以脾脓肿为首发表现的慢性中性粒细胞白血病1例报道

Splenic abscess as the first manifestation of chronic neutrophilic leukemia:a case report

慢性中性粒细胞白血病(chronic neutrophilic leukemia,CNL)是一种以外周血成熟中性粒细胞持续性增多为特征,且BCR-ABL融合基因阴性的骨髓增殖性肿瘤(myeloproliferative neoplasm,MPN),发病率极低[1]。CSF3R突变编码的集落刺激因子受体,在粒细胞的增殖、分化和维持正常功能中发挥重要的作用。2016年WHO血液肿瘤分类中将CSF3R突变纳入CNL诊断标准[2]。

To improve the diagnosis and therapy of chronic neutrophilic leukemia(CNL),we reviewed the hematological and clinical characteristics of a patient with CNL.The onset of disease showed splenomegaly and splenic abscess.Peripheral white blood cell count went up obviously and then transiently decreased as disease progression,followed by anemia,thrombocytopenia,myelofibrosis and CSF3 R mutation.The patient was first misdiagnosed as atypical chronic myeloid leukemia,and then diagnosed as CNL by MICM reassessment.Splenctomy was performed but hemoglobin level and platelet count had not been improved significantly,and allogeneic hematopoietic stem cell transplantation is planned.CNL is a rare hematological disease with high clinical heterogeneity,and splenic abscess as the first manifestation has not been reported.