Atlani Mahendra, Sharma Tanya, Joshi Deepti, Kumar Aman
Department of Nephrology, All India Institute of Medical Sciences, Bhopal, Madhya Pradesh, India.
Department of Pathology and Lab Medicine, All India Institute of Medical Sciences, Bhopal, Madhya Pradesh, India.
Indian J Nephrol. 2021 Jan-Feb;31(1):50-53. doi: 10.4103/ijn.IJN_222_19. Epub 2021 Jan 27.
A case of prefibrotic myelofibrosis with immune complex-mediated glomerulonephritis is presented. A 45-year-old female, with history of right subclavian and axillary vein thrombosis, presented with abdominal distension, facial puffiness, and pedal edema. Evaluation revealed deranged renal functions with nephrotic range proteinuria and acute kidney injury. JAK2 mutation evaluated in view of portal vein thrombosis and splenomegaly was positive. Renal biopsy revealed mesangial proliferative glomerulonephritis with full house immune complex deposition on direct immunofluorescence (DIF). The patient had no signs or symptoms of systemic lupus erythematosus and serological markers for autoimmune or collagen vascular disease were negative. Renal involvement in myeloproliferative neoplasms (MPNs) is uncommon and histological patterns of DIF negative mesangial proliferative glomerulonephritis, focal segmental glomerulosclerosis, and immunoglobulin A nephropathy have been reported.
本文报告一例伴有免疫复合物介导的肾小球肾炎的纤维化前期骨髓纤维化病例。一名45岁女性，有右锁骨下静脉和腋静脉血栓形成史，出现腹胀、面部浮肿和足部水肿。评估显示肾功能紊乱，伴有肾病范围蛋白尿和急性肾损伤。鉴于门静脉血栓形成和脾肿大而检测的JAK2突变呈阳性。肾活检显示系膜增生性肾小球肾炎，直接免疫荧光（DIF）显示有满堂免疫复合物沉积。该患者无系统性红斑狼疮的体征或症状，自身免疫或胶原血管病的血清学标志物均为阴性。骨髓增殖性肿瘤（MPN）累及肾脏并不常见，已有报道其组织学表现为DIF阴性的系膜增生性肾小球肾炎、局灶节段性肾小球硬化和免疫球蛋白A肾病。
