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基于原始髓细胞和白细胞计数的慢性粒单核细胞白血病精细分类

Refined medullary blast and white blood cell count based classification of chronic myelomonocytic leukemias.

作者信息

Schuler E, Schroeder M, Neukirchen J, Strupp C, Xicoy B, Kündgen A, Hildebrandt B, Haas R, Gattermann N, Germing U

机构信息

Department of Hematology, Oncology and Clinical Immunology, Heinrich-Heine-University, Düsseldorf, Germany.

Department of Hematology, Oncology and Clinical Immunology, Heinrich-Heine-University, Düsseldorf, Germany.

出版信息

Leuk Res. 2014 Dec;38(12):1413-9. doi: 10.1016/j.leukres.2014.09.003. Epub 2014 Sep 17.

Abstract

Since 2001, chronic myelomonocytic leukemia (CMML) is classified by the WHO as myeloproliferative/myelodysplastic neoplasm. Herein we tried to better describe CMML patients with regard to hematological characteristics and prognosis using data of the Duesseldorf registry. We created 6 CMML subgroups, by dividing dysplastic and proliferative CMML at the cut-off of white blood cell count of 13,000/μL and splitting these two groups into 3 subgroups: CMML 0 with <5% blasts (n=101), CMML I with 5-9% blasts (n=204) and CMML II with 10-19% blasts (n=81). For comparison we included patients with RCMD, RAEB I and II. The newly created CMML 0 group had better prognosis than CMML I and II, median survival times were 31 months (ms), 19ms and 13ms, respectively (p<0.001). Median survival times between the corresponding dysplastic and proliferative subgroups 0 and 1 differed significantly: CMML 0 dysplastic 48ms and CMML 0 proliferative 17ms (p=0.03), CMML I dysplastic 29ms and CMML I proliferative 15ms (p=0.008), CMML II dysplastic 17ms and CMML II proliferative 10ms (p=0.09). Outcome of CMML patients worsens with increasing medullary blasts and when presenting as proliferative type. Therefore it is justified to separate CMML with <5% medullary blasts.

摘要

自2001年起,世界卫生组织将慢性粒单核细胞白血病(CMML)归类为骨髓增殖性/骨髓发育异常肿瘤。在此,我们试图利用杜塞尔多夫登记处的数据,更好地描述CMML患者的血液学特征和预后。我们通过将发育异常型和增殖型CMML按白细胞计数13,000/μL的临界值进行划分,并将这两组再细分为3个亚组,从而创建了6个CMML亚组:原始细胞<5%的CMML 0(n = 101)、原始细胞为5 - 9%的CMML I(n = 204)和原始细胞为10 - 19%的CMML II(n = 81)。为作比较,我们纳入了难治性血细胞减少伴多系发育异常(RCMD)、难治性贫血伴原始细胞增多I型(RAEB I)和难治性贫血伴原始细胞增多II型(RAEB II)患者。新创建的CMML 0组预后优于CMML I和CMML II组,中位生存时间分别为31个月(ms)﹑19ms和13ms(p<0.001)。相应的发育异常型和增殖型亚组0和1之间的中位生存时间存在显著差异:CMML 0发育异常型为48ms,CMML 0增殖型为17ms(p = 0.03);CMML I发育异常型为29ms,CMML I增殖型为15ms(p = 0.008);CMML II发育异常型为17ms,CMML II增殖型为10ms(p = 0.09)。CMML患者的预后随着骨髓原始细胞增多以及表现为增殖型而恶化。因此,将原始细胞<5%的CMML区分开来是合理的。

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