Clinical features and therapy of chronic myelogenous leukemia in childhood / 解放军医学杂志

ABSTRACT

Objective To investigate the clinical features of the childhood chronic myelogenous leukemia (CCML), including the pathogenesis, incidence, clinical characteristics, diagnostic criterion, prognostic significance and the treatment strategies, etc. Method The data of 148 cases of CCML were comprehensively reviewed and analyzed, and international and domestic literature in the last two decades was reviewed. Results The CCML was found to be rare with unknown etiology, and was an acquired malignant disease of clonal proliferation of hematopoietic stem cells in children. The disease included two clinical types adult CCML and juvenile CCML. 72.3% of CCML patients were diagnosed as the adult CCML. The clinical feature of CCML consisted of fatigue, low fever, anemia, hepatomegaly, splenomegaly, and lymphadenopathy. The laboratory findings of a typical CCML patient comprised of peripheral blood leukocytosis, basophilia and eosinophilia, myeloid differentiation in different stages, and increased megakaryocytes. The immunohistochemical features of the CCML consisted of highly positive MPO and CD68, significant lowering of neutrophil alkaline phosphatase (NAP), positive for Philadelphia chromosome (Ph) or chimeric BCR/ABL gene, etc. But in most cases of juvenile CCML, the Philadelphia chromosome could not be detected. The Gleevec therapy and hematopoietic stem cell transplantation (HSCT) might give better treatment result for CCML than the traditional therapy. Conclusions CCML has its characteristic clinical feature. The key of good therapeutic result is early diagnosis and treatment. The optimal therapy for CCML is Gleevec regime and HSCT.