急性早期前体T淋巴母细胞白血病1例并文献复习

Early T-cell precursor acute lymphoblastic leukemia:report of 1 case and review of literature

目的提高对急性早期前体T淋巴母细胞白血病(ETP-ALL)诊断及治疗的认识。方法回顾性分析兰州大学第二医院2020年10月收治的1例误诊为外周T细胞淋巴瘤-非特指型(PTCL-NOS)的ETP-ALL患者的临床资料,并复习相关文献。结果患者以腹股沟淋巴结肿大为首发症状,在当地医院行淋巴结活组织检查及病理检查,并在外院会诊均诊断为"PTCL-NOS",按照该诊断化疗2个疗程(CHOPE方案、GLD方案,具体用药及剂量不详)后疗效欠佳,就诊于兰州大学第二医院。因流式细胞术发现骨髓中有原始细胞,完善其他相关检查后,明确诊断为ETP-ALL,积极采用Hyper-CVAD及EA方案交替化疗,3个疗程后疾病进展,第4、5个疗程加用西达本胺,疾病仍有进展,后随访患者死亡,病程约12个月。结论ETP-ALL具有独特的免疫表型特征,常规诱导治疗缓解率低,且复发率高、预后差。目前缺乏有效的标准治疗方案,尽早行异基因造血干细胞移植或及时加用新药可改善预后。

Objective To improve the understanding of the diagnosis and treatment of early T-cell precursor acute lymphoblastic leukemia(ETP-ALL).Methods The clinical data of a patient with ETP-ALL who was misdiagnosed as peripheral T-cell lymphoma-not otherwise specified(PTCL-NOS)admitted to the Second Hospital of Lanzhou University in October 2020 were retrospectively analyzed,and the relevant literature was reviewed.Results The patient who presented"inguinal lymphadenopathy"as the first symptom underwent lymph node biopsy and pathological examination at local hospital,and he was diagnosed as PTCL-NOS according to the consultation of another 2 hospitals.After 2 courses of chemotherapy(CHOPE regimen,GLD regimen,unknown specific medication and dosage),the therapeutic efficacy was poor.For further diagnosis and treatment,this patient came to Lanzhou University Second Hospital.Flow cytometry found blast cells in the bone marrow,and then other related examinations were completed,he was finally diagnosed as ETP-ALL.The chemotherapy regimens of Hyper-CVAD and EA were alternatively used,progressive disease(PD)occurred after 3 courses of treatment,and chidamide was added in the 4th and 5th courses of treatment,the disease still progressed,and the patient died after follow-up.The disease course of the patient was about 12 months.Conclusions ETP-ALL has unique immunophenotypic characteristics.ETP-ALL patients have a low remission rate after conventional induction therapy,high recurrence rate and poor prognosis.Currently,there is no effective standard treatment regimen,and allogeneic hematopoietic stem cell transplantation or timely addition of new drugs may improve the prognosis.