以皮肤黏膜淋巴结改变为首发症状的ALK阳性间变性大细胞淋巴瘤1例报告及文献复习

ALK-positive anaplastic large cell lymphoma with changes in skin mucosal lymph nodes as first symptom:A case report and literature review

目的:分析儿童间变性大细胞淋巴瘤(ALCL)的临床特点、诊断标准和治疗方法,提高临床医生对该病的诊疗水平。方法:回顾性分析1例ALCL患儿的临床资料,包括临床症状、体征、辅助检查、诊断依据、鉴别诊断、治疗方案和疾病转归等,并结合文献复习总结该病的诊治要点。结果:该患儿以颈部淋巴结进行性增大为主要症状就诊,查体口唇殷红,表皮皲裂;周身皮肤干燥,以双手为著,掌心可见皲裂,指端可见膜状脱屑;双颈部见多个肿大淋巴结,边界不清,活动度差,伴压痛;舌乳头呈草莓舌。活检病理,ALK阳性ALCL;骨髓涂片未见异常;骨髓免疫分型、外周血免疫分型和脑脊液免疫分型均可见异常表型T淋巴细胞。按照《国际儿童非霍奇金淋巴瘤分期系统(2012年)》,该患儿明确诊断为ALK阳性ALCL(Ⅳ期,R3组)。给予APO方案诱导化疗后评估患者病情达部分缓解(PR),更换治疗方案为B-NHL-BFM-90方案;经过1个周期改良AA方案治疗后,患者骨髓和外周血免疫分型均转阴。结论:ALCL患儿的临床表现多样化,淋巴结活检是确诊ALCL的唯一依据,在明确诊断前不应盲目使用激素。

Objective:To analyze the clinical characteristics,diagnostic criteria and treatment methods of the children with degenerative large cell lymphoma(ALCL),and to improve the diagnosis and treatment level of the clinicians.Methods:The clinical data of one child with ALCL were retrospectively analyzed,including clinical symptoms,signs,auxiliary examination,diagnosis basis,differential diagnosis,treatmentPlan and disease outcome,and the key factors of diagnosis and treatment of the disease were summarized combined with literature review.Results:The child went to hospital because ofProgressive lymph node enlargement as the first symptom.The lips were red,the epidermis was cleft,the skin was dry,both hands wereProminent,and thePalm was cleft and the flaky desquamation was visible on the fingertips;multiple swollen lymph nodes were found on the sides of neck,the boundary was unclear,the activity wasPoor,and tenderness was also found;the tongue nipplePresented strawberry tongue.The biopsyPathological results showed ALK-positive ALCL.There were no abnormalities in the bone marrow smear;the abnormalPhenotypic T lymphocytes were found in the bone marrow immunotype,Peripheral blood immunotype and cerebrospinal fluid immunotype.According to the International Childhood Non-Hodgkin’s Lymphoma Staging System(2012),the chlid was clearly diagnosed as ALK-positive ALCL(stageⅣ,R3 group).After the induction chemotherapy of APO regimen was given,the condition of thePatient was up toPartial remission(PR),and the treatment regimen was replaced with B-NHL-BFM-90 regimen;after one cycle of modified AA regimen,the bone marrow andPeripheral blood immunotype of thePatient turned negative.Conclusion:The clinical manifestations of ALCLPatients are different;the lymph node biopsy is the only basis for the diagnosis of ALCL,and hormones could not be blindly used before definitive diagnosis.