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Key Features

Essentials of Diagnosis

  • Lymphadenopathy, often painless

  • Constitutional symptoms may or may not be present

  • Pathologic diagnosis by lymph node biopsy

General Considerations

  • Group of cancers characterized by lymph node biopsy showing Reed-Sternberg cells in an appropriately reactive cellular background

  • Malignant cell is a B lymphocyte

  • Divided into pathologic subtypes

    • Classic Hodgkin lymphoma (nodular sclerosis, mixed cellularity, lymphocyte rich, and lymphocyte depleted)

    • Non-classic Hodgkin lymphoma (nodular lymphocyte predominant)

  • Tendency to arise within single lymph node areas and spread in orderly fashion to contiguous lymph nodes

  • Widespread hematogenous dissemination occurs only late in course

Demographics

  • Bimodal age distribution

    • One peak occurs in persons age 20–30

    • Second peak occurs in persons over age 50

Clinical Findings

Symptoms and Signs

  • Painless lymphadenopathy (mass), commonly in neck

  • Constitutional symptoms, eg, fever, weight loss, or night sweats, or generalized pruritus

  • Pain in involved lymph node after alcohol ingestion is an unusual symptom

Differential Diagnosis

  • Non-Hodgkin lymphoma

  • Tuberculous lymphadenitis (scrofula)

  • Cat-scratch disease

  • Sarcoidosis

  • Infectious mononucleosis

  • Metastatic cancer

  • Drug-induced pseudolymphoma (eg, phenytoin)

Diagnosis

Diagnostic Procedures

  • Serum chemistries, whole body PET/CT scan, and bone marrow biopsy

  • Staging nomenclature (Ann Arbor)

    • Stage I, one lymph node region involved

    • Stage II, involvement of two or more lymph node regions on one side of diaphragm

    • Stage III, lymph node regions involved on both sides of diaphragm

    • Stage IV, disseminated disease with extranodal involvement

  • In addition, designated as stage A if there is a lack of constitutional symptoms and stage B if there is a 10% weight loss over 6 months, fever, or night sweats ("B symptoms")

Treatment

Medications

  • For stage I and II disease: combination of short course chemotherapy with involved-node radiotherapy

    • ABVD: doxorubicin, bleomycin, vinblastine, dacarbazine

    • Stanford V: doxorubicin, vinblastine, bleomycin, vincristine, nitrogen mustard, prednisone, etoposide

  • For stage III or IV disease: full course of ABVD (no radiotherapy) or Stanford V chemotherapy (with radiotherapy to masses > 5 cm)

  • Chemotherapy is the mainstay of treatment and ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine) remains the standard first-line regimen

  • Even though others such as Stanford V or BEACOPP (baseline or escalated) may improve response rates, they are usually associated with increased toxicity and lack a definitive overall survival advantage

  • Stage I and II disease: combination of short-course chemotherapy with involved-node radiotherapy or full course of chemotherapy alone (Table 39–3)

  • Stage II disease and a large mediastinal or other bulky mass: full course of ABVD for six cycles with involved-node radiotherapy

  • Stage III or IV disease: full course of ABVD (no radiotherapy)

  • Pulmonary toxicity can unfortunately occur following either chemotherapy or radiation and should be treated aggressively, since it can lead to permanent fibrosis and death

  • When ...

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