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Referral and warning signs
When to refer
Immediate urgent referral to specialized cancer centre for ALL suspected cases.
Red flags
- Rapidly growing jaw or abdominal mass in a child
- Signs of bowel obstruction
- Neurological deficits
- Signs of tumour lysis syndrome (renal failure, electrolyte disturbance)
Signs and symptoms
- Rapidly growing tumour/mass, often painless initially.
- Jaw swelling (most common presentation in endemic form).
- Abdominal mass / Abdominal distension / Pain.
- Intestinal obstruction / Intussusception.
- Ascites.
- Bone marrow involvement: Anaemia, thrombocytopenia (bleeding), neutropenia (infections).
- Central Nervous System (CNS) involvement: Headache, cranial nerve palsies, seizures, altered mental status.
- Lymphadenopathy (less common than other lymphomas).
- Constitutional symptoms (fever, night sweats, weight loss) may occur.
Risk factors
- Childhood (peak age 4-7 years for endemic form).
- Geographic location (endemic in malaria holoendemic areas of Africa).
- Epstein-Barr Virus (EBV) infection.
- Chronic malaria infection (immunosuppression/B-cell stimulation).
- HIV infection (associated with sporadic/immunodeficiency-related BL).
Diagnosis
Clinical assessment
Suspect in a child from endemic area with rapidly growing jaw or abdominal mass.
Investigations
- Biopsy of tumour mass (FNA or excisional/incisional): Essential for diagnosis. Histology shows characteristic 'starry sky' appearance. Immunohistochemistry confirms B-cell origin.
- Bone Marrow Aspiration and Biopsy: Assess for marrow involvement.
- Lumbar Puncture with CSF analysis (cytology): Assess for CNS involvement.
- Blood tests: CBC (cytopenias), LDH (Lactate Dehydrogenase - often very high, marker of tumour burden/turnover), Uric Acid (risk of tumour lysis syndrome), Renal/Liver function tests.
- Imaging: Abdominal Ultrasound/CT scan (assess abdominal disease extent), Chest X-ray.
- HIV testing.
Diagnostic criteria
Histopathological confirmation from biopsy.
Management
Non-pharmacological management
- Urgent referral to specialized cancer centre (UCI/RRH).
- Supportive care: Management of tumour lysis syndrome (hydration, allopurinol/rasburicase), infection control, nutritional support.
Pharmacological treatment
- Intensive multi-agent chemotherapy is the mainstay of treatment. Highly sensitive to chemo, cure rates high if treated appropriately.
- Specific protocols depend on stage and risk group (specialist management). Often include drugs like Cyclophosphamide, Vincristine, Methotrexate, Cytarabine, Doxorubicin, Rituximab.
- CNS prophylaxis/treatment is essential.
Monitoring
Close monitoring for treatment response and toxicity (myelosuppression, tumour lysis syndrome, infections). Long-term follow-up for relapse.
Complications
- Tumour Lysis Syndrome (metabolic emergency with chemo initiation)
- Bowel obstruction/perforation
- CNS involvement
- Spinal cord compression
- Treatment-related toxicity (myelosuppression, infertility, secondary malignancies)
Prevention
- Malaria control may play a role in reducing endemic BL incidence.
Patient education
- (To parents/guardians) Explain Burkitt's lymphoma as a fast-growing but treatable cancer.
- Need for urgent referral and intensive chemotherapy.
- Explain treatment plan, potential side effects, need for supportive care.
- Importance of adherence and follow-up.
Uganda-specific notes
Endemic Burkitt's lymphoma is the most common childhood cancer in Uganda. Strongly linked to EBV and malaria. UCI has established treatment protocols.
References
- Uganda Clinical Guidelines (UCG) 2023
Chapter 12, Section 12.3.1
- Uganda Cancer Institute Treatment Guidelines
(Specific protocols)