Harvey's Garden

Hodgkin lymphoma

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tags—
aliasesHL

4 min read

Epidemiology

Etiology

  • Strong association with Epstein-Barr virus (EBV) c
  • Immunodeficiency: e.g., organ or cell transplantation, immunosuppressants, HIV infection , chemotherapy
  • Autoimmune diseases (e.g., rheumatoid arthritis, sarcoidosis)

Hodgkin lymphoma vs non-Hodgkin lymphoma

FeatureHodgkin Lymphoma (HL)Non-Hodgkin Lymphoma (NHL)
Key CellReed-Sternberg (“owl-eye”)Malignant lymphocytes (B or T cell)
SpreadContiguous (orderly, predictable)Non-contiguous (disseminated)
PresentationLocalized, single node group (e.g., cervical, mediastinal)Multiple peripheral nodes, extranodal common
”B” SymptomsCommon (fever, night sweats, wt loss)Less common
AgeBimodal (20s, >50s)Typically >50 years old
PrognosisHigh cure rateVariable; depends on subtype
Key AssociationsEBVEBV, HTLV-1, HHV-8; translocations (e.g., t(14;18), t(8;14))
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Pathophysiology

Clinical features

  • Painless Lymphadenopathy (LAD):
    • Firm, rubbery, mobile or matted, non-tender nodes.
    • Anatomic distribution: Cervical (60–70%) > Supraclavicular > Axillary > Mediastinal.
    • Contiguous spread via lymphatic channels.
  • B Symptoms (correlate with advanced disease and poorer prognosis):
    • Unexplained fever (>38°C / 100.4°F).
    • Drenching night sweats.
    • Unintentional weight loss (>10% of body weight over 6 mo).
  • Pathognomonic / High-Yield Signs:
    • Alcohol-induced pain: Immediate severe nodal pain within minutes of alcohol consumption.
    • Pel-Ebstein fevers: Alternating cycles of high fever for 1–2 weeks followed by afebrile periods (rare but classic).
    • Generalized pruritus: Unexplained, severe itching without a primary rash.
  • Mass Effect (Mediastinal Mass):
    • Non-productive cough, dyspnea, retrosternal chest fullness.
    • Superior Vena Cava (SVC) Syndrome: Facial plethora, JVD, upper extremity edema.

Diagnostics

  • Initial / Definitive Diagnostic Step: Excisional lymph node biopsy.
    • Note: FNA is contraindicated/inadequate (preservation of node architecture required).
  • Histopathology / Confirmatory:
    • Reed-Sternberg (RS) cells: Giant, binucleated or multinucleated B-cells with prominent nucleoli (“Owl’s eye” appearance).
    • Immunohistochemistry (Classic HL): CD15(+), CD30(+), CD20(-), CD45(-).
  • Staging Workup (Ann Arbor Staging):
    • Whole-body FDG-PET/CT (skull base to mid-thigh) is the gold standard for staging and response assessment. c
    • Staging Classification:
      • Stage I: Single lymph node region.
      • Stage II: ≥2 lymph node regions on the same side of the diaphragm.
      • Stage III: Lymph node regions on both sides of the diaphragm.
      • Stage IV: Extranodal involvement (e.g., bone marrow, liver, lung).
      • Modifier: A = No systemic symptoms; B = B symptoms present.
  • Key Labs & Baseline Pre-treatment Tests:
    • CBC: Normocytic normochromic anemia, leukocytosis, eosinophilia.
    • ↑ ESR / CRP: Correlates with disease activity and prognosis.
    • Serum LDH: Non-specific marker of tumor turnover.
    • LFTs, BUN/Cr, viral serologies (HIV, Hep B, Hep C).
    • Bone marrow biopsy: Only indicated if cytopenias or high-risk Stage III/IV disease with equivocal PET.
    • Pre-chemo Organ Function:
      • Echocardiogram / MUGA scan: Baseline EF prior to anthracyclines (Doxorubicin).
      • PFTs with DLCO: Baseline pulmonary function prior to Bleomycin.

Treatment

  • Prognosis c
    • HL is highly curable; the majority of patients achieve complete remission with current multi-agent chemotherapy (ABVD) and/or radiation.
    • HL relapse (or recurrence): The leading cause of death for the first 8-10 years after treatment is HL relapse.  Relapses primarily occur in the first 2 years, but risk remains mildly elevated for life.
    • Second malignancy:  The leading cause of death >8-10 years after treatment is second malignancy, and this risk increases with time.
      • Caused by radiation and chemotherapy. Transformation of healthy somatic cells into clinical solid tumors typically takes ≥8–10 years.

Complications

  • Late Treatment-Related Toxicities (High-Yield for Step 2 CK):
    • Secondary Malignancies (leading cause of long-term mortality in cured HL survivors):
      • Breast Cancer: Extremely high risk in young females receiving chest/mediastinal ISRT.
        • Screening guideline: Annual mammogram + breast MRI starting at age 25 or 8–10 years post-radiation (whichever comes later).
      • Lung Cancer: Synergistic risk with cigarette smoking and chest radiation.
      • Therapy-Related Acute Myeloid Leukemia (t-AML) / MDS: Occurs 2–5 yrs post-treatment (alkylating agents, etoposide).
      • Thyroid Cancer: Secondary to neck radiation.
    • Cardiovascular Disease:
      • Accelerated Coronary Artery Disease (CAD), premature MI (radiation-induced endothelial injury).
      • Valvular heart disease (aortic/mitral stenosis or regurgitation).
      • Constrictive pericarditis and pericardial effusion.
      • Dilated cardiomyopathy (Doxorubicin toxicity; cumulative dose-dependent, permanent).
    • Pulmonary Toxicity:
      • Bleomycin-induced pulmonary fibrosis / pneumonitis: Presents with non-productive cough, dyspnea, bilateral infiltrates, decreased DLCO.
      • Radiation pneumonitis / apical fibrosis.
    • Endocrine & Reproductive:
      • Hypothyroidism (up to 50% post-neck/mediastinal radiation; screen with annual TSH).
      • Premature ovarian failure / Azoospermia: Infertility from alkylating agents (offer sperm/oocyte banking prior to initiation).
  • Disease-Related Complications:
    • SVC syndrome.
    • Tracheal compression / airway compromise.
    • Spinal cord compression (epidural tumor extension).
    • Opportunistic infections secondary to cellular immune defects (e.g., Herpes zoster, Pneumocystis jirovecii).

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