Nodular lymphocyte predominant Hodgkin's lymphoma

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Nodular lymphocyte predominant Hodgkin's lymphoma
Classification and external resources
Popcorn cell in nodular lymphocyte predominant Hodgkin lymphoma - very high mag cropped.jpg
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.
ICD-10 C81.0
ICD-9 201.9
ICD-O: M9659/3

Nodular lymphocyte predominant Hodgkin's lymphoma (NLPHL) is a form of lymphoma.[1][2]

Hodgkin lymphoma, nodular lymphocyte predominant (low power view). Notice the nodular architecture and the areas of "mottling".(H&E)
Hodgkin lymphoma, nodular lymphocyte predominant (high power view). Notice the presence of L&H cells, also known as "popcorn cells". (H&E)

It is no longer classified as a form of classic Hodgkin's lymphoma (HL). This is because the Reed-Sternberg Cell (RSC) variants (popcorn cells) that characterize this form of the disease invariably express B lymphocyte markers such as CD20 (thus making NLPHL an unusual form of B cell lymphoma), and that (unlike classic HL) NLPHL may progress to diffuse large B cell lymphoma.

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's lymphoma with encouraging results.[3]

BCL6 gene rearrangements have been frequently observed.[4][5]

See also[edit]

References[edit]

  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. ^ 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. 
  4. ^ 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. 
  5. ^ 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.