Page 23 of 34

PE26.9 | Lymphoma in Children — SDL Guide (Part 2)

Management of Lymphoma

The management of lymphoma in children is risk-stratified by subtype, stage, and disease extent — and the goal in both HL and most NHL is cure. Modern paediatric lymphoma treatment achieves cure rates that are among the best in oncology, and minimising late treatment toxicity (particularly from radiotherapy) is an increasingly important part of therapy planning.

Provided image

Hodgkin lymphoma:
HL is one of the most chemotherapy-sensitive and radiotherapy-sensitive malignancies. The cornerstone chemotherapy regimen for paediatric HL is ABVD — Adriamycin (doxorubicin), Bleomycin, Vinblastine, Dacarbazine — given in 28-day cycles. Alternative regimens used in paediatric/adolescent protocols include OEPA (vincristine, etoposide, prednisolone, doxorubicin) and COPDAC (cyclophosphamide, vincristine, prednisolone, dacarbazine) based on risk group and sex. Involved-field radiotherapy (IFRT) was historically standard after chemotherapy; in modern paediatric protocols, the aim is to omit or minimise radiotherapy (especially in early-stage/good responders assessed by interim PET) to reduce long-term risks (secondary malignancy, cardiopulmonary toxicity, growth plate effects). Early-stage HL (Stage IA/IIA, no bulky disease, good early response) may achieve >95% cure without radiotherapy in current protocols.

Burkitt lymphoma:
Burkitt is highly chemosensitive but also extremely aggressive — the combination of rapid growth and high TLS risk at initiation of therapy makes it a haematological emergency. Treatment consists of intensive, short-course multi-agent chemotherapy (typically 3–6 cycles over 3–6 months, far shorter than HL) using protocols such as CODOX-M/IVAC, LMB/FAB, or BFM. Key drugs include high-dose cyclophosphamide, high-dose methotrexate, cytarabine, vincristine, and rituximab (anti-CD20, for mature B-cell NHL). CNS prophylaxis with intrathecal methotrexate is mandatory. Tumour lysis syndrome prophylaxis (aggressive IV hydration, allopurinol or rasburicase) must be started BEFORE chemotherapy, particularly in patients with high-bulk disease — large abdominal Burkitt carries one of the highest TLS risks of any tumour.

T-lymphoblastic lymphoma:
When bone marrow blasts are <25%, the entity is staged and treated as NHL (T-lymphoblastic lymphoma) using ALL-type intensive multi-drug protocols (similar to high-risk T-ALL induction/consolidation/maintenance). When marrow blasts ≥25%, it is reclassified as T-ALL and treated on ALL protocols. CNS prophylaxis (intrathecal chemotherapy) is essential given the high CNS tropism.

ALCL:
ALK-positive ALCL is treated with CHOP-based chemotherapy (cyclophosphamide, doxorubicin, vincristine, prednisolone) with good outcomes; targeted therapies (ALK inhibitors) are in clinical trials.

Self-Assessment

Apply your knowledge to the following case.

Case: A 14-year-old boy presents with a 3-month history of painless, progressive bilateral cervical and right axillary lymphadenopathy. He has had fever for 6 weeks (temperature 38.5–39°C, coming and going), drenching night sweats, and has lost 9 kg over the past 3 months. Chest X-ray shows a mediastinal mass. CBC: Hb 9.8 g/dL (mild normocytic anaemia), WBC 10,400/µL (normal), platelets 280,000/µL. LDH is elevated. CT chest/abdomen shows bilateral cervical, mediastinal, and right axillary nodes; no abdominal nodal enlargement; no hepatosplenomegaly. PET-CT confirms FDG-avid bilateral cervical, mediastinal, and axillary disease only.

Q1: What is the Ann Arbor stage?
Answer: Stage IIB — disease in two or more lymph node regions (cervical, mediastinal, axillary) on one side of the diaphragm (above); suffix B because fever, night sweats, and >10% weight loss (B symptoms) are all present. (All disease is above the diaphragm = Stage II, not Stage III.)

Q2: Why is a bone marrow biopsy indicated before starting treatment?
Answer: To exclude Stage IV disease (bone marrow involvement), which would change treatment intensity. Marrow infiltration in HL changes Ann Arbor staging to IV and requires more intensive therapy.

Q3: Before scheduling a biopsy under general anaesthesia, what is the most important safety step?
Answer: Quantify tracheal compression from the mediastinal mass with CT/MRI, involve a senior anaesthetist, and be prepared for airway collapse. If peripheral nodes are accessible, peripheral node biopsy under local anaesthesia is safer. General anaesthesia with a large mediastinal mass carries risk of complete airway obstruction.

Q4: What chemotherapy backbone is used for this patient's diagnosis, and what determines whether radiotherapy is added?
Answer: ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine) is the standard HL chemotherapy. Whether radiotherapy is added depends on interim PET response after 2–4 cycles (PET-guided adaptive therapy) and the stage/risk group — early complete metabolic responders may omit RT to reduce late toxicity.