Nodular lymphocyte predominant Hodgkin lymphoma

Nodular lymphocyte predominant Hodgkin lymphoma
Micrograph of a lymph node biopsy showing nodular lymphocyte predominant Hodgkin lymphoma, with the Reed–Sternberg cell variant that has a popcorn-shaped nucleus (left-bottom of image). H&E stain.
SpecialtyHematology and oncology
Frequency0.1-0.2 cases / 100000 per year

Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is a slow-growing CD20 positive form of Hodgkin lymphoma, a cancer of the immune system's B cells.[1][2]

NLPHL is an uncommon sub-type of Hodgkin lymphoma, making up 5-10% of Hodgkin lymphomas.[3] It is distinguished from classic Hodgkin lymphoma by the presence of CD20 positive lymphocyte predominant cells, also known as popcorn cells due to their polylobated nuclei, which look like popcorn.[3][4][5]

There are small but clear differences in prognosis between the various forms. Lymphocyte predominant HL is an uncommon subtype composed of vague nodules of numerous reactive lymphocytes admixed with large popcorn-shaped RSC. Unlike classic RSC, the non-classic popcorn-shaped RS cells of NLPHL are CD15 and CD30 negative while positive for the B cell marker CD20. The anti-CD20 monoclonal antibody Rituximab has been used in lymphocyte predominant Hodgkin lymphoma with encouraging results.[6]

BCL6 gene rearrangements have been frequently observed.[7][8]

  1. ^ Renné C, Martín-Subero JI, Hansmann ML, Siebert R (August 2005). "Molecular Cytogenetic Analyses of Immunoglobulin Loci in Nodular Lymphocyte Predominant Hodgkin's Lymphoma Reveal a Recurrent IGH-BCL6 Juxtaposition". J Mol Diagn. 7 (3): 352–6. doi:10.1016/S1525-1578(10)60564-8. PMC 1867541. PMID 16049307.
  2. ^ Achten R, Verhoef G, De Wolf-Peeters C (December 2003). "The importance of follow-up biopsies of clinically suspicious lymphadenopathy in nodular lymphocyte predominant Hodgkin's lymphoma". Haematologica. 88 (12): 1431–2. PMID 14688002.
  3. ^ a b Wang, Hao-Wei; Balakrishna, Jayalakshmi P; Pittaluga, Stefania; Jaffe, Elaine S. (January 2019). "Diagnosis of Hodgkin Lymphoma in the Modern Era". British Journal of Haematology. 184 (1): 45–59. doi:10.1111/bjh.15614. ISSN 0007-1048. PMC 6310079. PMID 30407610.
  4. ^ Stier JR, Vasquez RJ (2015). "Lymphocyte-Predominant Hodgkin's Disease in Children: A Case Study and Review of the Literature". Case Reports in Oncological Medicine. 2015: 351431. doi:10.1155/2015/351431. PMC 4388010. PMID 25878913.
  5. ^ Eichenauer, Dennis A.; Engert, Andreas (2020-12-24). "How I treat nodular lymphocyte-predominant Hodgkin lymphoma". Blood. 136 (26): 2987–2993. doi:10.1182/blood.2019004044. ISSN 0006-4971. PMID 32877522. S2CID 221476874.
  6. ^ Saini KS, Azim HA Jr, Cocorocchio E, Vanazzi A, Saini ML, Raviele PR, Pruneri G, Peccatori FA (2011). "Rituximab in Hodgkin lymphoma: Is the target always a hit?". Cancer Treat Rev. 37 (5): 385–90. doi:10.1016/j.ctrv.2010.11.005. PMID 21183282.
  7. ^ Wlodarska I, Stul M, De Wolf-Peeters C, Hagemeijer A (August 2004). "Heterogeneity of BCL6 rearrangements in nodular lymphocyte predominant Hodgkin's lymphoma". Haematologica. 89 (8): 965–72. PMID 15339680.
  8. ^ Wlodarska I, Nooyen P, Maes B, et al. (January 2003). "Frequent occurrence of BCL6 rearrangements in nodular lymphocyte predominance Hodgkin lymphoma but not in classical Hodgkin lymphoma". Blood. 101 (2): 706–10. doi:10.1182/blood-2002-05-1592. PMID 12393409.
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