Zhang Zhi-Rong, Dou Ai-Xia, Liu Yan, Zhu Hong-Bo, Jia Hai-Peng, Kong Qiu-Hong, Sun Li-Kun, Qin Ai-Qiong
Department of Haematology, The Second Affiliated Hospital of Shandong First Medical University, Ta'an 271000, Shandong Province, China.
Department of Haematology, The Second Hospital of Shandong University, Cheeloo College of Medicine, Shandong University, Jinan 250033, Shandong Province, China.
World J Clin Cases. 2023 Jun 16;11(17):4072-4078. doi: 10.12998/wjcc.v11.i17.4072.
Angioimmunoblastic T-cell lymphoma (AITL), a unique subtype of peripheral T-cell lymphoma, has relatively poor outcomes. High-dose chemotherapy with autologous stem cell transplantation (ASCT) can achieve complete remission and improve outcomes. Unfortunately, subsequent T-cell lymphoma-triggered hemophagocytic lymphohistiocytosis (HLH) has a worse prognosis than B-cell lymphoma-triggered HLH.
We here report a 50-year-old woman with AITL who achieved a favorable outcome after developing HLH 2 mo after receiving high-dose chemotherapy/ ASCT. The patient was initially admitted to our hospital because of multiple enlarged lymph nodes. The final pathologic diagnosis, made on biopsy of a left axillary lymph node was AITL (Stage IV, Group A). Four cycles of the following chemotherapy regimen were administered: Cyclophosphamide 1.3 g, doxorubicin 86 mg, and vincristine 2 mg on day 1; prednisone 100 mg on days 1-5; and lenalidomide 25 mg on days 1-14. The interval between each cycle was 21 d. The patient received a conditioning regimen (busulfan, cyclophosphamide, and etoposide) followed by peripheral blood stem cell infusion. Unfortunately, she developed sustained fever and a low platelet count 17 d after ACST, leading to a diagnosis of HLH after ASCT. During treatment, she experienced thrombocytopenia and pneumonia. The patient was successfully treated with etoposide and glucocorticoids.
It is possible that development of HLH is related to immune reconstitution after ASCT.
血管免疫母细胞性T细胞淋巴瘤(AITL)是外周T细胞淋巴瘤的一种独特亚型,预后相对较差。高剂量化疗联合自体干细胞移植(ASCT)可实现完全缓解并改善预后。不幸的是,随后发生的T细胞淋巴瘤引发的噬血细胞性淋巴组织细胞增生症(HLH)的预后比B细胞淋巴瘤引发的HLH更差。
我们在此报告一名50岁的AITL女性患者,在接受高剂量化疗/ASCT后2个月发生HLH,但预后良好。患者最初因多处淋巴结肿大入院。对左腋窝淋巴结活检做出的最终病理诊断为AITL(IV期,A组)。给予以下化疗方案4个周期:第1天给予环磷酰胺1.3 g、阿霉素86 mg和长春新碱2 mg;第1 - 5天给予泼尼松100 mg;第1 - 14天给予来那度胺25 mg。每个周期之间的间隔为21天。患者接受了预处理方案(白消安、环磷酰胺和依托泊苷),随后进行外周血干细胞输注。不幸的是,她在ASCT后17天出现持续发热和血小板计数低,导致ASCT后诊断为HLH。治疗期间,她出现血小板减少和肺炎。患者接受依托泊苷和糖皮质激素治疗成功。
HLH的发生可能与ASCT后的免疫重建有关。