Huang Yu-Rong, Xie Cai-Qin, Tong Jie-Feng, Zhang Xiao-Hong, Xu Yang, Yuan Xiang-Gui
Department of Hematology, the Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang Province, China.
Medicine (Baltimore). 2020 Apr;99(14):e19807. doi: 10.1097/MD.0000000000019807.
Very severe aplastic anemia (vSAA) with active infections is always fatal. Adequate infection control before hematopoietic stem cell transplantation is recommended.
A 38-year-old woman with vSAA suffered from acute perforated appendicitis and invasive pulmonary fungal infection, and she failed to respond to intense antimicrobial therapies.
She was diagnosed with refractory vSAA with stubborn acute perforated appendicitis and invasive pulmonary fungal infection.
We successfully completed an emergent reduced intensity conditioning-matched unrelated donor (MUD)-peripheral blood stem cell transplantation (PBSCT) as a salvage therapy in the presence of active infections. The conditioning regimens consisted of reduced cyclophosphamide 30 mg/kg/day from day-5 to day-3, fludarabine 30 mg/m/day from day-5 to day-3 and porcine-antilymphocyte immunoglobulin 15 mg/kg/day from day-4 to day-2 without total body irradiation. Cyclosporin A, mycophenolate mofetil and short-term methotrexate were administered as graft-versus-host disease (GVHD) prophylaxis. Neutrophils and platelets were engrafted on day+15 and day+21. Appendiceal abscess and severe pneumonia developed after neutrophil engraftment, which were successfully managed with intense antimicrobial therapy and surgical intervention.
Only limited cutaneous chronic GVHD was observed 5 months after transplantation. The patient still lives in a good quality of life 2 years after transplantation.
Active infections may be no longer a contraindication to hematopoietic stem cell transplantation for some patients with vSAA.
伴有活动性感染的极重型再生障碍性贫血(vSAA)通常是致命的。建议在造血干细胞移植前进行充分的感染控制。
一名38岁的vSAA女性患者患有急性穿孔性阑尾炎和侵袭性肺部真菌感染,强化抗菌治疗无效。
她被诊断为难治性vSAA,伴有顽固性急性穿孔性阑尾炎和侵袭性肺部真菌感染。
在存在活动性感染的情况下,我们成功完成了紧急降低强度预处理-匹配无关供者(MUD)-外周血干细胞移植(PBSCT)作为挽救治疗。预处理方案包括从第-5天至第-3天给予环磷酰胺30mg/kg/天、从第-5天至第-3天给予氟达拉滨30mg/m²/天以及从第-4天至第-2天给予猪抗淋巴细胞免疫球蛋白15mg/kg/天,不进行全身照射。给予环孢素A、霉酚酸酯和短期甲氨蝶呤预防移植物抗宿主病(GVHD)。中性粒细胞和血小板分别于+15天和+21天植入。中性粒细胞植入后出现阑尾脓肿和严重肺炎,通过强化抗菌治疗和手术干预成功处理。
移植后5个月仅观察到有限的皮肤慢性GVHD。患者移植后2年仍生活质量良好。
对于一些vSAA患者,活动性感染可能不再是造血干细胞移植的禁忌证。