Rare Disease — Nodular Lymphocyte-Predominant Hodgkin’s Lymphoma: Literature Review and Own Data

LYMPHOID TUMORS , , , ,

E.A. Demina1, G.S. Tumyan1, A.A. Chekan1, M.Yu. Kichigina1, A.S. Antipova1, N.A. Probatova1, A.I. Pavlovskaya1, N.V. Kokosadze1, A.M. Kovrigina2, O.P. Trofimova1, E.A. Osmanov1

1 N.N. Blokhin Russian Cancer Research Center, 24 Kashirskoye sh., Moscow, Russian Federation, 115478

2 Hematology Research Center under the Ministry of Health of the Russian Federation, 4а Novyi Zykovskii pr-d, Moscow, Russian Federation, 125167

For correspondence: E.A. Demina, DSci, Professor, 24 Kashirskoye sh., Moscow, Russian Federation, 115478; Tel: +7(499)324-90-89; e-mail: drdemina@yandex.ru

For citation: Demina E.A., Tumyan G.S., Chekan A.A., Kichigina M.Yu., Antipova A.S., Probatova N.A., Pavlovskaya A.I., Kokosadze N.V., Kovrigina A.M., Trofimova O.P., Osmanov E.A. Rare Disease — Nodular Lymphocyte-Predominant Hodgkin’s Lymphoma: Literature Review and Own Data. Klin. Onkogematol. 2014; 7(4): 522–532 (In Russ.).

ABSTRACT

Nodular lymphocyte-predominant Hodgkin’s lymphoma (NLPHL) is a rare disorder; it constitutes only 5 % of all cases of Hodgkin’s lymphoma (the morbidity rate is 1.5 per one million). The disease differs from classical Hodgkin’s lymphoma (cHL) in both immunohistochemical (marked CD20 expression in LP cells) and clinical features (prevalence of the early stage disease, indolent course with delayed relapses and trends toward transformation into diffuse large B cell lymphoma). Since the number of patients in prospective NLPHL-focused trials is limited, treatment algorithms have been based on retrospective data; these are usually obtained from cHL and indolent B cell lymphoma treatment strategies. Patients rarely die from NLPHL; in fact, secondary malignancies and other treatment related toxicities generally contribute to overall mortality. Over the past decade, there has been a series of NLPHL-related publications describing prescription of rituximab for newly diagnosed diseases and for relapses, including patients at high risk of diseases transformation. Besides, the role of the “wait and watch”, radiation treatment, and chemotherapy has been discussed. Our own experience in the use of rituximab in NLPLH patients demonstrated its efficacy at different stages and at different phases of the disease.

Keywords: nodular lymphocyte-predominant Hodgkin’s lymphoma, diagnosis, clinical characteristics, treatment, rituximab.

Accepted: September 8, 2014

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