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

机译:血管免疫母细胞性T细胞淋巴瘤患者因结节性多动脉炎引起的肾梗死:病例报告和文献简述。

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摘要

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.
机译:摘要:血管免疫母细胞性T细胞淋巴瘤(AITL)是周围性T细胞淋巴瘤最常见的亚型之一(占所有病例的15-20%),约占所有非霍奇金淋巴瘤的1-2%。它经常表现出自身免疫现象,包括溶血性贫血,血小板减少,肾小球肾炎和循环免疫复合物(CIC)。结节性多发性动脉炎(PAN)是一种自身免疫性疾病,其特征是中血管坏死性血管炎,很少伴随血液系统恶性疾病发展。在此,我们报道一例AITL的男性患者,该患者患有继发于PAN的肾梗塞,模仿了肿瘤性病变。一名40岁男子因怀疑结节病而接受了淋巴结活检。根据组织学和免疫组织化学结果,对AITL进行了诊断。该患者已成功接受基于阿糖胞苷的方案治疗6个周期。初步诊断为AITL后三个月,全身CT扫描显示左肾下极有病变。怀疑有肾细胞癌,因此进行了肾切除术。组织学发现与结节性多发性动脉炎相容。据我们所知,PAN和AITL之间的关联仅被描述过一次。这种关系可能是继发于淋巴瘤诱发自身免疫现象并形成循环免疫复合物,导致血管壁损伤。在治疗有肾功能衰竭的AITL患者时,需要进行仔细评估,以避免治疗延迟和器官损伤。

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