Hodgkin lymphoma (HL) is a neoplasm of the lymphatic system representing one of the common cancers in young adults; characterized by a low number of malignant cells deriving from B lymphocytes and an extensive inflammatory microenvironment
- Historical background:
- Described first in 1832 by Thomas Hodgkin about a series of six patients with clinical findings different from those with tuberculosis, syphilis, and inflammation
- German pathologist Carl Sternberg (1898) and American pathologist Dorothy Reed (1902) independently provided accounts of the giant “Reed-Sternberg” (RS) cells—the microscopic hallmarks of Hodgkin lymphoma.
- Initially treated with herbs, surgery, and arsenic in the 19th century; noted to shrink upon exposure to x-rays in the beginning of the 20th century
- Chemotherapy instituted as first-line treatment in the early 1970s with the introduction of two different regimen—MOPP (mechlorethamine, vincristine [Oncovin], procarbazine, prednisone) and ABVD (doxorubicin [Adriamycin], bleomycin, vinblastine, and dacarbazine); noted to have 80–90% remission rates
- Two subtypes:
- Classical Hodgkin lymphoma (cHL)—95% of cases; includes nodular sclerosing, mixed cellularity, lymphocyte rich, and lymphocyte depleted
- Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL)—5% of cases
- Two subtypes:
- Incidence: 2 to 3 per 100,000 per year
- Predominance: 11% of all lymphoid malignancies
- Has a bimodal age distribution—between 20 and 40 years and a second peak at around 55 years; typically diagnosed at age 20 to 34 years with median age 39 years at diagnosis given decreasing bimodal age distribution
- 1.3:1 male-to-female ratio
Etiology and Pathophysiology
- cHL is a B-cell lymphoma of germinal center origin that has lost its B-cell phenotype.
- RS cells harbor clonal rearrangements of hypermutated, class-switched immunoglobulin genes resulting in nonfunctional immunoglobulin genes lacking the expression of the cell surface B-cell receptor. In a healthy B cell, this should lead to apoptosis; however, in HL, these cells appear to be “rescued” from apoptosis by additional oncogenic events.
- NLPHL lacks typical RS cells but has lymphocytic and histiocytic cells, characterized by larger cells with folded multilobulated nuclei (“popcorn cells” or LP cells)—show a nucleus with multiple nucleoli that are basophilic and smaller than RS cells.
- Genome-wide association studies identified 19p13.3 at intron 2 of TCF3.
- First-degree relative: 3 to 9 times risk
- Siblings of younger patients: 7 times risk
- Weak correlation between familial HL and HLA class I regions containing HLA-A1, HLA-B5, HLA-B8, HLA-B18 alleles
- HIV: increased risk of HL in patients who are HIV positive
- Epstein-Barr virus (EBV): detected in nearly 45% patients with HL
- Genetic predisposition: significantly increased risk in identical twins indicating role of genetics in HL
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Domino, Frank J., et al., editors. "Hodgkin Lymphoma." 5-Minute Clinical Consult, 27th ed., Wolters Kluwer, 2020. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/117595/all/Hodgkin_Lymphoma.
Hodgkin Lymphoma. In: Domino FJF, Baldor RAR, Golding JJ, et al, eds. 5-Minute Clinical Consult. Wolters Kluwer; 2020. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/117595/all/Hodgkin_Lymphoma. Accessed June 1, 2023.
Hodgkin Lymphoma. (2020). In Domino, F. J., Baldor, R. A., Golding, J., & Stephens, M. B. (Eds.), 5-Minute Clinical Consult (27th ed.). Wolters Kluwer. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/117595/all/Hodgkin_Lymphoma
Hodgkin Lymphoma [Internet]. In: Domino FJF, Baldor RAR, Golding JJ, Stephens MBM, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2020. [cited 2023 June 01]. Available from: https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/117595/all/Hodgkin_Lymphoma.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Hodgkin Lymphoma ID - 117595 ED - Domino,Frank J, ED - Baldor,Robert A, ED - Golding,Jeremy, ED - Stephens,Mark B, BT - 5-Minute Clinical Consult, Updating UR - https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/117595/all/Hodgkin_Lymphoma PB - Wolters Kluwer ET - 27 DB - Medicine Central DP - Unbound Medicine ER -