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目的 探讨小儿慢性粒-单核细胞白血病(CMML)临床和血液学的特点。方法 患儿均行血常规和骨髓细胞形态学检查,部分行骨髓粒-单祖细胞(CFU-GM)体外培养。结果6例均有贫血和血小板减少,外周血和骨髓单核细胞增多,骨髓至少一系血细胞呈病态造血。用现行标准诊断的小儿CMML,从临床上拟可分成两个组群。一组起病年龄小,明显肝脾淋巴结肿大,外周血白细胞数高,可见原幼粒细胞,骨髓原+早幼粒细胞可增多,带有一些骨髓增殖性疾病(MPD)的特点。另一组起病年龄大,无明显肝脾淋巴结肿大,外周血三系减少,可见原幼单核细胞,骨髓幼稚单核细胞明显增多,除单核系细胞外,有些符合骨髓增生异常综合征(MDS)中难治性贫血(RA)亚型的特点。3例送骨髓CFU-GM体外培养,2例集簇明显增多。结论 小儿CMML拟可分成两个组群,归属于兼有MDS/MPD特征的疾病较为合适。骨髓CFU-GM体外培养表现为对粒-单集落刺激因子(GM-CSF)高度敏感。
Objective To investigate the clinical and hematological characteristics of children with chronic myelomonocytic leukemia (CMML). Methods All children underwent routine blood tests and bone marrow cell morphometry. Some of them were cultured in vitro with CFU-GM. Results All 6 patients had anemia and thrombocytopenia, peripheral blood and bone marrow mononuclear cells increased, at least one line of blood cells in bone marrow showed pathological hematopoiesis. Pediatric CMML diagnosed with current standards can be clinically divided into two groups. A group of onset age is small, obvious hepatosplenomegaly, white blood cell count is high, showing that the original promyelocytic, promyelocytic + promyelocytic increased with some myeloproliferative disorders (MPD) characteristics. Another group of onset age, no obvious liver and spleen lymph nodes, peripheral blood three lines decreased, showing that young monocytes, bone marrow mononuclear cells increased significantly, in addition to mononuclear cells, some consistent with myelodysplastic syndrome Characteristics of refractory anemia (RA) subtype in patients with leukemia (MDS). Three cases of bone marrow CFU-GM were cultured in vitro, two cases of cluster increased significantly. Conclusions Pediatric CMML can be divided into two groups, which are suitable for the diseases characterized by both MDS and MPD. Bone marrow CFU-GM showed high sensitivity to granulocyte-monocyte colony stimulating factor (GM-CSF) in vitro.