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Abstract：
To investigate the long-term clinical efficacy of allogeneic hematopoietic stem cell transplantation (allo-HSCT) for patients with human herpes virus 8 (HHV8)-positive multicentric Castleman\'s disease (MCD).
A 17-year-old female patient was admitted to Henan Provincial People\'s Hospital with the complaint of febrile for half a month, headache, and enlarged superficial lymph nodes on October 5, 2010. HHV8-positive mixed cellular Castleman\'s disease was found by pathological diagnosis of lymph nodes biopsy. After the administration of CHOP and Hyper-CVAD-B, the patient was still febrile, we administrated the followed COAP, two courses of VAD(Vincristine, Adriamycin, Dexamethasone), the patient received CR. Six months after CR, the patient relapsed, we administrated VAD and two courses of bortezomide+dexamethasone chemotherapy, and then the patient received PR. After that, the patient underwent allo-HSCT from his human leukocyte antigen (HLA)-matched unrelated donor after conditioning with Bu/Cy+Etoposide+Smoustin.graft-vs-host disease (GVHD) prophylaxis, which consisted of ATG (7.5 mg/kg, qd, ivdrip) from d-5 to d-2, cyclosporine (3 mg/kg/d, qd, ivdrip, for 24 h) started from day-1, MMF(0.5 g, tid, po.) started from day+1 to +28, and MTX (15 mg per time, ivdrip, d+1,+4,+7,+11). She received 3.5×106/L CD34+cells and 8.1×108/LMNC.
Granulocyte engraftment occurred on day+12, platelet engrafted on day+14. Bone marrow biopsy showed normalization of trilineage hematopoiesis on day+33, chimerism: 97.6%. The transplantation was successful and followed up for 7 years with CR.
Allo-HSCT might cure patients with refractory/relapsed HHV8+ MCD.
A 17-year-old female patient was admitted to Henan Provincial People\'s Hospital with the complaint of febrile for half a month, headache, and enlarged superficial lymph nodes on October 5, 2010. HHV8-positive mixed cellular Castleman\'s disease was found by pathological diagnosis of lymph nodes biopsy. After the administration of CHOP and Hyper-CVAD-B, the patient was still febrile, we administrated the followed COAP, two courses of VAD(Vincristine, Adriamycin, Dexamethasone), the patient received CR. Six months after CR, the patient relapsed, we administrated VAD and two courses of bortezomide+dexamethasone chemotherapy, and then the patient received PR. After that, the patient underwent allo-HSCT from his human leukocyte antigen (HLA)-matched unrelated donor after conditioning with Bu/Cy+Etoposide+Smoustin.graft-vs-host disease (GVHD) prophylaxis, which consisted of ATG (7.5 mg/kg, qd, ivdrip) from d-5 to d-2, cyclosporine (3 mg/kg/d, qd, ivdrip, for 24 h) started from day-1, MMF(0.5 g, tid, po.) started from day+1 to +28, and MTX (15 mg per time, ivdrip, d+1,+4,+7,+11). She received 3.5×106/L CD34+cells and 8.1×108/LMNC.
Granulocyte engraftment occurred on day+12, platelet engrafted on day+14. Bone marrow biopsy showed normalization of trilineage hematopoiesis on day+33, chimerism: 97.6%. The transplantation was successful and followed up for 7 years with CR.
Allo-HSCT might cure patients with refractory/relapsed HHV8+ MCD.
摘要：
探讨异基因造血干细胞移植(allo-HSCT)治疗人类疱疹病毒8型(HHV8)阳性多中心Castleman病(MCD)患者的长期临床疗效。
河南省人民医院收治一名17岁女性患者，因发热住院半个月，头痛,2010年10月5日浅表淋巴结肿大。淋巴结活检病理诊断发现HHV8阳性混合细胞Castleman病。CHOP和Hyper-CVAD-B给药后，病人还在发热,我们管理了随后的COAP，两节VAD（长春新碱，阿霉素,地塞米松），患者接受CR。CR后六个月，病人复发了,我们给予VAD和两个疗程的硼替佐米+地塞米松化疗，然后病人接受了PR.之后,患者在接受Bu/Cy+依托泊苷+Smoustin预处理后,接受了人类白细胞抗原(HLA)匹配的非亲缘供者的allo-HSCT.移植物抗宿主病（GVHD）预防，由ATG(7.5mg/kg，qd,静脉滴注)从d-5到d-2，环孢菌素(3mg/kg/d，qd,ivdrip,24小时)从第1天开始，MMF(0.5g，tid,po.)从第+1天到第+28天开始，MTX(每次15mg，ivdrip,d+1、+4、+7、+11)。患者接受3.5×106/LCD34+细胞和8.1×108/LMNC。
粒细胞移植发生在第+12天,血小板移植发生在第+14天。骨髓活检显示三系造血在第33天恢复正常，嵌合状态：97.6％。移植成功，随访7年。
Allo-HSCT可能治愈难治性/复发性HHV8+MCD患者。
河南省人民医院收治一名17岁女性患者，因发热住院半个月，头痛,2010年10月5日浅表淋巴结肿大。淋巴结活检病理诊断发现HHV8阳性混合细胞Castleman病。CHOP和Hyper-CVAD-B给药后，病人还在发热,我们管理了随后的COAP，两节VAD（长春新碱，阿霉素,地塞米松），患者接受CR。CR后六个月，病人复发了,我们给予VAD和两个疗程的硼替佐米+地塞米松化疗，然后病人接受了PR.之后,患者在接受Bu/Cy+依托泊苷+Smoustin预处理后,接受了人类白细胞抗原(HLA)匹配的非亲缘供者的allo-HSCT.移植物抗宿主病（GVHD）预防，由ATG(7.5mg/kg，qd,静脉滴注)从d-5到d-2，环孢菌素(3mg/kg/d，qd,ivdrip,24小时)从第1天开始，MMF(0.5g，tid,po.)从第+1天到第+28天开始，MTX(每次15mg，ivdrip,d+1、+4、+7、+11)。患者接受3.5×106/LCD34+细胞和8.1×108/LMNC。
粒细胞移植发生在第+12天,血小板移植发生在第+14天。骨髓活检显示三系造血在第33天恢复正常，嵌合状态：97.6％。移植成功，随访7年。
Allo-HSCT可能治愈难治性/复发性HHV8+MCD患者。
