SN7
SHORT NOTE
5M
Define Hodgkin Lymphoma. Describe its classification, aetiology, clinical features and investigations.
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:
| Subtype | Key features |
|---|---|
| Nodular sclerosis | Commonest (~70%); adolescents; mediastinal mass; lacunar cells |
| Mixed cellularity | 2nd commonest; EBV-associated; older/immunocompromised |
| Lymphocyte-rich | Few RS cells; good prognosis |
| Lymphocyte-depleted | Many 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)
| Stage | Extent |
|---|---|
| I | A single lymph-node region |
| II | ≥ 2 regions on the same side of the diaphragm |
| III | Regions on both sides of the diaphragm (± spleen) |
| IV | Diffuse 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)
| Feature | Hodgkin lymphoma | Non-Hodgkin lymphoma |
|---|---|---|
| Cell | Reed-Sternberg cell | No RS cell |
| Spread | Contiguous (orderly) | Non-contiguous ('skip' lesions) |
| Extranodal disease | Uncommon | Common |
| Site | Usually cervical/mediastinal | Often abdominal / widespread |
| Presentation | Nodal, B symptoms | May be an emergency (mass, tumour lysis) |
| Prognosis | Excellent | Variable |
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.
All questions in Haematology & Oncology
- Q1Define Iron Deficiency Anaemia. Describe its aetiology, pathophysiology, clinical features and investigations. ★
- Q2Define Beta-Thalassaemia Major. Describe its pathophysiology, clinical features, investigations and management. ★
- Q3Define Acute Lymphoblastic Leukaemia. Describe its clinical features, investigations, management and prognosis. ★
- Q4Define Haemophilia. Describe its clinical features, investigations, management and complications. ★
- Q5Define Approach to a Child with Anaemia. Describe its classification, investigations and management. ★
- SN1Define Immune Thrombocytopenic Purpura. Describe its clinical features, investigations, diagnosis and management. ★
- SN2Define Sickle Cell Disease. Describe its clinical features, investigations, management and types. ★
- SN3Define Megaloblastic Anaemia. Describe its causes, clinical features, investigations and diagnosis.
- SN4Define Aplastic Anaemia. Describe its causes, clinical features, investigations and complications.
- SN5Define G6PD Deficiency. Describe its clinical features, investigations, pathophysiology and management.
- SN6Define Neuroblastoma. Describe its clinical features, investigations and prognosis.
- SN7Define Hodgkin Lymphoma. Describe its classification, aetiology, clinical features and investigations.