Hodgkin lymphoma
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Description
The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs.
Orpha Number: 98293
Definition
Hodgkin lymphoma (HL) is a heterogeneous group of malignant lymphoid neoplasms of B-cell origin characterized histologically by the presence of Hodgkin and Reed-Sternberg (HRS) cells in the vast majority of cases.
Epidemiology
HL is an uncommon cancer with an incidence of about 1/40,000 in North America and Europe. There are about 8500 new cases reported in the U.S. each year.
Clinical description
HL is comprised of 2 major forms: classical Hodgkin lymphoma (CHL; see this term), seen in 95% of all HL cases, and nodular lymphocyte predominant Hodgkin lymphoma (NLPHL; see this term), seen in only 5% of all HL cases. Disease onset occurs most frequently in young adults (age 15-35) followed by older adults (over the age of 55) and usually begins with the painless swelling of a lymph node in the upper body. Disease can spread to multiple lymph node regions or lymphoid structures and associated systemic symptoms (weight loss > 10% of baseline, fevers, night sweats) are observed in about 20% of patients.
Etiology
The exact cause is unknown but immunological, genetic and environmental factors are thought to be involved.
Management and treatment
Treatment with radiotherapy and systemic chemotherapy leads to a very good prognosis. Follow-up physical exams, blood tests and x-rays are needed to check for recurrence and check for any long-term side effects of the toxic treatments. Possible long-term side effects include secondary cancers, infertility, reduced immunity, thyroid problems, heart disease and stroke.
Prognosis
The international prognostic score (IPS) is a score given based on the presence of 7 risk factors (male sex, >45 years of age, stage 4 disease, serum albumin <4g/dl, hemoglobin <10.5 g/l, lymphocytopenia and WBC count
15,000/mm3) at the time of diagnosis. The more factors present, the less favorable the prognosis. The cure rates are 90% for early-stage HL and 70% for advance stage HL.
Visit the Orphanet disease page for more resources.
Source: GARD Last updated on 05-01-20
Symptoms
Symptoms and their Known Frequencies
New York, NY, 10005, United States Phone: +1-212-349-2910 Toll Free: 1-800-500-9976 Fax : +1-212-349-2886 Email: helpline@lymphoma.org Url: https://www.lymphoma.org/ Name: Leukemia and Lymphoma Society 3 International Drive, Suite 200
Rye Brook, NY, 10573, United States Phone: 1-(888) 557-7177 (general) Toll Free: 1-(800) 955-4572 (patients and families) Email: https://www.lls.org/content/contact-us Url: https://www.lls.org/ Name: Lymphoma Coalition 8 Stavebank Road N, Unit #401
Mississauga, Ontario, L5G 2T4, Canada Email: https://www.lymphomacoalition.org/contact/contact-us Url: https://lymphomacoalition.org Name: Lymphoma Action 3 Cromwell Court New Street
Aylesbury, HP20 2PB, United Kingdom Phone: 0808 808 5555 Toll Free: 0808-808-5555 Email: information@lymphoma-action.org.uk Url: https://lymphoma-action.org.uk
Note, these links are external searches against the National Laboratory of Medicine's drug database. You may need to adjust the search if there are no results found.
| Drug Name | Generic Name |
|---|---|
| Adcetris | brentuximab vedotin |
| Opdivo | nivolumab |
| Keytruda | pembrolizumab |
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