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Referral and warning signs
When to refer
Referral to specialized cancer centre for ALL suspected cases for biopsy, staging, and treatment.
Red flags
- Persistent, painless lymphadenopathy (especially supraclavicular)
- Presence of 'B' symptoms (unexplained fever, night sweats, weight loss)
- Mediastinal mass on Chest X-ray
- Generalized pruritus
Signs and symptoms
- Painless enlargement of lymph nodes (lymphadenopathy), often cervical, supraclavicular, or axillary. Nodes typically firm, rubbery, non-tender.
- Mediastinal mass (may cause cough, dyspnoea, SVC obstruction).
- Splenomegaly.
- Hepatomegaly (less common).
- 'B' Symptoms (occur in ~30-40%): Unexplained fever (>38°C), Drenching night sweats, Unexplained weight loss (>10% body weight in 6 months).
- Pruritus (generalized itching).
- Pain in involved lymph nodes after alcohol consumption (rare but specific).
Risk factors
- Epstein-Barr Virus (EBV) infection (associated with some subtypes).
- Immunodeficiency (e.g., HIV).
- Family history (rare).
- Bimodal age distribution (peaks in young adulthood 15-35 yrs, and older age >55 yrs).
Diagnosis
Clinical assessment
Suspect in patient (often adolescent/young adult) with persistent, painless lymphadenopathy, +/- B symptoms. Differentiate from reactive lymphadenopathy (infection), other lymphomas (NHL), TB lymphadenitis, metastatic cancer.
Investigations
- Excisional Lymph Node Biopsy: Essential for diagnosis. Histopathology identifies characteristic Reed-Sternberg cells within an inflammatory background. Allows subtyping (e.g., Nodular Sclerosis, Mixed Cellularity). FNA is inadequate.
- Blood tests: CBC (may show anaemia, eosinophilia, lymphopenia), ESR/CRP (often elevated, used for staging/prognosis), LDH, LFTs, RFTs.
- Imaging for Staging: CT scan (Neck, Chest, Abdomen, Pelvis) +/- PET-CT scan (more sensitive for determining extent of disease).
- Bone Marrow Aspiration and Biopsy: Assess for marrow involvement (part of staging).
- HIV testing.
Diagnostic criteria
Histopathological confirmation of Hodgkin lymphoma (presence of Reed-Sternberg cells) on lymph node biopsy.
Management
Non-pharmacological management
- Referral to specialized cancer centre (UCI/RRH) for staging and treatment.
- Supportive care.
Pharmacological treatment
- Combination Chemotherapy: Mainstay of treatment. Regimens vary based on stage and risk factors (e.g., ABVD, BEACOPP).
- Radiotherapy: Often used consolidation therapy to involved sites, especially in early-stage disease or bulky disease.
- Highly curable, especially in early stages.
Monitoring
Monitor for treatment response (imaging - CT/PET-CT). Monitor for treatment toxicity (chemo side effects, radiation side effects). Long-term follow-up for relapse and late complications (secondary malignancies, cardiovascular disease, infertility, hypothyroidism).
Complications
- Complications related to bulky disease (e.g., SVC obstruction).
- Treatment-related toxicity (acute and long-term).
- Infertility.
- Secondary malignancies (leukaemia, solid tumours - related to chemo/radiation).
- Cardiovascular disease (radiation-related).
- Hypothyroidism (neck radiation).
- Relapse.
Prevention
- No specific prevention.
Patient education
- Explain Hodgkin Lymphoma and high cure rates.
- Need for biopsy, staging investigations.
- Treatment plan (chemotherapy +/- radiotherapy).
- Potential side effects (acute and long-term).
- Importance of adherence and follow-up.
- Fertility preservation discussion before treatment if applicable.
Uganda-specific notes
Occurs in Uganda, may be associated with EBV/HIV. Diagnosis requires adequate lymph node biopsy and pathology services. Treatment (chemo/radio) available at specialized centres.
References
- Uganda Clinical Guidelines (UCG) 2023
Chapter 12, Section 12.3.1 (Listed under Paediatric Cancers but occurs in adults too)
- Uganda Cancer Institute Treatment Guidelines
(Specific protocols)