Renal infarction due to polyarteritis nodosa in a patient with angioimmunoblastic T-cell lymphoma: a case report and a brief review of the literature.

Diagnostic Pathology (Impact Factor: 1.85). 05/2012; 7(1):50. DOI: 10.1186/1746-1596-7-50
Source: PubMed

ABSTRACT Angioimmunoblastic T-cell lymphoma (AITL) 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 (CIC). Polyarteritis nodosa (PAN) 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 male patient with AITL who had a renal infarction secondary to PAN, mimicking a neoplastic lesion. A 40-year-old man underwent lymph node biopsy in the suspicious of sarcoidosis. On the basis of histological and immunohistochemical findings, a diagnosis of AITL was performed. The patient was successfully treated with a cytarabine-based regimen for 6 cycles. Three months after the initial diagnosis of AITL, a whole body CT-scan showed a lesion in the lower pole of the left kidney. A 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 PAN and AITL 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 vessel walls injury. A careful evaluation is needed in the management of AITL patients with signs of renal failure in order to avoid delay of treatment and organ damage. Key words: renal infarction, polyarteritis nodosa, T-cell lymphoma.

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    ABSTRACT: INTRODUCTION: Polyarteritis nodosa is a rare disease resulting from blood vessel inflammation (vasculitis), causing damage to organ systems and featuring an extended range of possible symptoms. The cause of polyarteritis nodosa is unknown. CASE PRESENTATION: In the present report we describe the presentation and treatment of polyarteritis nodosa involving the hard palate in an 88-year-old Caucasian woman. Clinical and laboratory analyses showed stenosis of the greater palatine artery, which led to necrosis of the affected area. At one year after pharmacological treatment, the lesion has regressed completely. CONCLUSIONS: We successfully treated a case of polyarteritis nodosa via a pharmacological approach, which we describe here.
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