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Source: Chan A, Yee GC. Lymphomas. In: DiPiro, JT, Talbert RL, Yee GC, Matzke GR, Wells BG, Posey LM. Pharmacotherapy: A Pathophysiologic Approach. 8th ed. http://accesspharmacy.com/content.aspx?aid=8009338. Accessed August 4, 2012.

  • Clonal malignant lymphoid disease of transformed lymphocytes
    • Malignant cell known as Reed-Steinberg cell

  • Unknown
    • Laboratory evidence supports infectious exposure as potential cause
      • Increased risk with Epstein-Barr virus (EBV)
    • Genetic factors

  • B-cell transcriptional processes disrupted
    • Prevents expression of B-cell surface markers and production of immunoglobulin messenger RNA (mRNA).
    • Alterations in normal apoptotic pathways favor cell survival and proliferation.
  • Malignant Reed–Sternberg cells overexpress nuclear factor-κ B.
    • Associated with cell proliferation and anti-apoptotic signals.
    • Up-regulated by infections with viral and bacterial pathogens.
      • Epstein–Barr virus found in many, but not all, Hodgkin lymphoma (HL) tumors.

  • HL represents <1% of all known cancers in United States.
  • Bimodal distribution with first peak in third decade and small peak occurring after age 50

  • EBV
  • HIV

  • Most patients with HL present with painless, rubbery, enlarged lymph node in supradiaphragmatic area and commonly have mediastinal nodal involvement.

Signs and Symptoms

  • Constitutional (B symptoms) in ~25% of all patients
    • Fever
    • Drenching night sweats
    • Weight loss

Means of Confirmation and Diagnosis

  • Requires presence of Reed–Sternberg cells in lymph node biopsy.

Laboratory Tests

  • Metabolic panel
  • Complete blood count (CBC)
  • Erythrocyte sedimentation rate (ESR)

Imaging

  • Chest radiography
  • Computed tomography (CT) of chest, abdomen, and pelvis
  • Positive emission tomography (PET)
  • Integrated PET-CT

Diagnostic Procedures

  • Excisional lymph node biopsy
  • Laparoscopy or laparotomy of strategic sites
  • Bone marrow aspiration and biopsy with advanced-stage disease

Staging

  • Based on clinical or pathologic findings
    • Localized disease (stages, I, II, and IIE) in ~50% of patients
    • Advanced disease (stage III or IV) in other half
      • 10–15% have metastatic disease (stage IV)

Differential Diagnosis

  • Maximize curability while minimizing short- and long-term treatment-related complications.

  • Combination chemotherapy is primary treatment modality.

  • Therapeutic role of surgery limited, regardless of stage.
  • Radiation therapy (RT)
    • Early-stage HL treated with combination chemotherapy and RT.
    • Advanced-stage HL treated with combination chemotherapy with or without RT.

  • Initial chemotherapy
    • Two to eight cycles of chemotherapy given depending on stage of disease and presence of risk factors (Tables 1 and 2).
    • Salvage chemotherapy
    • Treatment options for relapse after initial complete response
      • Repeat same regimen
      • Non–cross-resistant regimen
      • RT
      • High-dose chemotherapy and autologous hematopoietic stem cell transplantation (HSCT)
    • Response to salvage therapy depends on:
      • Extent and site of recurrence
      • Previous therapy
      • Duration of first remission
    • Choice of salvage therapy should be guided by response to initial therapy and patient’s ability to tolerate therapy.

Table 1. General Treatment Recommendations for Hodgkin Lymphoma

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