ANCA +ve/anti-GBM +ve vasculitis following bone marrow transplantation.
ANCA +ve/anti-GBM +ve vasculitis following bone marrow transplantation.
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骨髓移植后 ANCA ve/抗 GBM ve 血管炎。
DOI：
10.1093/ndt/17.12.2280
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发表时间：
2002
期刊：
影响因子：
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通讯作者：
J. Anderton
中科院分区：
文献类型：
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作者：
Harish B. Shetty;A. Howat;J. Anderton
先生，2002 年 2 月号《肾病透析移植》中，Kingdon 等人[1]报告了一例自体干细胞移植后出现 ANCA qve 血管炎的病例。他们的文献综述仅揭示了骨髓或干细胞移植后发生小血管炎的另外三例[2-4]。我们希望报告一例成功骨髓移植后 20 个月出现严重急性肾功能衰竭的“双阳性”（即 ANCA 和抗 GBM qve）血管炎病例。该患者是一名 42 岁女性，1996 年因宫颈腺癌接受了子宫切除术和放射治疗。两年后，她出现了急性髓系白血病（AML M2 型），推测是继发于之前的放射治疗。在克拉克森最初的化疗（即柔红霉素和胞嘧啶）后，她的病情复发。因此，她接受了进一步的化疗，包括胞嘧啶阿糖苷-阿糖胞苷、米托蒽醌和依托泊苷，并于2000年4月接受了匹配的无关骨髓移植。除了轻微的移植物抗宿主病外，她的移植后进展顺利。 2001 年 10 月复查时，血液学特征和肾功能正常（肌酐 109 µmolul）。她于 2002 年 1 月再次就诊，当时已经有 3 周不适，伴有不适和呼吸困难。她没有发烧、血容量正常，没有败血症的证据。获得以下结果：钾7.0mmol，尿素92.8mmol，肌酸1667μmol，C反应蛋白182mgul。抗髓过氧化物酶抗体 (pANCA) 阳性 31.6 ELISA 单位（正常范围 0–4），抗肾小球基底膜抗体（抗 GBM）阳性 42 ELISA 单位（正常范围 0–2.5）。上述两个实验室结果在不同的参考实验室得到证实，抗GBM在149 Uuml（正常范围0-20）时呈阳性，pANCA在38 Uuml（正常范围0-6）时呈阳性。她的另一次自身抗体筛查结果呈阴性。血液学特征正常并且所有培养物都是无菌的。胸部X光检查和一氧化碳转移因子正常，排除肺出血。紧急肾活检显示 74 个肾小球，其中 30 个肾小球全面硬化，其中 40 个肾小球呈新月形；这些主要是细胞性的，少数显示出纤维化。偶尔可见肾小球簇状和新月状的局灶性坏死。有几个区域出现肾小管萎缩； 60% 的皮质是有活力的。免疫组织化学结果呈阴性，无肾小球沉积物。诊断为“双阳性”（ANCAuanti-GBM qve）系统性血管炎。她接受了血液透析、大剂量类固醇、环磷酰胺和五次血浆置换治疗。五次疗程后实现了透析独立性，现在，就诊后 3 个月，她的血管炎得到缓解，肾功能稳定（肌酐 200 µmolul）。最近的血液测试显示不存在抗 GBM 和 pANCA 抗体。在我们的案例中，免疫组织化学结果呈阴性可以用我们的实验室使用免疫过氧化物酶而不是免疫荧光这一事实来解释。据充分证明，线性抗 IgG 沉积物通常无法通过免疫过氧化物酶检测到 [5, 6]。肾功能的良好恢复有利于肾脏病变主要是 ANCA 相关性肾小球肾炎，而不是抗 GBM 疾病，后者只有在病程后期治疗时才会异常恢复。病毒和细菌感染被认为是移植后血管炎的触发因素，但本例中没有感染记录。正如 Sanmarco 等人所指出的[7]，骨髓移植后可能会检测到多种自身抗体。此外，他们是……
Sir, In the February 2002 issue of Nephrology Dialysis Transplantation, Kingdon et al.[1] reported a case of ANCA qve vasculitis following autologous stem cell transplantation. Their literature review revealed only three other cases of small vessel vasculitis following bone marrow or stem cell transplantation [2–4]. We wish to report a case of ‘double positive’(ie ANCA and anti-GBM qve) vasculitis presenting with severe acute renal failure 20 months after successful bone marrow transplantation. The patient, a 42-year-old female, was treated for adenocarcinoma of the cervix in 1996 with hysterectomy and radiotherapy. Two years later, she presented with acute myeloid leukaemia (AML type M2), presumed secondary to her previous radiotherapy. She relapsed following initial Clarkson’s chemotherapy (ie daunorubicin and cytosine). Consequently, she received further chemotherapy with cytosine arabinoside-cytarabine, mitoxantrone and etoposide and underwent matched unrelated bone marrow transplantation in April 2000. Her post-transplant progress was smooth, aside from mild graft vs host disease. At the time of review in October 2001, haematology profile and renal function were normal (creatinine 109 цmolul). She presented again in January 2002, having been unwell for 3 weeks with malaise and dyspnoea. She was afebrile and euvolaemic with no evidence of sepsis. The following results were obtained: potassium 7.0 mmolul, urea 92.8 mmolul, creatine 1667 цmolul, C-reactive protein 182mgul. Antimyeloperoxidase antibody (pANCA)-positive 31.6 ELISA units (normal range 0–4), anti-glomerular basement membrane antibody (anti-GBM)-positive 42 ELISA units (normal range 0–2.5). The above two laboratory results were confirmed at a different reference laboratory, anti-GBM was positive at 149 Uuml (normal range 0–20) and pANCA was positive at 38 Uuml (normal range 0–6). Her other auto antibody screen was negative. Haematology profile was normal and all cultures were sterile. Chest X-ray and carbon monoxide transfer factor were normal, excluding pulmonary haemorrhage. Urgent renal biopsy showed 74 glomeruli, 30 globally sclerosed, with 40 having crescents; these were predominantly cellular with a few showing fibrosis. Focal necroses were seen in occasional glomerular tufts and crescents. There were several areas of tubular atrophy with only; 60% of the cortex being viable. Immunohistochemistry was negative with no glomerular deposits.A diagnosis of ‘double positive’(ANCAuanti-GBM qve) systemic vasculitis was made. She received haemodialysis, high-dose steroids, cyclophosphamide and five plasma exchange treatments. Dialysis-independence was achieved after five sessions and now, 3 months following presentation, her vasculitis is in remission with stable renal function (creatinine 200 цmolul). A recent blood test showed absence of anti-GBM and pANCA antibodies. In our case the negative immunohistochemistry could be explained by the fact that our laboratory used immunoperoxidase rather than immunofluroscence. It is well documented that linear anti-IgG deposits are often impossible to detect by immunoperoxidase [5, 6]. The good recovery of renal function favours the renal lesion being predominantly ANCA-associated glomerulonephritis rather than anti-GBM disease, which only exceptionally recovers when treated late in the course of the disease. Viral and bacterial infections are postulated triggers for post-transplant vasculitis, but no infection was documented in this case. As noted by Sanmarco et al.[7], a variety of auto antibodies may be detected following bone marrow transplantation. Furthermore, they are …