Angioimmunoblastic Lymphoma With Proliferative Glomerulonephritis: A Rare Association

Abstract

Abstract Introduction Angioimmunoblastic T-cell lymphoma (AILT) is a subtype of peripheral T-cell lymphomas characterized by a polymorphous infiltrate involving lymph nodes, with a prominent proliferation of high endothelial venules and follicular dendritic cells along with systemic disease. It accounts for only 1%-2% of non-Hodgkin lymphomas. It usually involves peripheral lymph nodes, liver, spleen, bone marrow, and skin. Proliferative glomerulonephritis with AILT is extremely rare and is reported in only 5 cases in the literature. We describe a unique case of proliferative glomerulonephritis without immune deposits in a patient with AILT. Patients and Methods A 68-year-old male presented with complaints of shortness of breath, fever, and weakness since 3 months. Physical examination revealed generalized lymphadenopathy with multiple, nontender, mobile lymph nodes with average size between 2-5 cm and hepatosplenomegaly. 1+ pitting edema was noted on the lower extremities, and skin examination revealed evidence of generally distributed pigmented skin macules associated with itching. Patient clinical course was worsened by a progressive renal failure and nephrotic range proteinuria. Axillary lymph node biopsy revealed architectural effacement, vascular growth with monoclonal T-cell population with T-cell receptor gamma gene restriction consistent with angioimmunoblastic T-cell lymphoma. Kidney biopsy revealed proliferative glomerulonephritis with increased mesangial cellularity and endocapillary proliferation and mild interstitial fibrosis. Immunofluorescence showed focal 1+ C3 staining in the glomerular capillary walls and mesangium. There were no immune deposits on electron microscopy. Patient required 1 episode of dialysis with improvement in renal function. A single cycle of chemotherapy with the CHOP regimen (cyclophosphamide, doxorubicin, vincristine, and prednisone) was given. However, the patient developed neutropenic sepsis and died. Results The occurrence of proliferative glomerulonephritis with AILT is extremely rare. Most of the cases described so far had immune deposits. Our case is unique since the kidney biopsy did not show any evidence of immune deposits at any site. Mechanisms proposed to explain the renal lesions in lymphoid malignancies include expression of tumor antigens, autologous nontumor antigens, viral or fetal antigens, immune complex deposition and disordered T-cell function. Conclusion In our opinion, cell-mediated immune reaction and/or alternate complement pathway activation (as evidenced by focal C3 staining) might have contributed to the glomerulonephritis in this case. The number of cases with this presentation is very few and the optimum management is not known. This case adds to the literature and helps us in better understanding the disease.