Kasinathan Ganesh, Kori Ahlam Naila, Azmie Norasyikin Mohamad
Haematology Unit, Department of Internal Medicine, Tengku Ampuan Afzan Hospital, 25100, Kuantan, Pahang, Malaysia.
Haematopathology Unit, Department of Pathology, Tengku Ampuan Afzan Hospital, Kuantan, Pahang, Malaysia.
J Med Case Rep. 2019 Jul 22;13(1):223. doi: 10.1186/s13256-019-2164-y.
Post-transplant lymphoproliferative disorder is a serious disorder which occurs post hematopoietic stem cell transplant or solid organ transplantation. T-prolymphocytic leukemia is a T cell type monomorphic post-transplant lymphoproliferative disorder which accounts for only 2% of all mature lymphocytic leukemias in adults over the age of 30.
A 59-year-old man of Chinese ethnicity presented to our hematology unit with headache, lethargy, and exertional dyspnea for the past 1 month. He underwent an uneventful cadaveric renal transplant 20 years ago for chronic glomerulonephritis-induced end-stage renal disease. He had been on long-term immunosuppressants since then consisting of orally administered prednisolone 10 mg daily and orally administered cyclosporine A 50 mg twice daily. On examination, he was pale with a palpable liver and spleen. He had a functioning renal graft. Marrow flow cytometry confirmed T-prolymphocytic leukemia with lymphocytes expressing CD2, CD3, CD7, CD52, and TCL-1. His human T-cell lymphotropic virus and Epstein-Barr virus serology and deoxyribonucleic acid (DNA) were negative. He was treated with one cycle of cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy to which he failed to respond. In view of his renal allograft, he was not suitable for alemtuzumab due to the risk of nephrotoxicity. He was given orally administered venetoclax but he died on day 17 due to severe auto tumor lysis syndrome.
The place of immunophenotyping in the diagnosis and treatment of this disorder is of significant importance. More research needs to be carried out to further comprehend the pathophysiology and treatment modalities for this disorder.
移植后淋巴细胞增生性疾病是一种严重的疾病,发生于造血干细胞移植或实体器官移植后。T-原淋巴细胞白血病是一种T细胞型单形性移植后淋巴细胞增生性疾病,在30岁以上的成年人中仅占所有成熟淋巴细胞白血病的2%。
一名59岁的华裔男性因过去1个月出现头痛、嗜睡和劳力性呼吸困难就诊于我们的血液科。20年前,他因慢性肾小球肾炎导致的终末期肾病接受了一次顺利的尸体肾移植。从那时起,他一直在服用长期免疫抑制剂,包括每日口服泼尼松龙10mg和每日两次口服环孢素A 50mg。检查时,他面色苍白,肝脏和脾脏可触及。他的肾移植功能良好。骨髓流式细胞术确诊为T-原淋巴细胞白血病,淋巴细胞表达CD2、CD3、CD7、CD52和TCL-1。他的人类嗜T细胞病毒和EB病毒血清学及脱氧核糖核酸(DNA)检测均为阴性。他接受了一个周期的环磷酰胺、阿霉素、长春新碱和泼尼松化疗,但治疗无效。鉴于他的肾移植情况,由于存在肾毒性风险,他不适合使用阿仑单抗。他接受了口服维奈克拉治疗,但在第17天因严重的自体肿瘤溶解综合征死亡。
免疫表型分析在该疾病的诊断和治疗中具有重要意义。需要开展更多研究以进一步了解该疾病的病理生理学和治疗方式。