Progress in understanding the biology of nodular lymphocyte-predominant Hodgkin lymphoma

Straus, David J.

doi:10.3324/haematol.2021.278785

Cited by 1 publication

(1 citation statement)

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“…Many differences distinguish cHL and NLPHL, leading to the consideration of NLPHL as a low-grade B-cell lymphoma. However, as with cHL, GEP demonstrated that the lymphocyte predominant (LP) cells in NLPHL show a strong NFkB activity signature, but this activation is a consequence of different mechanisms than those observed in cHL [ 70 , 71 ].…”

Section: Specificities Of Nodular Lymphocyte Predominant Hlmentioning

confidence: 99%

NFkB Pathway and Hodgkin Lymphoma

Jardin

2022

Biomedicines

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The tumor cells that drive classical Hodgkin lymphoma (cHL), namely, Hodgkin and Reed-Sternberg (HRS) cells, display hallmark features that include their rareness in contrast with an extensive and rich reactive microenvironment, their loss of B-cell phenotype markers, their immune escape capacity, and the activation of several key biological pathways, including the constitutive activation of the NFkB pathway. Both canonical and alternative pathways are deregulated by genetic alterations of their components or regulators, EBV infection and interaction with the microenvironment through multiple receptors, including CD30, CD40, BAFF, RANK and BCMA. Therefore, NFkB target genes are involved in apoptosis, cell proliferation, JAK/STAT pathway activation, B-cell marker expression loss, cellular interaction and a positive NFkB feedback loop. Targeting this complex pathway directly (NIK inhibitors) or indirectly (PIM, BTK or NOTCH) remains a challenge with potential therapeutic relevance. Nodular predominant HL (NLPHL), a distinct and rare HL subtype, shows a strong NFkB activity signature because of mechanisms that differ from those observed in cHL, which is discussed in this review.

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Section: Specificities Of Nodular Lymphocyte Predominant Hlmentioning

confidence: 99%