免疫缺陷相關多形性淋巴增殖性疾病的腎臟侵犯：罕見病例報告

曾博鴻、黃勝賢

彰化基督教醫院 外科部 泌尿科

Renal involvement in immunodeficiency-associated polymorphic lymphoproliferative disorder: a rare case report

Po-Hung Tseng, Sheng-Hsien Huang

Divisions of Urology, Department of Surgery, Changhua Christian Hospital, Changhua, Taiwan

Introduction

Polymorphic lymphoproliferative disorders refer to a group of heterogeneous conditions characterized by abnormal growth of lymphocytes in various organs and tissues of the body. These disorders can involve B cells, T cells, or natural killer cells. Immunodeficiency-associated polymorphic lymphoproliferative disorders can involve renal manifestations, although they are relatively rare. These disorders encompass a spectrum of conditions where the immune system is compromised, leading to abnormal proliferation of lymphoid cells. It can manifest in several ways such as interstitial nephritis, glomerulonephritis, renal tumors and obstructive uropathy.

Case report

A 64-year-old male with history of asthma, type 2 diabetes mellitus and cervical cancer post-surgical hysterectomy and radiation therapy.

The female patient presented to the internal medicine outpatient clinic with intermittent low-grade fever over several weeks. Abdominal ultrasonography revealed masses in the left kidney and adrenal gland. Due to a known contrast allergy, she underwent renal magnetic resonance imaging showed a 5.5 x 4.9 x 7.7-cm mass lesion in left kidney direct invasion of left adrenal gland, and encasing ipsilateral renal vessels. Metastatic paraaortic lymph nodes were suspected. CT-guided biopsy reported lymphohistiocytic aggregate. Under the impression of renal tumor, malignancy could not be ruled out, she received robotic assisted single port laparoscopic radical nephrectomy, converted to hand-assisted method due to inflammation and severe adhesion noted during operation. Final pathology report showed polymorphic lymphoproliferative disorder associated to immune deficiency.

Discussion

Management of renal involvement in immunodeficiency-associated PLPD requires a multidisciplinary approach involving oncologists, nephrologists, and other specialists. Treatment strategies may include immunosuppressive therapy, chemotherapy, targeted therapy, and supportive care for renal dysfunction. Prognosis depends on the underlying PLPD subtype, extent of renal involvement, and response to treatment. Early recognition and management of renal complications are essential for optimizing outcomes in these patients.