44  Lymphoma

Author

xxx

Published

September 20, 2025

Lymphomas are malignant neoplasms arising from the lymphoid tissues and are the third most common childhood cancer after leukaemia and brain tumours. They account for approximately 10–15% of childhood malignancies. Lymphomas are broadly classified into Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL), with the latter being more frequent in children. Among NHL subtypes, Burkitt’s lymphoma is particularly common in tropical Africa, including Ghana, where it is one of the leading childhood cancers.

Understanding the epidemiology, aetiology, pathophysiology, clinical features, and treatment of childhood lymphoma is vital for medical students, as early diagnosis and timely intervention significantly improve outcomes.

44.1 Introduction

Lymphoma is a malignancy of the lymphoid system, originating from either B lymphocytes or T lymphocytes at various stages of differentiation. In children, the disease behaves more aggressively compared to adult lymphomas, but it is also more curable with modern treatment protocols.

  • Hodgkin lymphoma (HL): Characterised by the presence of Reed-Sternberg cells. Typically presents in older children and adolescents.
  • Non-Hodgkin lymphoma (NHL): Includes Burkitt’s lymphoma, lymphoblastic lymphoma, and large cell lymphoma. These are high-grade tumours with rapid proliferation.

44.2 Incidence and Prevalence

  • Lymphomas account for 10–15% of childhood cancers worldwide.
  • NHL is more common in children than HL, particularly in those under 10 years of age.
  • In sub-Saharan Africa, Burkitt’s lymphoma is endemic and accounts for up to 50% of childhood cancers in some regions.
  • Peak age for Burkitt’s lymphoma: 5–10 years.
  • Hodgkin lymphoma is less common in African children but occurs worldwide, often peaking in adolescence.
  • There is a slight male predominance, particularly in the case of NHL.

44.3 Aetiology

The development of lymphoma is multifactorial and involves both genetic and environmental influences.

  • Genetic predisposition: Mutations affecting oncogenes and tumour suppressor genes (e.g., MYC translocation in Burkitt’s lymphoma).
  • Infectious agents:
    • Epstein–Barr virus (EBV) is strongly associated with Burkitt’s lymphoma and some cases of HL.
    • Human immunodeficiency virus (HIV) predisposes to NHL.
  • Immunodeficiency states: Congenital (e.g., Wiskott–Aldrich syndrome) or acquired (HIV/AIDS, post-transplant immunosuppression).
  • Environmental factors: Chronic malaria infection in endemic regions contributes to immune dysregulation, facilitating EBV-driven oncogenesis in Burkitt’s lymphoma.

44.4 Pathophysiology

Lymphomas arise from clonal proliferation of lymphoid cells.

  • Hodgkin lymphoma:
    • Originates from germinal centre B-cells that become transformed into Reed-Sternberg cells.
    • These cells secrete cytokines that recruit inflammatory cells, explaining the prominent systemic symptoms.
  • Non-Hodgkin lymphoma:
    • High-grade and rapidly proliferating.
    • Burkitt’s lymphoma is characterised by a translocation involving the MYC gene on chromosome 8 (t(8;14) most common).
    • Malaria-induced chronic immune stimulation reduces T-cell control over EBV-infected B-cells, facilitating malignant transformation.
  • Organ infiltration: Lymphomas can spread to extranodal sites such as the bone marrow, CNS, and abdominal viscera.
  • The hallmark of Burkitt’s lymphoma in Africa is jaw involvement, though abdominal presentations are also common.

44.5 Signs and Symptoms

The presentation of lymphoma varies depending on the subtype and site of involvement.

  • General features:
    • Fever, weight loss, night sweats (“B symptoms”).
    • Fatigue, anorexia.
  • Hodgkin lymphoma:
    • Painless lymphadenopathy (often cervical or supraclavicular).
    • Mediastinal mass causing cough, dyspnoea, or SVC obstruction.
    • Hepatosplenomegaly.
  • Non-Hodgkin lymphoma:
    • Rapidly enlarging lymph nodes, often extranodal.
    • Abdominal involvement: distension, pain, palpable mass, intussusception, or bowel obstruction.
    • CNS infiltration: seizures, cranial nerve palsies, spinal cord compression.
    • Burkitt’s lymphoma:
      • Endemic type: jaw/facial bone swelling, often bilateral.
      • Sporadic type: abdominal masses, ileocecal involvement.

44.6 Differential Diagnosis

Conditions that mimic childhood lymphoma include:

  • Infectious diseases: Tuberculosis, HIV lymphadenopathy, EBV infection.
  • Other malignancies: Leukaemia, neuroblastoma, Wilms’ tumour.
  • Rheumatological disorders: Juvenile idiopathic arthritis, systemic lupus erythematosus.
  • Benign causes of lymphadenopathy: Reactive hyperplasia, cat-scratch disease.

44.7 Investigations

Evaluation of suspected lymphoma requires a combination of laboratory, imaging, and histological studies.

  • Laboratory tests:
    • Full blood count: may reveal anaemia, cytopenias if marrow involvement.
    • ESR and LDH: often elevated.
    • Uric acid and renal function: to assess for tumour lysis risk.
  • Imaging:
    • Chest X-ray: mediastinal mass.
    • Ultrasound/CT/MRI: delineate abdominal or nodal masses.
  • Histology:
    • Excisional lymph node biopsy is the gold standard.
    • Reed-Sternberg cells → Hodgkin lymphoma.
    • “Starry sky” appearance → Burkitt’s lymphoma.
  • Bone marrow aspiration and biopsy: To check for infiltration.
  • Lumbar puncture: Especially for Burkitt’s lymphoma and lymphoblastic lymphoma to detect CNS disease.

44.8 Treatment

Management depends on subtype, stage, and extent of disease. Multidisciplinary care is essential.

44.8.1 Emergency Management

  • Stabilise airway, breathing, circulation.
  • Manage tumour lysis syndrome: hydration, allopurinol or rasburicase.
  • Empirical antibiotics for febrile neutropenia.
  • Blood products as required.

44.8.2 Definitive (Ongoing) Therapy

  • Chemotherapy is the mainstay:
    • HL: ABVD (adriamycin, bleomycin, vinblastine, dacarbazine) or equivalent protocols.
    • NHL: intensive multiagent regimens (e.g., cyclophosphamide, vincristine, doxorubicin, methotrexate, cytarabine).
    • Burkitt’s lymphoma responds dramatically to short, intensive chemotherapy cycles.
  • Radiotherapy: Occasionally used in HL but less so in children due to long-term side effects.
  • CNS prophylaxis: Intrathecal chemotherapy for NHL.
  • Stem cell transplantation: Considered in refractory or relapsed cases.

44.8.3 Preparation for Discharge

  • Educate caregivers on infection prevention, adherence to chemotherapy, and recognition of complications.
  • Ensure follow-up schedules are clear.
  • Provide psychosocial and nutritional support.

44.8.4 Long-Term Management

  • Monitor for relapse with clinical examination and imaging as indicated.
  • Surveillance for late effects: growth disturbances, infertility, cardiotoxicity, secondary malignancies.
  • Ongoing psychosocial support and school reintegration.

44.9 Complications

  • Early: Tumour lysis syndrome, airway obstruction from mediastinal masses, sepsis.
  • During therapy: Chemotherapy toxicities (mucositis, myelosuppression, cardiotoxicity).
  • Late: Relapse, secondary cancers, endocrine dysfunction, infertility, psychosocial issues.

44.10 Prevention

  • No definitive primary prevention strategies exist.
  • Reducing malaria transmission may indirectly lower Burkitt’s lymphoma incidence.
  • HIV prevention and treatment help reduce NHL burden.
  • Early recognition and referral are key to improving survival.

44.11 Prognosis

  • Prognosis depends on subtype, stage, and response to therapy.
  • Hodgkin lymphoma: Excellent prognosis with >90% 5-year survival in early-stage disease.
  • Non-Hodgkin lymphoma: Cure rates of 70–90% with appropriate therapy.
  • Burkitt’s lymphoma: Rapidly fatal if untreated, but highly curable with intensive short-course chemotherapy. Survival is significantly improved when diagnosed early and managed promptly.

44.12 Conclusion

Childhood lymphoma is a significant health problem, particularly in sub-Saharan Africa, where Burkitt’s lymphoma is endemic. It is an aggressive but highly treatable malignancy. For medical students, key learning points include recognition of clinical presentations, understanding the role of EBV and malaria in endemic Burkitt’s lymphoma, and appreciating the importance of early diagnosis and intensive chemotherapy. With improved healthcare infrastructure, supportive care, and public health measures, outcomes for children with lymphoma can continue to improve.