Nodular lymphocyte predominant Hodgkin lymphoma in children and adolescents – a comprehensive review of biology, clinical course and treatment options

Abstract

SummaryNodular lymphocyte predominant Hodgkin lymphoma (nLPHL) is a unique variant of Hodgkin lymphoma with an overall good prognosis. It is conspicuously different from classical Hodgkin lymphoma (cHL) and is now recognized as distinctive form of B cell lymphoma. Although it has an indolent clinical course, it has a propensity for multiple and often late relapses. Although the majority of children present with early stage disease and without B symptoms, treatment strategy has, until recently, been identical to that used for cHL. This approach is excessively toxic as it predisposes these children and adolescents to serious late effects including end organ damage to heart, gonads, lungs, thyroid and second malignant neoplasms. The aim of this article is to review the published literature on the treatment outcomes of nLPHL in affected children and adolescents, and discuss the options for treatment including surgery, chemotherapy, radiotherapy and targeted anti-CD 20 antibody therapy.

How I treat nodular lymphocyte predominant Hodgkin lymphoma

“…However, its distinction from NLPHL is reliably possible by immunophenotyping, as the transformed germinal centers do not harbor cells expressing the typical immunophenotype of LP cells, and occasionally, PTGC and NLPHL may exist in the same lymph node. 17 …”

Thomas Hodgkin and Hodgkin lymphoma

“…Among prepubertal children, it accounts for a higher proportion of Hodgkin lymphomas [11,12]. There is a male/female ratio of 3:1.…”

“…Typically, there are long disease-free intervals, but relapses are common. This is an indolent disease with excellent survival in most cases, and the intermittent isolated relapses are not usually associated with poor overall survival [10,12]. Patients who present with high-stage disease typically do not have a favorable outcome [12,13].…”

“…This is an indolent disease with excellent survival in most cases, and the intermittent isolated relapses are not usually associated with poor overall survival [10,12]. Patients who present with high-stage disease typically do not have a favorable outcome [12,13]. Bone marrow involvement is quite uncommon; it appears to be found only when there is other clinical or pathologic evidence of aggressive disease [14].…”

“…There is evidence of activation of both nuclear factor-kappa B (NF-κB) and Janus kinase/signal transducer and activator of transcription (JAK/STAT) signaling pathways; inactivating mutations of suppressor of cytokine signaling 1 (SOCS1) have been documented that are believed to contribute to JAK/STAT activation [12].…”