CAJ | 학술논문

目的探讨儿童粒细胞肉瘤（GS ）的临床表现、病理组织学及免疫组化特点、治疗及预后。方法收集2004年1月-2014年1月，复旦大学附属儿科医院血液科收治的6例GS 患儿资料，对其临床表现、诊断、治疗及预后进行分析。结果6例GS 患儿均累及软组织、淋巴结、椎体、腹腔脏器等多个部位。4例诊断为原发性GS；2例同时伴骨髓浸润，骨髓形态及免疫分型均提示急性髓细胞白血病（AML）。5例患儿接受了联合化疗，目前2例CR（分别持续44个月、3个月），2例C R后复发，1例诱导治疗无效，复发与无效者均已放弃治疗。结论 GS临床罕见，临床表现多样，细胞形态学检查及免疫组织化学检查对其准确诊断非常必要。针对AML的化疗方案仍是首选的治疗方案。预后相对较差，难治复发性GS的治疗选择仍是难点。
목적탐토인동립세포육류（GS ）적림상표현、병리조직학급면역조화특점、치료급예후。방법수집2004년1월-2014년1월，복단대학부속인과의원혈액과수치적6례GS 환인자료，대기림상표현、진단、치료급예후진행분석。결과6례GS 환인균루급연조직、림파결、추체、복강장기등다개부위。4례진단위원발성GS；2례동시반골수침윤，골수형태급면역분형균제시급성수세포백혈병（AML）。5례환인접수료연합화료，목전2례CR（분별지속44개월、3개월），2례C R후복발，1례유도치료무효，복발여무효자균이방기치료。결론 GS림상한견，림상표현다양，세포형태학검사급면역조직화학검사대기준학진단비상필요。침대AML적화료방안잉시수선적치료방안。예후상대교차，난치복발성GS적치료선택잉시난점。
Objective To study the clinical characteristics,pathologic features ,diagnosis and treatment as well as prognosis of childhood granulocytic sarcoma(GS).Methods From January 2004 through January 201 4,six cases diagnosed as GS in our hospital were included in this study.Clinical manifestations,histopathological characteristics,diagnosis and therapy protocol were studied.Results Four patients were diagnosed as primary GS,two out of four cases occurred concurrently with AML.The most primary sites of GS are soft tissue, lymph node, vertebral body and abdominal viscera . Myeloperoxidase(6/6,1 00%)was the most sensitive and specific immunohistochemical marker.Five patients were treated with systemic chemotherapy two of them received tumor resection before chemotherapy,one reached CR then went to next course,one patient got sustained remission after chemotherapy.Two patients relapsed after remission,One patient′s induction therapy was unresponsive. Conclusions GS is a rare disease with diverse clinical manifestations,the prognosis is generally poor. Right diagnosis of GS is very much detailed morphological and immunohistochemical study dependent. Anti-AML chemotherapy is still the first line treatment.The treatment for refractory and relapsed GS is still difficult.