Chan Gavin Shueng-Wai, Chim Stella, Fan Yuen Shan, Chan Kwok Wah
Department of Pathology, University of Hong Kong, Queen Mary Hospital, Hong Kong.
Hum Pathol. 2006 Dec;37(12):1607-10. doi: 10.1016/j.humpath.2006.07.011. Epub 2006 Oct 3.
Heavy proteinuria after bone marrow transplantation (BMT) is rare. Pathology shows membranous glomerulonephritis (MGN) in most cases. After BMT, focal segmental glomerulosclerosis (FSGS) after resolution of MGN has not been reported. We describe a 13-year-old boy who had matched unrelated donor allogeneic BMT for relapsed acute lymphoblastic leukemia, complicated by chronic graft-versus-host disease. Nephrotic syndrome developed 1 year after BMT and renal biopsy revealed MGN. Immunosuppressive therapy achieved good clinical remission, and treatment was stopped after 15 months. He developed significant proteinuria 55 months later. The second renal biopsy showed FSGS without changes of MGN. This distinctive disease evolution gives inspiring implications. Complete morphological resolution of graft-versus-host disease-associated MGN, achieved in our case, has not been previously documented. Recurrent significant proteinuria after BMT is not necessarily due to previous renal lesion, and a repeat renal biopsy is indicated. The pathogenesis of MGN and FSGS are different, and different mechanisms of glomerular injury can interplay in a single patient after BMT. This case helps to expand our knowledge of the temporal morphological spectrum of renal lesions associated with BMT.
骨髓移植(BMT)后出现重度蛋白尿的情况较为罕见。病理检查在大多数病例中显示为膜性肾小球肾炎(MGN)。BMT后,MGN消退后出现局灶节段性肾小球硬化(FSGS)的情况尚未见报道。我们报告一名13岁男孩,他因复发性急性淋巴细胞白血病接受了匹配的无关供者异基因BMT,并并发慢性移植物抗宿主病。BMT后1年出现肾病综合征,肾活检显示为MGN。免疫抑制治疗取得了良好的临床缓解,15个月后停止治疗。55个月后他出现了大量蛋白尿。第二次肾活检显示为FSGS,无MGN改变。这种独特的疾病演变具有启发性意义。在我们的病例中实现的移植物抗宿主病相关MGN的完全形态学消退,此前尚未有文献记载。BMT后复发性大量蛋白尿不一定是由于先前的肾脏病变,需要再次进行肾活检。MGN和FSGS的发病机制不同,在BMT后的单一患者中,不同的肾小球损伤机制可能相互作用。该病例有助于扩展我们对与BMT相关的肾脏病变时间形态学谱的认识。