Chronic myelomonocytic leukemia in childhood. A retrospective analysis of 110 cases.

Chronic myelomonocytic leukemia in childhood. A retrospective analysis of 110 cases.
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儿童时期患有慢性粒单核细胞白血病。

DOI:
10.1182/blood.v89.10.3534.3534_3534_3543
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发表时间:
1997
期刊:
影响因子:
20.3
通讯作者:
M. Zimmermann
M. Zimmermann
中科院分区:
医学1区
文献类型:
--
作者:
C. Niemeyer;M. Aricò;G. Basso;A. Biondi;A. C. Rajnoldi;U. Creutzig;O. Haas;J. Harbott;H. Hasle;G. Kerndrup;F. Locatelli;G. Mann;B. Stollmann;E. T. Veer;E. Wering;M. Zimmermann

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慢性髓细胞白血病(CMML)是一种罕见的儿童造血恶性肿瘤。为了确定该疾病的临床和血液学特征,我们对110名诊断为CMML的儿童进行了回顾性分析,而不考虑核型。诊断时的中位年龄为1.8岁。1型神经纤维瘤病患儿占14%,其他临床异常患儿占7%。入院时,白细胞计数中位数为35 × 109/L,单核细胞计数中位数为7 × 109/L。36%的患者发现骨髓细胞核型异常,而26%的儿童有7号单体。7号单体儿童在临床表现上与正常核型儿童没有区别。然而,他们确实表现出一些典型的血液学特征。在110名儿童中,38名接受了异体骨髓移植(BMT)。BMT组的10年生存率为0.39(标准误差[SE] = 0.10),非BMT组的72例患者的10年生存率为0.06 (SE = 0.4)。血小板计数、年龄和诊断时的血红蛋白F是非bmt组生存时间的主要预测因素。目前迫切需要对骨髓增生异常综合征(MDS)患儿的命名法达成广泛一致。我们在此建议使用法国-美国-英国的儿童MDS分类。
Chronic myelomonocytic leukemia (CMML) is a rare hematopoietic malignancy of childhood. To define the clinical and hematologic characteristics of the disease, we performed a retrospective analysis of 110 children given the diagnosis CMML irrespective of karyotype. Median age at diagnosis was 1.8 years. Neurofibromatosis type 1 was known in 14% and other clinical abnormalities in 7% of the children. At presentation, the medium white blood count was 35 × 109/L, with a median monocyte count of 7 × 109/L. Karyotypic abnormalities in bone marrow cells were noted in 36% of the patients, whereas 26% of the children had monosomy 7. Children with monosomy 7 did not differ from those with normal karyotype with respect to their clinical presentation. However, they did display some characteristic hematologic features. Of 110 children, 38 received an allogeneic bone marrow transplant (BMT). The probability of survival at 10 years was 0.39 (standard error [SE] = 0.10) for the BMT group and 0.06 (SE = 0.4) for the 72 patients of the non-BMT group. Platelet count, age, and hemoglobin F at diagnosis were the main predicting factors for the length of survival in the non-BMT group. There is a strong need for a broad agreement on nomenclature in children with myelodysplastic syndromes (MDS). We propose here to use the French-American-British classification for MDS in childhood.
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期刊: Blood
影响因子: 20.3
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发表时间: 1988
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