• In Hodgkin's disease tissues: most cells -- small lymphocytes with a mature T cell phenotype (CD2, CD3, CD4 > CD5, CD8)

• Disease process::
  • Initially -- localized disease
  • Next -- spreads to nearby lymphoid tissue
  • Disseminates -- to non-lymphoid tissue

• Clinical Presentation:
  • Common presentation: newly observed mass or lymph node group (firm, typically not tender,freely movable )
  • About 50% of patients: head or supraclavicular area adenopathy
  • About 70% of patients: superficial lymph node enlargement
  • About 60% of patients: mediastinal adenopathy
  • Most patients upon presentation: fewer or no disease-related symptoms.
    • About 20% to 30% of patients, symptomatic:
      • low-grade fever
      • night sweats (may be only complaint)
  • Patients with mediastinal involvement or involvement of pleura or pericardium:
    • cough
    • chest pain
    • shortness of breath
    • hypertrophic osteoarthropathy
  • Bone involvement: bone pain

•  Staging (Ann Arbor staging system)
  • Stage I: Involvement in single lymph node region or single extralymphatic site.
  • Stage II:
    • Involvement of two or more lymph node regions on the same side of diaphragm.
    • Localized contiguous involvement of only one extralymphatic sites and lymph node region (stage IIE)
  • Stage III: Involvement of lymph node regions on both sides of diaphragm; may involve spleen.
  • Stage IV: Disseminated involvement of one or more extralymphatic organs with or without lymph node involvement. (p.701, ref. below)

Treatment

• Cure rate: about 85% of patients
  • Localized disease: radiotherapy -- cure rate: about 80%
  • Disseminated disease: chemotherapy -- curator: about 70%

• Treatment approach depends on disease staging

• Chemotherapeutic approach may involve initial treatment and subsequent salvage therapy.

• Early Stage Disease: Stage I and stage II disease
  • Mainstay: radiation
    • Localized radiation -- 80% in long-term disease-freeing survival beyond ten years.
    • Consideration is being given to using combination chemotherapy for early stage Hodgkin's disease
      • avoidance of complications of radiation treatment
      • success of combination chemotherapy is at least equal to radiation treatment
• Advanced disease
  • Combination chemotherapy (MOPP)
    •  mechlorethamine (Mustargen)
    •  vincristine (Oncovin)
    •  procarbazine (Matulane)
    •  prednisone  (Deltasone)
  • Complete Remission (CR): 85% to 90% of patients
    • 2/3 of clinical remission cases are durable
  •  MOPP therapy:
    • Significant toxicity
    • nausea and vomiting
    • bone marrow suppression
    • About 2% of patients on this protocol develope myelodysplasia and/or leukemia 4--6 years after treatment
    • Leukemia is unlikely to occur in patients whose survival is 10 years or longer
    • infertility
  • Protocols for MOPP-resistant disease
    •  Combination chemotherapy (ABVD)
      •  Adriamycin (doxorubicin)
      •  bleomycin  Blenoxane)
      •  vinblastine  (Velban)
      •  dacarbazine dacarbazine (DTIC)
    • ABVD is comparable to MOPP in efficacy in previously untreated patients.
      • comparisons of MOPP and ABVD and MOPP/ABVD combinations suggest some differences in various risks. For example:
        • ABVD: decreased risk of infertility;increased risk of pulmonary toxicity.
• Salvage Therapy
  • Following relapse and re-staging:
  • Further radiotherapy
  • Alternative chemotherapy protocol
    •  if MOPP initially, then ABVD following relapse.
    •  if ABVD initially, then MOPP following relapse.
    •  if ABVD/MOPP combination initially, then:
      • carmustine (BNCU,BiCNU), etoposide (VP-16,VePe-sid), cytarabine (ARA-C), and melphalan (Alkeran) 
  • Conventional-dose salvage combination chemotherapy:
    • 30% to 40% -- complete remission, long-term survival: 10% to 25%