Jain Richa, Gupta Kirti, Bhatia Anmol, Bansal Arun, Bansal Deepak
Departments of Pediatrics (Hematology-Oncology Division), *Histopathology and Radiodiagnosis; PGIMER, Chandigarh, India. Correspondence to: Dr Kirti Gupta, Professor, Department of Histopathology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh. India.
Indian Pediatr. 2017 Sep 15;54(9):765-770. doi: 10.1007/s13312-017-1172-5.
Veno-occlusive disease of the liver is mostly encountered as a complication of hematopoietic stem cell transplantation with myeloablative regimens with an incidence estimated to be 13.7%. It is clinically characterized by tender hepatomegaly, jaundice, weight gain and ascites. Strong clinical suspicion and an early recognition of clinical signs are essential to establish the diagnosis and institute effective regimen. Another complication of cytotoxic drugs given for cancers, is development of busulfan-induced lung injury. A strong index of suspicion is needed for its diagnosis, especially in setting where opportunistic fungal and viral infections manifest similarly. We illustrate the clinical and autopsy finings in a 2½-year-old boy who received autologous stem-cell transplantation following resection of stage IV neuroblastoma. He subsequently developed both hepatic veno-occlusive disease and busulfan-induced lung injury. The autopsy findings are remarkable for their rarity.
肝静脉闭塞病大多是清髓性造血干细胞移植的并发症，采用清髓方案时估计发病率为13.7%。其临床特征为肝肿大伴压痛、黄疸、体重增加和腹水。强烈的临床怀疑和对临床体征的早期识别对于确立诊断和制定有效的治疗方案至关重要。用于癌症治疗的细胞毒性药物的另一个并发症是白消安诱导的肺损伤。其诊断需要高度怀疑指数，尤其是在机会性真菌和病毒感染表现相似的情况下。我们展示了一名2岁半男孩的临床和尸检结果，该男孩在IV期神经母细胞瘤切除术后接受了自体干细胞移植。他随后发生了肝静脉闭塞病和白消安诱导的肺损伤。尸检结果因其罕见性而引人注目。
