Gologan R, Berceanu Ana, Colita Adriana, Codreanu Valeria, Dobrea Camelia, Geoada Liana, Iacob Viorica, Niculescu-Mizil Emilia, Ostroveanu Daniela, Puşcariu Tatiana, Ursuleac Iulia
Clinic of Hematology, Fundeni Clinical Institute, Bucharest, Romania.
Rom J Intern Med. 2002;40(1-4):95-102.
Chronic myelomonocytic leukemia (CMML) has long been recognized as a disorder with both myelodysplastic and myeloproliferative characteristics, some patients showing clinical and morphological features resembling myelodysplastic syndrome (MDS) especially refractory anemia with excess of blasts (RAEB) with monocytosis, and others leukocytosis with neutrophilia, monocytosis and splenomegaly resembling myeloproliferative syndrome (MPS). The intrinsec differences determined at first the separation of CMML in two forms, one named "dysplastic", more similar with RAEB, and the other "proliferative", closer to chronic myeloid leukemia and then included by the recent WHO classification into a separate new created group--myelodysplastic diseases (MDD)/chronic myeloproliferative diseases (CMPD). The aim of this study was the analysis of some features of 20 cases of CMML, with emphasis on the differences between the two forms. The proliferative form of CMML differed from the dysplastic one by greater white blood cells and neutrophils counts (P < 0.001), a more important monocytosis in periferal blood (P = 0.07), and by the size and frequency of splenomegaly (P = 0.03). The sex and age of the patients, the frequency of the general symptoms, the frequency of the general symptoms and signs and that of infections, the hemoglobin and hematocrit values, the platelet counts, the percentage of myeloblasts and monocytes in bone marrow, the frequency of dysplastic traits, the percentage of reticulocytes and the modified Bournemouth prognostic index were not significantly different. These findings support the concept that CMML is a heterogeneous "overlap" syndrome between MDS and CMPD.
慢性粒单核细胞白血病（CMML）长期以来一直被认为是一种具有骨髓增生异常和骨髓增殖特征的疾病，一些患者表现出类似于骨髓增生异常综合征（MDS）的临床和形态学特征，尤其是伴有单核细胞增多的难治性贫血伴原始细胞增多（RAEB），而另一些患者则表现为白细胞增多伴中性粒细胞增多、单核细胞增多和脾肿大，类似于骨髓增殖综合征（MPS）。最初，内在差异决定了CMML分为两种形式，一种称为“发育异常型”，与RAEB更相似，另一种称为“增殖型”，更接近慢性髓性白血病，随后被最近的世界卫生组织分类纳入一个单独的新创建组——骨髓增生异常疾病（MDD）/慢性骨髓增殖性疾病（CMPD）。本研究的目的是分析20例CMML患者的一些特征，重点是两种形式之间的差异。CMML的增殖型与发育异常型的区别在于白细胞和中性粒细胞计数更高（P<0.001），外周血单核细胞增多更明显（P = 0.07），以及脾肿大的大小和发生率（P = 0.03）。患者的性别和年龄、一般症状的发生率、一般症状和体征以及感染的发生率、血红蛋白和血细胞比容值、血小板计数、骨髓中原始粒细胞和单核细胞的百分比、发育异常特征的发生率、网织红细胞百分比和改良的伯恩茅斯预后指数均无显著差异。这些发现支持了CMML是MDS和CMPD之间的异质性“重叠”综合征这一概念。
