医学
肾病综合征
低蛋白血症
膜性肾病
阿纳斯卡
他克莫司
胃肠病学
内科学
蛋白尿
美罗华
移植物抗宿主病
移植
免疫学
外科
淋巴瘤
肾
作者
Stefanie W. Benoit,Pooja Khandelwal,Michael Grimley
摘要
Membranous nephropathy (MN) is the most common cause of glomerulopathy after hematopoietic cell transplantation (HCT), most often occurring in the setting of graft versus host disease (GVHD). Twenty percent of patients will fail to respond to standard therapy and may progress to end stage renal disease. Here we present the case of a pediatric patient who developed chronic oral GVHD more than one-year post-HCT, who subsequently developed nephrotic syndrome (anasarca, nephrotic range proteinuria, hypoalbuminemia) and had a renal biopsy consistent with MN. Treated with ibrutinib for her GVHD, and steroids, tacrolimus, and rituximab for her MN, she failed to achieve even partial remission of her kidney disease after 8 months. Due to steroid toxicity and 0% CD19 cells on lymphocyte subpopulation flow cytometry, the decision was made to trial plasma cell depletion therapy with daratumumab.She received three doses of daratumumab at weeks 1, 4, and 17.Her nephrotic syndrome resolved and her serum albumin was greater than 3.0 gm/dl by week 10. She was weaned off of both steroids and tacrolimus by week 16, at which time she had near-complete remission of her renal disease.Daratumumab may be an important, novel therapeutic option for post-HCT MN patients who are not responsive to standard therapies.
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