Nephrotic syndrome: an unusual manifestation of classical Hodgkin’s Lymphoma in the adolescent

Objective: Hodgkin lymphoma (HL), is a malignant tumor of unknown etiology which is characterized by the presence of clonal multinuclear Hodgkin/Reed-Sternberg cells embedded in a mixture of reactive T-cells, histiocytes, eosinophils and plasma cells. HL has been shown to be associated with various autoimmune phenomena, e.g. autoimmune hemolytic anemia or immune-mediated thrombocytopenia. In about 1% of patients with classical Hodgkin lymphoma (cHL) minimal change nephrotic syndrome (MCNS) is diagnosed which is considered to be an immune-mediated glomerular disease. Recently, aberrant expression of c-mip, a protein involved in podocyte dysfunction in MCNS was found in patients with cHL and MCNS but not in cHL-patients without MCNS. We report on a 13-year old girl with simultaneous occurrence of cHL and nephrotic syndrome (NS).

Case Report: A previously healthy 13-year-old girl was diagnosed with cervical lymphadenitis and prescribed amoxicillin by her primary physician. Lymph nodes did not regress in size and 12 days later generalized edema developed. The girl was admitted to the local hospital and the diagnosis of NS was made. In order to clarify the etiology of NS, a lymph node biopsy was performed and led to the diagnosis of cHL of nodular-sclerosis subtype. The patient was then referred to our hospital where staging revealed stage IIA and treatment was started according to the protocol EuroNet-PHL-C1. NS already resolved during the first cycle of chemotherapy (OEPA) and cHL completely responded after two cycles omitting the need for radiotherapy. The patient has been now in complete remission for 10 months.

Conclusion: cHL can be a rare cause of nephrotic syndrome in children and adolescents. Therefore, in the presence of nephrotic syndrome and lymph node enlargement of unknown etiology, a lymph node biopsy is warranted.

Nephrotic syndrome - classical Hodgkin´s lymphoma.