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Key Features

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Essentials of Diagnosis
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  • Fever > 38.0°C

  • Weight loss of 10% in the previous 6 months

  • Drenching night sweats

  • Painless cervical adenopathy

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General Considerations
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  • 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%)

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Clinical Findings

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  • 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

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Diagnosis

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Laboratory Findings
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  • 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

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Imaging
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  • 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

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Diagnostic Procedures
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  • 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

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Treatment

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  • 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

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Outcome

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

  • Overall 5-year survival in children with stages I and II Hodgkin lymphoma is 90–95%

  • Two-thirds ...

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