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SOURCE

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Source: Chan A, Sessions J. Lymphomas. In: DiPiro JT, Talbert RL, Yee GC, Matzke GR, Wells BG, Posey LM, eds. Pharmacotherapy: A Pathophysiologic Approach. 10th ed. New York, NY: McGraw-Hill; 2017. http://accesspharmacy.mhmedical.com/content.aspx?bookid=1861&sectionid=133894009. Accessed April 13, 2017.

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DEFINITION

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  • Clonal malignant lymphoid disease of transformed lymphocytes.

    • Malignant cell known as Reed–Steinberg cell.

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ETIOLOGY

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  • Unknown.

    • Laboratory evidence supports infectious exposure as potential cause.

      • Increased risk with Epstein–Barr virus (EBV)

    • Genetic factors.

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PATHOPHYSIOLOGY

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  • 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.

    • Upregulated by infections with viral and bacterial pathogens.

      • Epstein–Barr virus found in many, but not all, Hodgkin lymphoma (HL) tumors.

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EPIDEMIOLOGY

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  • 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

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RISK FACTORS

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  • EBV

  • HIV

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CLINICAL PRESENTATION

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

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SIGNS AND SYMPTOMS
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  • Constitutional (B symptoms) in ~25% of all patients.

    • Fever.

    • Drenching night sweats.

    • Weight loss.

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DIAGNOSIS

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MEANS OF CONFIRMATION AND DIAGNOSIS
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  • Requires presence of Reed–Sternberg cells in lymph node biopsy.

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LABORATORY TESTS
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  • Metabolic panel.

  • Complete blood count (CBC)

  • Erythrocyte sedimentation rate (ESR)

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IMAGING
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  • Chest radiography.

  • Computed tomography (CT) of chest, abdomen, and pelvis.

  • Positive emission tomography (PET)

  • Integrated PET-CT

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DIAGNOSTIC PROCEDURES
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  • Excisional lymph node biopsy.

  • Laparoscopy or laparotomy of strategic sites.

  • Bone marrow aspiration and biopsy with advanced-stage disease.

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STAGING
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  • 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)

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DIFFERENTIAL DIAGNOSIS
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DESIRED OUTCOMES

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

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TREATMENT: GENERAL APPROACH

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  • Combination chemotherapy is primary treatment modality.

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TREATMENT: NONPHARMACOLOGIC THERAPY

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  • 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

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TREATMENT: PHARMACOLOGIC THERAPY

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  • Initial chemotherapy.

    • Two to eight cycles of chemotherapy given, depending on stage of disease and presence ...

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