Hodgkin Lymphoma

Descriptive text is not available for this image BASICS

Hodgkin Lymphoma (HL) is a neoplasm of the lymphatic system representing one of the common cancers in young adults; characterized by a low number of malignant cells deriving from B lymphocytes and an extensive inflammatory microenvironment

DESCRIPTION

  • Historical background:
    • Described first in 1832 by Thomas Hodgkin who noted differences in patients from tuberculosis and syphilis
    • Carl Sternberg (1898) and Dorothy Reed (1902) independently identified the hallmark Reed-Sternberg (RS) cells—the microscopic hallmarks of Hodgkin disease.
    • Initially treated with herbs, surgery, and arsenic in the 19th century; HL showed sensitivity to X-rays in the beginning of the 20th century. Chemotherapy is instituted as first-line treatment in the early 1970s.
  • Subtypes:
    • Classical HL (cHL)—95% of cases; includes nodular sclerosing 70%, mixed cellularity 20–25%, lymphocyte rich 5% and lymphocyte depleted <5%
    • Nodular lymphocyte predominant HL (NLPHL)—5% of cases

EPIDEMIOLOGY

  • Incidence: 2 to 3 per 100,000 per year
  • 11% of all lymphoid malignancies
  • Bimodal age distribution peaks at 20 to 40 years and around 55 years, typically, diagnosed at median age of 39 years
  • 1.3:1 male-to-female ratio

ETIOLOGY AND PATHOPHYSIOLOGY

  • cHL is a B-cell lymphoma of germinal center origin that has lost its B-cell phenotype.
  • RS cells have clonal rearrangements of immunoglobulin genes resulting in nonfunctional immunoglobulins and expression of apoptosis through oncogenic events.
  • NLPHL lacks typical RS cells but has lymphocytic and histiocytic cells, characterized by larger cells with folded multilobulated nuclei (“popcorn cells” or LP cells)
  • Genome-wide association studies identified 19p13.3 at intron 2 of TCF3.

Genetics

  • First-degree relative: 3 to 9 times risk
  • Siblings of younger patients: 7 times risk
  • Weak correlation with certain HLA alleles

RISK FACTORS

  • HIV: increased risk of HL in patients who are HIV positive
  • Epstein-Barr virus (EBV): detected in nearly 45% of HL cases
  • Genetic predisposition: significantly in identical twins

GENERAL PREVENTION

Avoid exposure to chemicals such as pesticides, herbicides, and benzene.

COMMONLY ASSOCIATED CONDITIONS

HIV infection, geography, socioeconomic factors and family history

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