Hodgkin Lymphoma
Basics
Basics
Basics
Description
Description
Description
B lineage malignant lymphoid neoplasm. Typically presents with painless lymphadenopathy, often cervical (70–80%) or supraclavicular (25%). Mediastinal mass present in 50%. Constitutional symptoms (e.g., fatigue, fever, night sweats, weight loss, cough, pruritus) may or may not be present. Constitutional symptoms sometimes precede development of lymphadenopathy.
Epidemiology
Epidemiology
Epidemiology
- Represents 7% of childhood cancer
- 11.7 cases/million/y age <20 years
- Most common cancer for ages 15 to 19 years
- Rarely seen in children age <5 years
- M > F age <15 years
- F > M age 15 to 19 years
- Bimodal age distribution in adults: early peak mid/late 20s; late peak > age >50 years
Risk Factors
Risk Factors
Risk Factors
- Few known risk factors include the following:
- Immune deficiency (e.g., HIV infection)
- Autoimmune disorders
- Lower socioeconomic status for childhood form (age 14 years or younger)
- Higher socioeconomic status for young adult form
- Decreased risk if
- Multiple older siblings
- Exposure to common infections in preschool
Genetics
Genetics
Genetics
- Familial Hodgkin lymphoma (HL) rare, accounting for 4.5% of cases
- Familial cases may reflect
- Genetic influences, including inherited immunodeficiency states
- Environmental factors
- Exposure to viruses
Pathophysiology
Pathophysiology
Pathophysiology
- Reed-Sternberg cells, a clonal population of large binucleate cells arising from B cells, are the malignant cells in HL. Surface antigen expression includes CD30 but not CD20.
- Only 1% of cells in involved nodes are Reed-Sternberg cells and morphologic variants; the rest are inflammatory cells: lymphocytes, macrophages, fibroblasts, plasma cells, eosinophils.
- Two clinically distinct subtypes of HL recognized:
- Classical HL (90–95% of cases)
- Nodular sclerosing
- Mixed cellularity
- Lymphocyte depleted
- Lymphocyte rich
- Nodular lymphocyte predominant (5–10% of cases; treated differently from classical HL. See National Comprehensive Cancer Network (NCCN) guidelines for treatment recommendations.)
Etiology
Etiology
Etiology
- Cause unknown
- Association between Epstein-Barr virus (EBV) infection and HL: 20–50% of patients with classical HL have monoclonal or oligoclonal proliferation of EBV-infected cells.
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