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慢性粒细胞性白血病在儿童期较少见,仅占儿童白血病的1~5%,且幼年型慢粒Ph染色体常为阴性,起病较急,临床贫血、出血、发热、感染等表现较成人明显,白细胞增高相对不明显,对治疗的反应差,病程短,预后差。本文总结11例小儿慢粒,男8例,女3例,年龄5月~12岁。就诊前病程2天~3年,多在1—2月。体格检查:贫血貌9例,出血点2例,肝大1例,脾大1例,肝脾大9例。实验室检查Hb62~120g/L,WBC(10.6~820.0)×109/L,中性粒细胞0.04~0.84,BPC(27~582)×109/L,骨髓检查:增生极度活跃8例,明显活跃3例,粒系0.545~0.965,红系0~0.405,巨核细胞1~95个/片,NAP检查8例,阳性率5~58%,染色体检查4例,亚二倍体增多1例,8月后转为15P+,21三体1例,Ph+1例,正常1例。治疗4例,其余7例放弃治疗。小儿慢粒常易与其他白细胞增高性疾病混淆,在诊断中应加以鉴别。
Chronic granulocytic leukemia is rare in childhood, accounting for only 1 to 5% of childhood leukemia, and juvenile CK-negative Ph chromosome is often negative, onset of acute, clinical anemia, bleeding, fever, infection, etc. than adults Obviously, leukocytosis is relatively insignificant, poor response to treatment, short course, poor prognosis. This article summarizes 11 cases of children with CML, 8 males and 3 females, aged from May to 12 years old. Pre-treatment course of 2 days to 3 years, mostly in January-February. Physical examination: anemia in 9 cases, bleeding in 2 cases, 1 case of liver, splenomegaly, liver and spleen in 9 cases. Laboratory tests showed that Hb62 ~ 120g / L, WBC (10.6 ~ 820.0) × 109 / L, neutrophil 0.04 ~ 0.84, BPC 27 ~ 582 × 109 / Hyperplasia was extremely active in 8 cases, 3 cases were obviously active, 0.545 ~ 0.965 in granule, 0 ~ 0.405 in erythroid, 1 ~ 95 in megakaryocyte, 8 in NAP, the positive rate was 5 ~ 58% 4 cases of chromosome examination, sub-diploid increased in 1 case, after August 15P +, 21 trisomy in 1 case, Ph + 1 case, normal in 1 case. Treatment in 4 cases, the remaining 7 cases to give up treatment. Children with CML are often confused with other leukocytosis, and should be identified in the diagnosis.