疑难病研究——幼年型慢性粒细胞白血病

幼年型慢性粒细胞白血病是一种少见的儿童慢性髓系白血病,现将其分类为骨髓增生异常综合征(MDS)中的一型,与慢性粒单核细胞白血病(CMML),单体7综合征统一命名为幼年型粒单核细胞白血病(JMML)。儿童罕见,其病因未明。临床表现以发热、贫血和出血,肝脾肿大;血白细胞显著增多,以中性杆状核和晚幼粒细胞占优势,并可发现有核红细胞,血小板减少;HbF增高;骨髓示粒系增生。对药物效果欠佳,预后不良。干细胞移植后可望得到长期缓解。

A Difficult and Complicated Case Study: Juvenile Chronic Myeloid Leukemia.

Juvenile chronic myeloid leukemia(JCML) is a rare disease of unknown etiology in children.It is classified to a type of myelodysplastic syndrome(MDS) according to the WHO classification of myelodysplastic and myeloproliferative diseases,which was named juvenile myelomonocytic leukemia(JMML) including chronic myelomonocytic leukemia(CMML),JCML and the monosomy 7 syndrome.The clinical features consist of fever,pallor,hepatosplenomegaly,lymphadenopathy and skin rash.The laboratory findings comprise white cell count >10×10~9/L,monocyte count >1×10~9/L,thrombocytopenia,myeloid precursors and normoblasts in blood smear;bone marrow blast count <20%;hemoglobin F increased for age.The therapeutic effectiveness with chemotherapy for this disorder is bad and with worse prognosis.Hematopoietic stem cell transplantation(HSCT) in children with JMML might have better outcome for longer time.