Nephrotic Syndrome Leading to the Diagnosis of Classical Hodgkin’s Lymphoma in a 13-Year-Old Girl

Introduction

Classical Hodgkin’s Lymphoma (cHL) is a monoclonal lymphoid neoplasm composed of mononuclear Hodgkin cells and multinucleated Hodgkin-Reed-Sternberg cells (HRSs), which are embedded in a reactive cellular background consisting of T cells, histiocytes, granulocytes, plasma cells, and fibroblasts. HRSs are derived from germinal center B cells, rarely from T cells (Kuppers R, Nat Rev Cancer 2009; 9: 15–27).

Nephrotic syndrome (NS) is a renal disorder defined by heavy urinary protein loss, hypoalbuminemia, high cholesterol and triglyceride levels and generalized edema (Eddy AA et al., Lancet 2003; 362: 629–639). A variety of mechanisms leading to glomerular injury in NS have been described such as mutations in podocyte slit diaphragm-associated proteins, circulating immune and non-immune factors. NS in patients with HL is rare and its pathogenesis so far only poorly understood. An immune origin has been suggested, as there is also a well established association of HL with autoimmune disorders such as rheumatoid arthritis, systemic lupus erythematosus, sarcoidosis and autoimmune cytopenias (Ishimoto T et al., Semin Nephrol 2011; 31: 320–325, Landgren O et al., J Natl Cancer Instit 2006; 98: 1321–1330). However, no clear pathomechanism has been established so far how HL leads to autoimmunity and vice versa. In completion to a former abstract on a 13-year-old girl who simultaneously presented with NS and cHL (Klin Padiatr 2011; 223 (Suppl. 1): S68–S69) we present additional clinical data and a compilation of other cases.