Hodgkin Lymphoma

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

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

• Even though others such as escalated BEACOPP may improve response rates, it is usually associated with increased toxicity and lacks a definitive overall survival advantage

• Stage I and II disease: combination of short-course chemotherapy with involved-node radiotherapy (INRT), but INRT can be eliminated for those with an early negative PET/CT scan without a significant change in outcomes (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 disease relapses,

  • High-dose chemotherapy with autologous hematopoietic stem cell transplantation

  • The antibody-drug conjugate brentuximab vedotin has shown impressive activity ...