[Nodular lymphocyte predominant Hodgkin lymphoma (paragranuloma of Poppema) in children: Case report, review of the literature and treatment]

Abstract

We present the case of a 12 year old child with a limp. The diagnostic work-up reveals splenomegaly, multifocal bone involvement and abdominal adenopathies. A biopsy of an intra-abdominal lesion shows a lymphoid mass with a nodular architecture composed of poorly defined nodules. We identify large cells with irregular, sometimes poly-lobulated nuclei with a particular immunohistochemical profile. Those "pop-corn" cells are positive for CD20, CD79a, pax-5 and bcl-6 and are negative for CD15, CD30, bcl-2, TdT, CD56 and EMA. There is a diffuse follicular helper T cell population that is located in between the tumour cells. The overall picture is indicative of a nodular lymphocyte predominant Hodgkin lymphoma. Advanced stage of this disease is rare in children and there is currently little data to guide optimal treatment. Because of a stage IV disease, the patient is treated with chemotherapy after which complete metabolic remission is observed. 3.5 years after the initial diagnosis, our patient relapses. He is treated with chemotherapy and an autologous peripheral blood stem cell transplantation. He remains in complete remission since then. This case illustrates the favorable prognosis of the disease even after relapse.

Keywords: Autogreffe de cellules souches hématopoïétiques périphériques (CSHP); Autologous peripheral blood stem cell transplantation (PBSCT); Cellules popcorn; Cellules à prédominance lymphocytaire (cellules LP); Chemotherapy; Chimiothérapie; Lymphocyte predominant cells (LP cells); Lymphome de Hodgkin nodulaire à prédominance lymphocytaire (LHNPL); Lymphome de l’enfant; Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL); Paediatric lymphoma; Popcorn cells.