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Hodgkin lymphoma
Other Resources UpToDate PubMed

Hodgkin lymphoma

Contributors: Nina Haghi MD, Patricia Ting MD, Ben Barankin MD, Paritosh Prasad MD
Other Resources UpToDate PubMed

Synopsis

Hodgkin lymphoma is a hematopoietic neoplasm that was first described in 1832 by Sir Thomas Hodgkin in patients with weight loss, lymphadenopathy, and splenomegaly, resulting in death. The World Health Organization (WHO) categorizes Hodgkin lymphoma into 2 main types: classical Hodgkin lymphoma (which accounts for 90% of cases) and nodular lymphocyte predominant Hodgkin lymphoma.

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:
  1. Nodular sclerosis cHL (the most common subtype; > 70% of cases)
  2. Mixed cellularity cHL (up to 20%-25% of cases; more often associated with EBV)
  3. Lymphocyte-rich cHL (up to 5% of cases)
  4. Lymphocyte-depleted cHL (< 1% of cases; more often associated with EBV)
The hallmark histopathologic finding in cHL are Reed-Sternberg (RS) cells, which are large atypical cells with prominent cherry red nucleoli, often with a bilobed nucleus. They are derived from germinal center B-cells. The cellular background (lymphocyte rich / depleted, mixed cellularity, or sclerosis) is what differentiates the various subtypes of cHL.

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.

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References

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Last Reviewed:07/24/2020
Last Updated:07/30/2020
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