儿童粒细胞肉瘤4例报告并文献复习

Children Granulocytic Sarcoma:four cases report and review of literature

目的提高对粒细胞肉瘤(GS)的起病方式、临床病理特征、诊断及治疗的认识。方法对4例儿童GS进行起病方式、免疫病理、诊断及治疗的分析并复习相关文献。结果非白血病性GS3例,白血病性GS1例。4例免疫病理检测中3例MPO均为阳性;2例CD99阳性,1例CD99(+/-);2例CD68(Kp-1)阳性;1例CD43阳性。而作为淋巴瘤标记的CD3、CD20、TDT均为阴性。结论GS诊断主要依据病理活检及免疫组化检测,其治疗主要是按照AML的方案给予系统化疗,为达到长期无病生存,在系统化疗取得缓解后,可及早进行造血干细胞移植。

Objective To investigate mode of onset, clinicopathological features, diagnosis and therapy of children granulocytic sarcoma will be helpful for improvement on recognization the diasease. Methods Clinical manifestations, histopathological characteristics, diagnosis and therapy protocol of four cases of children granulocytic sarcoma were studied and the related literature was reviewed. Results Of 4 cases, 3 cases were isolated granulocytic sarcoma and 1 case was leukosarcoma. Immunophenotype analysis： MPO were positive in 3 cases, CD99 and CD68 were positive in 2 cases, CD45 was positive in 1 case. Tumor cells of 4 cases were negtive for CD3, CD20 and TDT. Chemotherapy protocol of AML was given to all of 4 cases. Case 1 and case 3 respectively progressed to AML in 25 months and 10 months after diagnosis. Case 2 died of intracranial hemprrrhage due to bone marrow relapse in 3 months after diagnosis. Case 4 showed good response to chemotherapy with nomal bone marrow image for 13 months now. Conclusion Diagnosis of granulocytic sarcoma mainly based on immunohistochemistry examination. Common therapy is itdopted chemotherapy protocol of AMI. To achieve long-term disease free survival, haemopoietic stem cell transplantation shoud be carried out in time during, remission after given intensive chemotherapy.