Schuler E, Schroeder M, Neukirchen J, Strupp C, Xicoy B, Kündgen A, Hildebrandt B, Haas R, Gattermann N, Germing U
Department of Hematology, Oncology and Clinical Immunology, Heinrich-Heine-University, Düsseldorf, Germany.
Department of Hematology, Oncology and Clinical Immunology, Heinrich-Heine-University, Düsseldorf, Germany.
Leuk Res. 2014 Dec;38(12):1413-9. doi: 10.1016/j.leukres.2014.09.003. Epub 2014 Sep 17.
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.
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。