CAJ | 학술논문

目的：通过10例幼年型粒单核细胞白血病（JMML）的临床分析阐明该病的临床特点、诊断要点、治疗及预后。方法：对10例JMML患儿的临床资料进行回顾性分析。结果：临床症状中发热7例，肝大8例，脾大9例，皮疹4例，白细胞中位值45.30×109/L，血红蛋白中位值83 g/L，血小板中位值25×109/L，外周血见原始幼稚者8例。8例JMML行HbF检查均增高。10例行JMML相关基因检测，3例阳性。结论：JMML临床以发热、肝脾肿大为主要表现，多有白细胞增高，HbF增高，基因有助于诊断，误诊率高，预后不良，异基因造血干细胞移植为唯一可以治愈本病的方法。
목적：통과10례유년형립단핵세포백혈병（JMML）적림상분석천명해병적림상특점、진단요점、치료급예후。방법：대10례JMML환인적림상자료진행회고성분석。결과：림상증상중발열7례，간대8례，비대9례，피진4례，백세포중위치45.30×109/L，혈홍단백중위치83 g/L，혈소판중위치25×109/L，외주혈견원시유치자8례。8례JMML행HbF검사균증고。10례행JMML상관기인검측，3례양성。결론：JMML림상이발열、간비종대위주요표현，다유백세포증고，HbF증고，기인유조우진단，오진솔고，예후불량，이기인조혈간세포이식위유일가이치유본병적방법。
Objective:To clarify clinical features,diagnostic criteria,treatment and prognosis of the disease according to analysis 10 children suffered juvenile myelomonocytic leukemia(JMML).Method:Clinical data of 10 children with JMML were retrospectively analyzed.Result:Clinical symptoms included 7 cases with fever,8 cases with hepatomegaly, 9 cases with splenomegaly,4 cases with rash,the median white blood cell count was 45.30×109/L,the median hemoglobin was 83 g/L,and the median platelet count was 25×109/L.8 peripheral blood smear had naive cells.HbF tests were performed in 8 cases,all increased.10 cases were given genetic testing,3 cases were positive.Conclusion:JMML Mainly manifest with fever,hepatosplenomegaly,most with increased white blood cells and HbF,genetic testing contribute to the diagnosis,it has a high misdiagnosis rate,and poor prognosis.Allogeneic hematopoietic stem cell transplantation is the only way to cure the disease.