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SN7 SHORT NOTE 5M

Define Hodgkin Lymphoma. Describe its classification, aetiology, clinical features and investigations.

Pediatrics · Final Professional

Definition

  • Hodgkin lymphoma (HL) is a malignant lymphoma arising in lymph nodes, characterised by the presence of the diagnostic Reed-Sternberg (RS) cell — a large binucleate cell with prominent eosinophilic nucleoli giving an 'owl-eye' appearance — surrounded by a reactive inflammatory background.
  • It shows a bimodal age distribution (adolescents/young adults and older adults).

Histological Classification (WHO)

Two broad groups — Classical HL (~95%) (RS cells are CD15+ and CD30+) and Nodular lymphocyte-predominant HL:

SubtypeKey features
Nodular sclerosisCommonest (~70%); adolescents; mediastinal mass; lacunar cells
Mixed cellularity2nd commonest; EBV-associated; older/immunocompromised
Lymphocyte-richFew RS cells; good prognosis
Lymphocyte-depletedMany RS cells; worst prognosis; HIV-associated
Nodular lymphocyte-predominant'Popcorn' (L&H) cells, CD20+; indolent, late relapses

Etiology & Risk Factors

  • Epstein-Barr virus (EBV) — strongly linked to mixed-cellularity HL.
  • Immunodeficiency (HIV), immunosuppression; family history.

Clinical Features

  • Painless, firm, rubbery lymphadenopathy — most often cervical/supraclavicular; spreads in a contiguous (predictable, node-to-adjacent-node) fashion.
  • Mediastinal mass — cough, dyspnoea, or superior vena cava obstruction.
  • 'B symptoms' — fever > 38 °C, drenching night sweats, and weight loss > 10% over 6 months (indicate a worse prognosis).
  • Classical clues — Pel-Ebstein (cyclical) fever, alcohol-induced nodal pain, and generalised pruritus.
  • Hepatosplenomegaly and, in advanced disease, marrow involvement.

Investigations

  • Excisional lymph-node biopsy — the diagnostic test; demonstrates Reed-Sternberg cells with immunophenotyping (CD15/CD30).
  • CBC (anaemia), ESR and LDH (raised; prognostic), renal/liver function.
  • Contrast CT (neck, chest, abdomen, pelvis) and pet-CT for staging; chest X-ray for a mediastinal mass.
  • Bone-marrow biopsy for advanced-stage/B-symptom disease.

Ann Arbor Staging (with Cotswold Modification)

StageExtent
IA single lymph-node region
II≥ 2 regions on the same side of the diaphragm
IIIRegions on both sides of the diaphragm (± spleen)
IVDiffuse extranodal spread (liver, marrow, lung)

Suffixes: A (no B symptoms), B (B symptoms present), E (extranodal extension), S (splenic), X (bulky disease).

Hodgkin VS Non-hodgkin Lymphoma (high-yield Comparison)

FeatureHodgkin lymphomaNon-Hodgkin lymphoma
CellReed-Sternberg cellNo RS cell
SpreadContiguous (orderly)Non-contiguous ('skip' lesions)
Extranodal diseaseUncommonCommon
SiteUsually cervical/mediastinalOften abdominal / widespread
PresentationNodal, B symptomsMay be an emergency (mass, tumour lysis)
PrognosisExcellentVariable

Management

  • Risk-adapted chemotherapy — the standard regimen is ABVD (Adriamycin/doxorubicin, Bleomycin, Vinblastine, Dacarbazine), with the number of cycles guided by stage and pet response.
  • Involved-field/site radiotherapy is added for bulky or residual disease (used cautiously in children to limit late effects).
  • Treatment intensity is tailored (de-escalated for pet-negative early disease).

Prognosis & Late Effects

  • Excellent prognosis — cure rates > 90% in early-stage childhood HL.
  • Late effects of therapy — second malignancies, anthracycline cardiotoxicity, bleomycin lung toxicity, hypothyroidism (neck radiation) and infertility — hence long-term survivorship follow-up.

Painless rubbery cervical nodes with B symptoms.

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