Lymphomas
Overview
Lymphomas are malignant tumours of the lymphoreticular system, classified into:
-
Hodgkin Lymphoma (HL)
-
Non-Hodgkin Lymphoma (NHL)
Hodgkin Lymphoma (HL)
Description
| Feature | Details |
|---|---|
| Cell of origin | Germinal centre B-lymphocytes |
| Histology | Presence of Hodgkin Reed–Sternberg (HRS) cells |
| Forms | Childhood (≤14 yrs); Young adult (15–34 yrs) |
Signs and Symptoms
| Symptoms | Notes |
|---|---|
| Progressive lymphadenopathy | Cervical, axillary, supraclavicular |
| B symptoms | Fever >38°C, drenching night sweats, weight loss ≥10% in 6 months |
| Other features | Loss of appetite, pruritus, pallor, alcohol-induced pain in involved tissue, hepatosplenomegaly |
Investigations
| Investigation | Purpose |
|---|---|
| Excisional lymph node biopsy | Diagnostic |
| FBC/DC | Baseline haematology |
| LFTs, Urea, Creatinine, Electrolytes | Organ function |
| Serum ferritin | Disease activity |
| ESR, LDH | Biomarkers |
| Chest X-ray | Mediastinal involvement |
| Abdominal–pelvic ultrasound | Staging |
| Contrast-enhanced CT (skull base → mid-thigh) | Staging |
| HIV test ± CD4 | Risk association |
| Bilateral bone marrow aspirate & trephine | Stage III/IV or presence of B-symptoms |
Staging (Lugano classification, Cotswold modifications)
| Stage | Description |
|---|---|
| I | One lymph node region |
| II | ≥2 regions on same side of diaphragm |
| III | Regions on both sides of diaphragm |
| IV | Extra-lymphatic organ involvement (lung, liver, bone, marrow) |
| Modifier | Meaning |
|---|---|
| A | No B symptoms |
| B | B symptoms present |
| Bulky Disease | Definition |
|---|---|
| Present | Abdominal LN ≥10 cm, mediastinal mass ≥10 cm or >1/3 thoracic diameter, nodal conglomerate ≥6 cm |
| Extra-nodal “E” | Definition |
|---|---|
| Limited extension | Stage I–II disease only |
| Diffuse/disseminated | Considered stage IV |
Treatment
Low Stage (I & II, no bulky disease, no B-symptoms)
ABVD every 4 weeks × 4 cycles
| Drug | Dose & Schedule |
|---|---|
| Doxorubicin | 25 mg/m² D1 & 15 |
| Bleomycin | 10 units/m² D1 & 15 |
| Vinblastine | 6 mg/m² D1 & 15 |
| Dacarbazine | 375 mg/m² D1 & 15 |
-
Assess response after 4–6 weeks post-chemo.
-
CR → No radiotherapy
-
Non-CR → Low-dose involved field radiotherapy (LD-IFRT)
High Stage (III & IV, bulky disease or B-symptoms)
ABVD × 6 cycles every 4 weeks
OR
OEPA/COPDac × 6 cycles every 3 weeks
Assessment same as above.
OEPA Regimen × 2 cycles
| Drug | Dose |
|---|---|
| Vincristine | 1.5 mg/m² D1, 8, 15 |
| Etoposide | 125 mg/m² D3–6 |
| Prednisolone | 60 mg/m² D1–14 |
| Doxorubicin | 40 mg/m² D1 & 15 |
COPDac Regimen × 4 cycles
| Drug | Dose |
|---|---|
| Cyclophosphamide | 500 mg/m² D1 & 8 |
| Vincristine | 1.5 mg/m² D1 & 8 |
| Prednisolone | 60 mg/m² D1–14 |
| Dacarbazine | 250 mg/m² D1–3 |
3. Non-Hodgkin Lymphoma (NHL)
Description
| Feature | Details |
|---|---|
| Nature | Heterogeneous group |
| Association | HIV/AIDS & immunodeficiency ↑ risk ×100 |
| Pediatric pattern | Mostly aggressive/high-grade |
| Gender | Male predominance |
| Age | Rare <5 yrs |
Investigations
| Investigation | Purpose |
|---|---|
| FBC, U&E, LFTs | Baseline |
| ESR, LDH | Tumour markers |
| Lumbar puncture | CSF cytology |
| Biopsy (LN or extranodal) | Histology, IHC, flow, cytogenetics |
| Bone marrow biopsy | Staging |
| CXR/Abdo US | In absence of CT |
| Contrast CT | Full staging |
| ECG & Echo | Baseline cardiac |
Treatment
-
Lymphoblastic lymphoma → Treat using low-risk and high-risk ALL regimens (per Zambian Paediatric Protocols).
-
Refer to detailed protocols for staging-specific regimens.
Burkitt Lymphoma (BL)
Classification
| Type | Notes |
|---|---|
| Endemic | Common in malaria-endemic areas |
| Sporadic | Non-malarial regions |
| Immunodeficiency-related | HIV-associated |
Signs and Symptoms
| Feature | Endemic | Sporadic | Immunodeficiency-related |
|---|---|---|---|
| Peak age | 4–7 yrs | 6–12 yrs | Any |
| Gender | M>F | M>F | — |
| Primary site | Jaw/face > abdomen | Abdomen > jaw/face | LN, BM, CNS |
| Metastatic sites | CSF, breast, ovary, kidney, marrow, CNS | CNS, marrow, GI, liver, lung | As above |
Investigations
Same as NHL.
Treatment
B3 Regimen (6-week protocol)
| Week | Treatment |
|---|---|
| 1 | Vincristine, Cyclophosphamide 1200 mg/m², Prednisolone D1–5, IT MTX D1 |
| 2 | Vincristine, Doxorubicin 60 mg/m², Cyclophosphamide 1800 mg/m², IT MTX |
| 3–6 | Vincristine, Cyclophosphamide ± Etoposide depending on week, IT MTX |
Response assessment: 6 weeks
CODOX-M / IVAC Regimen
| Regimen | Components |
|---|---|
| CODOX-M | Cyclophosphamide, Vincristine, Doxorubicin, High-dose MTX + Folinic acid rescue |
| IVAC | Ifosfamide + MESNA, Etoposide, Cytarabine |
Cycles
-
Localised: 3 cycles CODOX-M
-
Disseminated: CODOX-M alternating with IVAC × 3 cycles each
CNS Prophylaxis
Includes intrathecal cytarabine, methotrexate, hydrocortisone, and folinic acid rescue.
Supportive Care
| Intervention | Notes |
|---|---|
| Anti-emetics | Dexamethasone 0.15 mg/kg/day; Ondansetron 0.15 mg/kg 8-hourly × 2–3 days |
| Tumour lysis prevention | Fluids 3L/m²/day × 5–7 days, monitor UO, electrolytes q12h |
| Allopurinol | 10–20 mg/kg/day × 7 days |
| MESNA | 360 mg/m² with cyclophosphamide/ifosfamide |
| Filgrastim | 5 mcg/kg/day until ANC >1.0 ×109/L |