Description

• Historical background:
  • Described in 1832 by Thomas Hodgkin in On Some Morbid Appearance of the Absorbent Glands and Spleen
  • First neoplasm to be: (i) Defined by cytologic grounds based on presence of Reed-Sternberg cells, (ii) clinically staged neoplastic disease, and (iii) treated with chemotherapy and/or radiotherapy
• Neoplastic Reed-Sternberg (RS) cells of monoclonal lymphoid B-cell origin within inflammatory background of lymphocytes (T-helper type 2 and regulatory T-cells), eosinophils, histiocytes, and plasma cells

• 2 subtypes: Classic Hodgkin lymphoma (CHL, 95% of cases) and nodular lymphocyte predominant Hodgkin lymphoma (NLPHL, 5% of cases) (1):
  • NLPHL: B-cells, neoplastic luteinizing hormone (LH) cells with multilobulated nuclei, small nucleoli, and popcornlike appearance
  • CHL histologic subdivisions: Nodular sclerosing (60%), mixed cellularity (30%), lymphocyte depleted (<10%), lymphocyte rich (<10%)
  • Frequency of lymph node involvement: Cervical > Mediastinal > Axillary > Para-aortic

Epidemiology

Incidence: 2.8/100,000/year (2)

• Less likely to tolerate intensive chemotherapy
• Less likely to be included in clinical trial

• Increased risk for males (3)
• Young females (<30 years of age) treated with thoracic radiation are at high risk for breast cancer and early breast cancer screening is recommended (4).

• Abdominal ultrasonography to detect subdiaphragmatic disease
• Treatment (4):
  • Delay until after delivery if asymptomatic and early-stage
  • ABVD safely used in 2nd and 3rd trimesters
  • Vinblastine monotherapy to control symptoms
  • 1st trimester: ABVD may or may not cause fetal malformations

• ~9,000 new cases in the US annually (2)
• Mean age at diagnosis: 38 years (2)

Prevalence
~175,000 living with Hodgkin lymphoma in the US in 2009

Risk Factors

• Immunodeficiency (inherited or acquired)
• Autoimmune disorders
• Epstein-Barr virus (EBV)
• Seasonal factors

• 1st-degree relative: 3–9× risk
• Siblings of younger patients: 7× risk
• Weak correlation between familial HL and HLA class I regions containing HLA A1, B5, B8, B18 alleles

Etiology

• RS cells likely derived from germinal center B cells with mutations in immunoglobulin variable chain
• Seasonal features and higher frequencies with EBV suggest environmental factors.
• T-lymphocyte defects persist even after successful treatment.

Commonly Associated Conditions

In HIV (4):

• AIDS-defining illness
• Predominantly mixed-cellularity or lymphocyte-depleted histologic subtypes
• At diagnosis: Widespread disease, extranodal involvement, systemic symptoms