IPGMER SSKM Kolkata.
J Assoc Physicians India. 2020 Jan;68(1):75.
Mantle Cell Lymphoma is a rare Non-Hodgkin, with unprecedented kidney involvement
case report
Herein IPGMER-SSKM Kolkata a 57-year-old male presented to us with Low-grade-intermittent fever & B-symptoms of 4months, arthralgia of 3months & oliguria of 10days. Mild pallor, mild hepatosplenomegaly & significant generalized lymphadenopathy. Hemoglobin 8.7% ESR 106 Uric acid 8.5 Potassium 6.3 Phosphate 4.5 Calcium 6.5 Urea 193 Creatinine 15 indicated Tumour Lysis Syndrome. Bilateral 10 cm kidney with Renal Parenchymal disease in USG. 25 RBC/hpf Albumin 3+ in Urine Study. 4.55gm protein per 24-hour-urine. Negative C3, C4, RF, anti-CCP, dsDNA. but 4+ ANCA, high 170 PR3. Crescents in 5 out of 8 glomeruli, focal interstitial lymphoid aggregates & (IgG, C3, C1q, IgM) positive Immune Complexes in Renal Biopsy. (CD20, CD5, BCL2, Cyclin-D1) Positive Mantle cell Lymphoma in Cervical Lymph Node Biopsy with 10% Ki67 index, High 6.18 MIPI score. R-CHOP regimen & Successive Hemodialysis improved condition of the patient. Discussion: paraneoplastic glomerulonephritis & direct lymphocytic infiltration lead to RPGN in our patient
To our knowledge, this is possibly first case report of a Mantle cell lymphoma presenting with RPGN due to both ANCA positive crescentic glomerulonephritis & lymphoid cell infiltration of Interstitium.
套细胞淋巴瘤是一种罕见的非霍奇金淋巴瘤，具有前所未有的肾脏受累。
病例报告。
在加尔各答的 IPGMER-SSKM，一名 57 岁男性患者因低级别间歇性发热和 B 症状（4 个月）、关节炎（3 个月）和少尿（10 天）就诊。轻度苍白、轻度肝脾肿大和明显全身淋巴结肿大。血红蛋白 8.7%、红细胞沉降率 106 U、尿酸 8.5、钾 6.3、磷 4.5、钙 6.5、尿素 193、肌酐 15，提示肿瘤溶解综合征。双侧 10cm 肾脏，超声显示肾实质疾病。尿沉渣检查每高倍镜视野（hpf）有 25 个红细胞，尿蛋白 3+，24 小时尿蛋白 4.55gm。C3、C4、RF、抗 CCP、dsDNA 阴性，但 4+ANCA、PR3 高 170。8 个肾小球中有 5 个出现新月体，局灶性间质淋巴样聚集和（IgG、C3、C1q、IgM）阳性免疫复合物，肾活检阳性。颈淋巴结活检显示（CD20、CD5、BCL2、Cyclin-D1）阳性套细胞淋巴瘤，Ki67 指数 10%，高 6.18 MIPI 评分。R-CHOP 方案和连续血液透析改善了患者的病情。讨论：副肿瘤性肾小球肾炎和直接淋巴细胞浸润导致我们的患者发生 RPGN。
据我们所知，这可能是首例因 ANCA 阳性新月体肾小球肾炎和间质淋巴细胞浸润导致 RPGN 的套细胞淋巴瘤病例报告。
