, in patients with chronic mild cytopenia, it may be a challenge for the physician to establish a correct diagnosis. In such patients, laboratory features and findings often reflect a diagnostic interface, so that criteria that are otherwise robust may hardly be applicable or are not helpful. Even if the BM is examined, the diagnosis often remains uncertain in these patients. In addition, more than one potential cause of cytopenia may be present, especially in the elderly. A myelodysplastic syndrome (MDS) or another BM disorder, but also an overt autoimmune or other inflammatory disease, may develop during follow-up in these patients. A key problem is that in an early phase of MDS, most laboratory and clinical signs are "nonspecific." One of the very few reliable peripheral blood parameters distinguishing between an early or "pre-phase" of MDS and most other causes of a mild cytopenia are the numbers of circulating colony-forming progenitor cells. In addition, flow cytometric and molecular investigations may sometimes assist in the delineation between clonal and reactive conditions underlying mild cytopenias. This review provides an overview of diagnostic approaches and algorithms for patients with mild unexplained cytopenia(s).

摘要

传统上,血细胞减少症分为由缺乏引起、免疫介导、骨髓衰竭引起、肾性或特发性,后者包括所谓的特发性未确定意义的血细胞减少症 (ICUS)。临床发现、症状、血细胞计数、骨髓发现和其他实验室参数通常足以揭示明显血细胞减少症的类型和原因。然而,对于医生来说,在慢性轻度血细胞减少症患者中建立正确的诊断可能是一个挑战。在这些患者中,实验室特征和发现通常反映了诊断的界面,因此其他情况下强大的标准可能难以适用或没有帮助。即使检查了骨髓,这些患者的诊断通常仍然不确定。此外,可能存在一种以上导致血细胞减少症的潜在原因,尤其是在老年人中。骨髓增生异常综合征 (MDS) 或其他骨髓疾病,但也可能是显性自身免疫或其他炎症性疾病,在这些患者的随访中可能会发展。一个关键问题是,在 MDS 的早期阶段,大多数实验室和临床迹象都是“非特异性的”。在 MDS 的早期或“前阶段”与导致轻度血细胞减少症的大多数其他原因之间区分开来的极少数可靠外周血参数之一是循环集落形成祖细胞的数量。此外,流式细胞术和分子研究有时可能有助于区分轻度血细胞减少症潜在的克隆和反应性情况。这篇综述提供了用于诊断轻度不明原因血细胞减少症患者的方法和算法概述。

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