Hodgkin lymphoma
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Synopsis
Classical Hodgkin lymphoma: Classical Hodgkin lymphoma (cHL) may be associated with Epstein-Barr virus (EBV) infection, immune suppression, or autoimmune disease in some cases. It has bimodal age distribution, with one peak at 15-34 years of age and the second peak after age 50. EBV-associated cases are more commonly seen in children or immunocompromised patients (eg, HIV-infected patients) and has a higher incidence in developing countries.
cHL is made up of four subtypes:
- Nodular sclerosis cHL (the most common subtype; > 70% of cases)
- Mixed cellularity cHL (up to 20%-25% of cases; more often associated with EBV)
- Lymphocyte-rich cHL (up to 5% of cases)
- Lymphocyte-depleted cHL (< 1% of cases; more often associated with EBV)
Most patients with cHL will present with asymptomatic lymphadenopathy or finding of an incidental mass on chest radiography. Approximately 4 in 10 will have B symptoms (fever, night sweats, or weight loss) at the time of diagnosis. While the disease tempo is variable, it is generally slow, and symptoms of lymphadenopathy and B symptoms are recognized in retrospect to have begun weeks to months prior to the patient's evaluation for cHL.
For cHL, prognosis depends mostly on the stage of disease, with a 5-year survival rate of 90% for stage 1 or 2a. Stage 4 disease has a 5-year survival rate of 60%.
Nodular lymphocyte predominant Hodgkin lymphoma: Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is less common (about 5% of patients diagnosed with Hodgkin lymphoma). It is a more indolent lymphoma and often asymptomatic. It tends to involve peripheral lymph nodes. Extranodal involvement is less common. Morphologically, it is characterized by lymphohistiocytic RS cell variant, L&H cells, which are mummified-appearing large cells that are sometimes referred to as "popcorn" cells due to their irregular contours and washed-out chromatin pattern.
For NLPHL, prognosis is favorable, with complete remission reported in > 90% of patients with favorable early-stage disease, 86% in unfavorable early-stage disease, and 77% in advanced-stage disease.
Related topic: non-Hodgkin lymphoma
Codes
ICD10CM:C81.90 – Hodgkin lymphoma, unspecified, unspecified site
SNOMEDCT:
118599009 – Hodgkin's disease
Look For
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
Given the indolent and nonspecific symptoms that often herald cHL, the differential can be broad.- Other reactive process (infectious, autoimmune, inflammatory, drug induced)
- Other types of lymphomas (anaplastic large cell lymphoma, primary mediastinal B-cell lymphoma, mediastinal gray zone lymphoma, T-cell histiocyte-rich large B-cell lymphoma, among others)
- EBV-positive mucocutaneous ulcer
Best Tests
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Management Pearls
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Therapy
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Drug Reaction Data
Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.Subscription Required
References
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Last Reviewed:07/23/2020
Last Updated:07/29/2020
Last Updated:07/29/2020
Hodgkin lymphoma