Minimal change nephrotic syndrome and classical Hodgkin's lymphoma: Report of 21 cases and review of the literature

Abstract

Minimal change nephrotic syndrome (MCNS) is described as a paraneoplastic manifestation of classical Hodgkin's lymphoma (cHL). We reassessed the pathophysiological and clinical significance of this association. A retrospective study was performed to evaluate a cohort of adult patients who developed MCNS and cHL. Twenty-one patients recruited in 15 French centers were analyzed. cHL was associated with inflammatory and general symptoms in most cases. The morphological subtype was predominantly nodular sclerosis (71.4%). MCNS appeared before the diagnosis of lymphoma in eight patients (38.1%) and in this case, it was characterized by a nephrotic syndrome (NS) frequently resistant (50%) or dependent (12.5%) to steroid treatment. Interestingly, diagnosis (3-120 months after MCNS) and effective treatment of the hemopathy were associated with the disappearance of the MCNS. cHL was diagnosed before MCNS in nine patients (42.9%), and in this case, glomerulopathy was associated with cHL relapse in 55.5% of cases. In four patients (19%), the two diseases occurred simultaneously. Extensive immunohistochemical study of lymph nodes was performed in eight patients and did not reveal particular features. In conclusion, MCNS associated with cHL is frequently dependent or resistant to steroid regimen, but remission of NS is obtained with the cure of lymphoma.

Onco-Nephrology

“…Minimal-change disease can occur at the time of relapse even if it was initially absent, emphasizing the need to evaluate proteinuria during the follow-up of classic Hodgkin lymphoma. Between-disease interval can be as long as 156 months (36). No particular subgroup of patients with classic Hodgkin lymphoma seems to be at higher risk for minimal-change disease with respect to age, sex, or disease stage (except for systemic symptoms and inflammatory syndrome, which occur more frequently).…”

“…The nephrotic syndrome usually appears early, revealing the lymphoma in about 40% of cases, and displaying a high frequency of steroid resistance (50%) and cyclosporine resistance (36). Effective treatment of classic Hodgkin lymphoma generally induces simultaneous remission of nephrotic syndrome whatever the therapeutic strategy, even without corticosteroids.…”

“…No particular subgroup of patients with classic Hodgkin lymphoma seems to be at higher risk for minimal-change disease with respect to age, sex, or disease stage (except for systemic symptoms and inflammatory syndrome, which occur more frequently). Minimal-change disease seems to be more frequent in classic Hodgkin lymphoma that exhibits a mixed cellularity and is of the nodular sclerosing subtype (36,37). The pathogenesis of minimalchange disease seems to involve a putative circulating factor secreted by T lymphocytes, leading to cytoskeleton disorganization and heavy proteinuria (38).…”

CA 125 Elevation Due to Minimal Change Disease

“…Çoğunlukla primer olup nadiren ilaçlar (14,15), neoplazmlar (özellikle hematolojik neoplazmlar) (16)(17)(18)(19), hepatit C gibi enfeksiyonlar (15,20,21), alerji, diğer glomerüler hastalıklar (22)(23)(24)(25) ve bazı otoimmün hastalıklar gibi sekonder nedenlere bağlı olarak da ortaya çıkabilmektedir (20). Olgumuzda ilaç ve/veya sigara kullanım öyküsü yoktu.…”

A 17-Year-Old With Steroid-Resistant Nephrotic Syndrome

“…12 Approximately 0.1% to 0.5% of patients with Hodgkin lymphoma have nephrotic syndrome. 13 The predominant subtype of nephrotic syndrome has been minimal change disease. 13 …”