Voglová J, Chrobák L, Neuwirtová R, Malasková V, Straka L
Department of Haematology, University Hospital, 50 005, Hradec Králové, Czech Republic.
Leuk Res. 2001 Jun;25(6):493-9. doi: 10.1016/s0145-2126(00)00159-4.
Several authors have tried to solve the problems in the classification of CMML. A fully suitable classification does not exist. The goal of our study was to determine common and different signs of MD and MP type of CMML and to observe frequency of shifts from MD to MP-CMML. Sixty nine CMML patients were divided according to FAB proposal into two groups: 31 patients into the MD group (WBC < or = 13 x 10(9)/l) and 38 patients into the MP group (WBC < or = 13 x 10(9)/l). Presenting features and the course of the disease in both groups were evaluated. The median age of patients was not different in both groups (71.5 and 74 years, respectively), male/female ratio was 1.1 and 2.4, respectively. The median follow-up time was 15.5 months (1-58.8) in MP group and 24 months (2-118) in MD group. In MP group splenomegaly, hepatomegaly, lymphadenopathy, abnormal karyotype and skin involvement were found more often than in MD group. Median LDH value was higher in MP group. Probability of survival was higher in the MD group than in MP group (median 30 and 11 months, respectively). Leukaemia transformation frequency was similar in both groups. In 12 out of 24 (50%) MD group patients WBC increased during the course of the disease over 13 x 10(9)/l. Oscillation of WBC values below and over 13 x 10(9)/l was observed in three patients. During the follow-up time number of patients with splenomegaly and/or immature granulocytes in the PB increased. After inclusion of 12 patients who shifted from MD to MP group a new CMML group resulted characterised by longer median survival (17 months) due to a higher number of patients in an earlier stage of the disease. Failure of evolution of myeloproliferative signs and lower frequency of AL in the remaining group might be explained by an early stage of CMML, untimely deaths due to unrelated causes and/or by patients suffering of RA with monocytosis rather than of CMML. In summary, our data suggest, that evolution from MD-CMML to MP-CMML is a frequent event and that MD-CMML could be the early stage of CMML in most of cases. The WBC at diagnosis as the single criterion for subclassification of CMML does not seem to be fully justified. We propose that CMML should not be divided in MD and MP types and that monitoring of patients and search for other signs of myeloproliferation such as PB immature granulocytes, splenomegaly, lymphadenopathy, skin involvement, pleural or peritoneal effusions, spontaneous growth of CFU-GM in vitro should be taken in consideration for a better classification of CMML, which would have an impact on the therapeutic approach.
几位作者试图解决慢性粒-单核细胞白血病（CMML）分类中的问题。但目前尚不存在完全合适的分类方法。我们研究的目的是确定CMML的骨髓增生异常（MD）型和骨髓增殖（MP）型的共同及不同特征，并观察从MD型向MP型CMML转变的频率。69例CMML患者根据FAB建议分为两组：31例患者归入MD组（白细胞计数≤13×10⁹/L），38例患者归入MP组（白细胞计数＞13×10⁹/L）。对两组患者的临床表现和病程进行了评估。两组患者的中位年龄无差异（分别为71.5岁和74岁），男女比例分别为1.1和2.4。MP组的中位随访时间为15.5个月（1 - 58.8个月），MD组为24个月（2 - 118个月）。与MD组相比，MP组脾肿大、肝肿大、淋巴结病、异常核型和皮肤受累更为常见。MP组的乳酸脱氢酶（LDH）中位值更高。MD组的生存概率高于MP组（中位生存期分别为30个月和11个月）。两组的白血病转化频率相似。MD组24例患者中有12例（50%）在病程中白细胞计数增加超过13×10⁹/L。3例患者观察到白细胞值在13×10⁹/L上下波动。随访期间，外周血中出现脾肿大和/或未成熟粒细胞的患者数量增加。将12例从MD组转变为MP组的患者纳入后，形成了一个新的CMML组，其特点是中位生存期较长（17个月），这是因为处于疾病早期阶段的患者数量较多。其余组中骨髓增殖体征演变失败和急性白血病（AL）频率较低可能是由于CMML处于早期阶段、因无关原因过早死亡和/或患有伴有单核细胞增多的类风湿关节炎（RA）而非CMML的患者所致。总之，我们的数据表明，从MD型CMML向MP型CMML的转变是常见事件，并且在大多数情况下MD型CMML可能是CMML的早期阶段。将诊断时的白细胞计数作为CMML亚分类的单一标准似乎并不完全合理。我们建议不应将CMML分为MD型和MP型，为了更好地对CMML进行分类，从而影响治疗方法，应考虑对患者进行监测并寻找其他骨髓增殖体征，如外周血未成熟粒细胞、脾肿大、淋巴结病、皮肤受累、胸腔或腹腔积液、体外CFU - GM的自发生长等。
