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髓系造血与骨髓增殖性疾病

Myelopoiesis and myeloproliferative disorders.

作者信息

Raskin R E

机构信息

Department of Physiological Sciences, College of Veterinary Medicine, University of Florida, Gainesville, USA.

出版信息

Vet Clin North Am Small Anim Pract. 1996 Sep;26(5):1023-42. doi: 10.1016/s0195-5616(96)50054-9.

DOI:10.1016/s0195-5616(96)50054-9
PMID:8863389
Abstract

Myeloid cells arise from a common stem cell whose development is regulated by stimulatory and inhibitory growth factors. Pluripotential hematopoietic stem cells are most influenced by IL-3, GM-CSF, and stem cell factor while committed progenitor cells are regulated by variable concentrations of GM-CSF, G-CSF, M-CSF, IL-5, Epo, and Tpo. As a result of their common origin, a key point to remember about myeloproliferative disorders is the involvement of multiple cell lines in dysplastic and neoplastic conditions. Dysplastic changes may signal early neoplastic changes with cases progressing to acute leukemia. Myelodysplastic syndrome (MDS) is associated with anemia or multiple cytopenias, normal to hypercellular bone marrow, ineffective hematopoiesis, and less than 30% blast cells of all nucleated cells in the bone marrow. Chronic myeloid leukemias also have less than 30% blast cells of all nucleated cells in the bone marrow and are distinguished from MDS by elevated cell counts of one or more cell lines with mature forms predominating. Acute myeloid leukemias, often the end result of all myeloproliferative disorders, are recognized by equal or greater 30% blast cells of all nucleated cells in the bone marrow. Additional diagnostic information from cytochemical stains, immunohistochemical staining, and cytogenetic analysis can influence the final diagnosis when morphology alone is equivocal. In conclusion, prognosis and response to treatment are best determined by application of a uniform set of standards in evaluating hematolymphatic neoplasia. Critical to diagnosis are complete blood and bone marrow evaluations including observation for dysplastic changes and blast cell quantitation. In addition, evidence for tissue infiltration identified through cytologic or histologic evaluations of lymph node, spleen, or liver is recommended.

摘要

髓系细胞起源于一种共同的干细胞,其发育受刺激和抑制性生长因子调控。多能造血干细胞受白细胞介素-3、粒细胞-巨噬细胞集落刺激因子和干细胞因子影响最大,而定向祖细胞则受不同浓度的粒细胞-巨噬细胞集落刺激因子、粒细胞集落刺激因子、巨噬细胞集落刺激因子、白细胞介素-5、促红细胞生成素和血小板生成素调控。由于它们起源相同,关于骨髓增殖性疾病需要记住的一个关键点是,在发育异常和肿瘤形成的情况下会涉及多种细胞系。发育异常的改变可能预示着早期肿瘤性改变,病例可能进展为急性白血病。骨髓增生异常综合征(MDS)与贫血或多种血细胞减少、骨髓正常至细胞增多、无效造血以及骨髓中所有有核细胞中原始细胞少于30%有关。慢性髓系白血病骨髓中所有有核细胞中原始细胞也少于30%,与MDS的区别在于一种或多种细胞系的细胞计数升高,且以成熟形式为主。急性髓系白血病通常是所有骨髓增殖性疾病的最终结果,其特征是骨髓中所有有核细胞中原始细胞等于或超过30%。当仅靠形态学诊断不明确时,来自细胞化学染色、免疫组织化学染色和细胞遗传学分析的额外诊断信息会影响最终诊断。总之,通过应用一套统一的标准来评估血液淋巴系统肿瘤,才能最好地确定预后和治疗反应。诊断的关键是进行全血细胞和骨髓评估,包括观察发育异常的改变和原始细胞定量。此外,建议通过对淋巴结、脾脏或肝脏进行细胞学或组织学评估来确定组织浸润的证据。

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