Don’t fight Hodgkin lymphoma alone.
Find your community on the free RareGuru App.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.
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Source: GARD Last updated on 05-01-20
80%-99% of people have these symptoms.
Click on a symptom to see definitions for associated terms.
Immunodeficiency |
Abnormality of the tibial metaphysis |
Fatigue |
Absent pigmentation of the ventral chest |
Lymphadenopathy |
Lymphoma |
30%-79% of people have these symptoms.
Click on a symptom to see definitions for associated terms.
Hyperhidrosis |
Pruritus |
Fever |
Cough |
Bruxism |
Generalized clonic seizures |
Abducens palsy |
Slender finger |
Recurrent opportunistic infections |
Anorexia |
Chest pain |
Weight loss |
5%-29% of people have these symptoms.
Click on a symptom to see definitions for associated terms.
Hepatomegaly |
Hyperphosphatemia |
Aplasia of the ulna |
Ankle flexion contracture |
Absent speech |
Hemiclonic seizures |
Ataxia |
Bone pain |
Dyspnea |
Headache |
Hemoptysis |
Peripheral neuropathy |
Splenomegaly |
An unknown % of people have these symptoms.
Click on a symptom to see definitions for associated terms.
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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