CAJ | 학술논문

BCR/ABL阴性的经典骨髓增殖性肿瘤包括真性红细胞增多症、原发性血小板增多症及原发性骨髓纤维化三种疾病.儿童BCR/ABL阴性的经典骨髓增殖性肿瘤的临床与分子生物学特点与成年人不尽相同,诊断与治疗方面也有其特殊性.该文结合近年的临床与基础研究,从临床特点、分子生物学特点、诊断与鉴别诊断及治疗等方面总结儿童BCR/ABL阴性的经典骨髓增殖性肿瘤的特点.
BCR/ABL음성적경전골수증식성종류포괄진성홍세포증다증、원발성혈소판증다증급원발성골수섬유화삼충질병.인동BCR/ABL음성적경전골수증식성종류적림상여분자생물학특점여성년인불진상동,진단여치료방면야유기특수성.해문결합근년적림상여기출연구,종림상특점、분자생물학특점、진단여감별진단급치료등방면총결인동BCR/ABL음성적경전골수증식성종류적특점.
BCR/ABL negative classical myeloproliferative neoplasms including three diseases,namely polycythemia veta,essential thrombocythemia and primary myelofibrosis.Progresses have been made in the study of these dieases in children following the development of that in adult,especially the research of molecular pathogenesis.Differences exist in clinical and molecular characteristics between children and adult BCR/ABL negative classical myeloproliferative neoplasms.There are specific characteristics in the diagnoses and treatment of childhood myeloproliferative neoplasms.This paper reviews the characteristics of childhood BCR/ABL negative classical myeloproliferative neoplasms in clinical and molecular features,diagnoses and treatment.