Ng Monica Suet Ying, Francis Leo, Pillai Elango, Mallett Andrew John
Kidney Health Service, Royal Brisbane and Women's Hospital, Brisbane, Australia.
Faculty of Medicine, The University of Queensland, Brisbane, Australia.
BMC Nephrol. 2018 Sep 10;19(1):224. doi: 10.1186/s12882-018-1034-y.
Paraneoplastic glomerulonephritis is rare in haematological malignancies and tends to manifest as minimal change disease, membranous glomerulonephritis or membranoproliferative glomerulonephritis. We present the first report of immunoglobulin A nephropathy and associated focal segmental glomerulosclerosis in a patient with asymptomatic low grade B-cell lymphoma.
A 53 year old gentleman presented with nephrotic range proteinuria (urine protein creatinine ratio of 662 mg/mmol) on a background of type 2 diabetes mellitus (glycosylated haemoglobin: < 6%), hypertension, obesity (body mass index: 47.6 kg/m) and degenerative spine disease. Bone marrow biopsy diagnosed a low grade B-cell lymphoma and renal biopsy was consistent with immunoglobulin A nephropathy. Lymphoma treatment with six cycles of cyclophosphamide/ rituximab/ prednisolone led to normalisation of urinary protein excretion (urine protein creatinine ratio: 14 mg/mmol at 26 months post-chemotherapy).
Paraneoplastic immunoglobulin A nephropathy can occur with a broad range of haematological malignancies regardless of stage. This case illustrates the importance of meticulous haematological system work-up for patients presenting with immunoglobulin A nephropathy. Recognition of paraneoplastic immunoglobulin A nephropathy and early diagnosis of associated malignancy can be life-saving.
副肿瘤性肾小球肾炎在血液系统恶性肿瘤中较为罕见，往往表现为微小病变型肾病、膜性肾小球肾炎或膜增生性肾小球肾炎。我们报告了首例无症状低度B细胞淋巴瘤患者并发免疫球蛋白A肾病及相关局灶节段性肾小球硬化症的病例。
一名53岁男性，患有2型糖尿病（糖化血红蛋白：<6%）、高血压、肥胖（体重指数：47.6kg/m²）和退行性脊柱疾病，出现肾病范围蛋白尿（尿蛋白肌酐比值为662mg/mmol）。骨髓活检诊断为低度B细胞淋巴瘤，肾活检结果符合免疫球蛋白A肾病。采用环磷酰胺/利妥昔单抗/泼尼松龙进行六个周期的淋巴瘤治疗后，尿蛋白排泄恢复正常（化疗后26个月时尿蛋白肌酐比值：14mg/mmol）。
副肿瘤性免疫球蛋白A肾病可发生于各种分期的血液系统恶性肿瘤。该病例说明了对免疫球蛋白A肾病患者进行细致血液系统检查的重要性。认识副肿瘤性免疫球蛋白A肾病并早期诊断相关恶性肿瘤可能挽救生命。
