Lymphoma, Hodgkin

Key Features

Essentials of Diagnosis

• Fever > 38.0°C

• Weight loss of 10% in the previous 6 months

• Drenching night sweats

• Painless cervical adenopathy

General Considerations

• Represents 50% of the lymphomas of childhood

• Only 15% of all cases occur in children aged 16 years or younger

• Children younger than 5 years account for 3% of childhood cases

• There is a 4:1 male predominance in the first decade

• Hodgkin disease is subdivided into four histologic groups, and the distribution in children parallels that of adults

  • Lymphocyte-predominant (10–20%)

  • Nodular sclerosing (40–60%) (increases with age)

  • Mixed cellularity (20–40%)

  • Lymphocyte-depleted (5–10%)

Clinical Findings

• Painless cervical adenopathy

• Lymph nodes are often firmer than inflammatory nodes and have a rubbery texture

  • May be discrete or matted together and are not fixed to surrounding tissue

  • Involved nodes may wax and wane in size over weeks to months

• Half of patients have asymptomatic mediastinal disease (adenopathy or anterior mediastinal mass), although symptoms due to compression of vital structures in the thorax may occur

• Splenomegaly or hepatomegaly is generally associated with advanced disease

Diagnosis

Laboratory Findings

• CBC is usually normal, although anemia, neutrophilia, eosinophilia, and thrombocytosis may be present

• Erythrocyte sedimentation rate and other acute-phase reactants are often elevated and can serve as markers of disease activity

• Immunologic abnormalities occur, particularly in cell-mediated immunity, and anergy is common in patients with advanced-stage disease

Imaging

• Chest radiograph should be obtained

• CT scan of the chest, abdomen, and pelvis should be considered

• Technetium bone scanning is usually reserved for patients with bone pain because bony involvement is rare

• Positron emission tomography is increasingly used for staging and follow-up

Diagnostic Procedures

• Indications for early lymph node biopsy include

  • Lack of identifiable infection in the region drained by the enlarged node

  • Node > 2 cm in size

  • Supraclavicular adenopathy

  • Abnormal chest radiograph

  • Lymphadenopathy increasing in size after 2 weeks or failing to resolve within 4–8 weeks

• Diagnosis requires histologic presence of the Reed-Sternberg cell or its variants in tissue

Treatment

• To achieve long-term disease-free survival while minimizing treatment toxicity, Hodgkin disease is increasingly treated by chemotherapy alone—and less often by radiation therapy

• Several combinations of chemotherapeutic agents are effective, and treatment times are relatively short compared with pediatric oncology protocols for leukemia

• AV-PC (Adriamycin [doxorubicin], vincristine, prednisone, and cyclophosphamide) used in patients with low-risk Hodgkin lymphoma

• Bleomycin and etoposide are added in the treatment of intermediate-risk patients

• Combined-modality therapy with chemotherapy and irradiation is used in advanced disease

Outcome

Prognosis

• When all stages are evaluated, 5- to 10-year overall survival rate is > 90%

• Overall 5-year survival in ...