Hodgkin lymphoma - Skin
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.
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.
C81.90 – Hodgkin lymphoma, unspecified, unspecified site
118599009 – Hodgkin's disease
- Non-Hodgkin's lymphoma
- Acute lymphoblastic leukemia – blast cells, chromosome 9 and 22 translocation (Philadelphia chromosome)
- Large cell anaplastic lymphoma – T-cell lymphoma, mimics CD30 positive lymphomas, EBV negative, paranuclear staining pattern
- Lymphomatoid papulosis – no lymph node involvement unless with concomitant lymphoma
- Brucellosis – Gram negative rods, from ingestion of unpasteurized milk or meat of infected animals
- Tuberculosis – aerobic bacillus, hemoptysis, positive tuberculin skin test and chest X-ray
- Toxoplasmosis – protozoan transmitted by infected cats and other animals
- Human immunodeficiency virus (HIV) or acquired immunodeficiency syndrome (AIDS) – may coexist with HD