Abstract
Angioimmunoblastic T-cell lymphoma is one of the most common subtypes of peripheral T-cell lymphoma (15-20% of all cases), accounting for approximately 1-2% of all non-Hodgkin lymphomas. It often presents autoimmune phenomena including hemolytic anemia, thrombocytopenia, glomerulonephrities and circulating immune complexes. Polyarteritis nodosa is an autoimmune disease characterized by necrotizing vasculitis of medium vessels, which rarely develops in association with hematological malignant disorders. Herein we report the case of a 40-year-old man who underwent lymph node biopsy in the suspicious of sarcoidosis. On the basis of histological and immunohistochemical findings, the diagnosis of angioimmunoblastic T-cell lymphoma was performed. The patient was successfully treated with cytarabine-based regimen for 6 cycles. Three months after the initial diagnosis of angioimmunoblastic T-cell lymphoma, a whole body computed tomography showed a lesion in the lower pole of the left kidney. Renal cell carcinoma was suspected, thus a nephrectomy was carried out. The histological findings were compatible with polyarteritis nodosa. To the best of our knowledge, the association between polyarteritis nodosa and angioimmunoblastic T-cell lymphoma has been described only once. This relation may be secondary to the induction of an autoimmune phenomenon by the lymphoma with the formation of circulating immune complexes, leading to vessels walls injury. A careful evaluation is needed in the management of angioimmunoblastic T-cell lymphoma patients with signs of renal failure in order to avoid delay of treatment and organ damage.
Highlights
The World Health Organization (WHO) classification of tumors of hematopoietic and lymphoid tissues defines the Angioimmunoblastic T-cell lymphoma (AITL) as one of the more common specific subtypes of peripheral T-cell neoplasms, accounting for approximately 15-20% of all cases, or 1-2% of all non-Hodgkin lymphomas [1]
During the clinical course of AITL, proliferative glomerulonephritis [3], minimal-change disease [4,5], A-type amyloidosis [2], acute renal failure [6], immunoglobulin (Ig)M-λ glomerular thrombi, and membranoproliferative glomerulonephritis-like lesions [7], myeloma-like kidney [8], direct invasion by lymphoma cells [9], interstitial nephritis [10,11], vasculitis [12,13], nephrocalcinosis [14], Fanconi syndrome [15] and nephrotic syndrome due to membranous nephropathy (MN) [16] may be rarely observed.To the best of our knowledge, there is only one previous case of AITL associated with polyarteritis nodosa (PAN) [17]
On the basis of clinical presentation and histological findings, the diagnosis of angioimmunoblastic T-cell lymphoma stage IVB was made according to the criteria of WHO classification [1]
Summary
The World Health Organization (WHO) classification of tumors of hematopoietic and lymphoid tissues defines the Angioimmunoblastic T-cell lymphoma (AITL) as one of the more common specific subtypes of peripheral T-cell neoplasms, accounting for approximately 15-20% of all cases, or 1-2% of all non-Hodgkin lymphomas [1]. After 3 months a restage whole body computed tomography (CT)-scan was performed which showed the disappearance of all previous pathologic findings, but evidenced a lesion of 45 mm in the lower pole of the left kidney (Figure 1). The patient was discharged from hospital without complications He completed the chemotherapy induction program (4 cycles) and underwent consolidation with high-dose chemotherapy and autologous stem cells transplantation. Macroscopic examination of the left kidney specimen showed a large pale area at the lower pole, approximately 4 cm in maximum diameter with a triangular morphology, centered on the renal cortex and consistent with an infarcted area (Figure 3A).
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