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Hodgkin lymphoma - Skin
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Hodgkin lymphoma - Skin

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Contributors: Patricia Ting MD, Ben Barankin MD, Jeffrey D. Bernhard MD, Noah Craft MD, PhD, Lindy P. Fox MD, Lowell A. Goldsmith MD, MPH, Michael D. Tharp MD
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Synopsis

Hodgkin's disease (HD), also known as Hodgkin lymphoma, 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 HD into classical and nodular lymphocyte predominant HD (NLPHD). Classical HD includes nodular sclerosis (the most common subtype; greater than 50% of cases), mixed cellularity (up to 30%), lymphocyte-rich classical Hodgkin's lymphoma (up to 5%), and lymphocyte-depleted classical Hodgkin's lymphoma (up to 1%). NLPHD is rare and often presents with a lack of Reed-Sternberg cells. Pathologically, HD is classified as a malignant B cell lymphoma affecting the reticuloendothelial system. Reed-Sternberg cells, a type of histiocyte, are usually derived from B-lymphocytes and are often (not always) found within lymph nodes as well as skin biopsies (for cutaneous HD). Occasionally, malignant T cells have been implicated in HD.

Genetic, environmental, and immunologic factors appear to be responsible in the etiopathogenesis. Incidence of HD is estimated to be approximately 8,500 cases per year in the United States. HD affects males and females equally with bimodal peaks in late adolescence (age 15-30) and adult middle age (age 45-55). HD occurs in higher frequencies in first-degree relatives and monozygotic twins, and a small number of cases have been linked to Epstein-Barr virus (EBV). 

Classically, HD presents with constitutional ("B") symptoms (fever, night sweats, and unexplained weight loss), lymphadenopathy, and splenomegaly. Lymphadenopathy is usually asymptomatic. Cutaneous HD is exceedingly rare, but paraneoplastic eruptions are not uncommon.

The Ann Arbor staging system for HD is frequently used:
  • Stage I - Single lymph node region or single extranodal site
  • Stage II - Two or more lymph node regions on the same side of the diaphragm, or single lymph node region and contiguous site
  • Stage III - Lymph node regions on both sides of the diaphragm and may include spleen and/or limited contiguous extralymphatic organ or site
  • Stage IV - Disseminated involvement of one or more extralymphatic organs (liver, bone marrow, lung) or tissues with or without associated lymph node (or spleen) involvement.
Following the designated Roman numeral stage (I to IV), either A or B and/or E designations may follow (ie, Stage IA). A and B indicate the absence and presence, respectively, of at least one of the constitutional symptoms described above. E indicates the extension or contiguous involvement of extranodal sites not considered metastatic or stage IV.

Pediatric Patient Considerations
HD in children affects mostly males (85%).
 
Immunocompromised Patient Considerations
HIV is associated with a higher incidence of HD, and 100% of these affected individuals are EBV positive.

For more information, see OMIM.

Codes

ICD10CM:
C81.90 – Hodgkin lymphoma, unspecified, unspecified site

SNOMEDCT:
118599009 – Hodgkin's disease

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Neoplastic:
Infectious:
Reed-Sternberg cells in other scenarios:

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 Updated: 03/29/2017
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Hodgkin lymphoma : Chest pain, Night sweats, Lymphadenopathy, Weight loss, Low grade fever
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