Overview Of Non-Hodgkin’s Lymphoma In Children

The Neck Lump That Doesn’t Hurt — and Won’t Go Away on Its Own , a painless swelling is easy to ignore. Biopsy is what separates lymphoma from reassurance.

A man in his fifties notices a firm swelling on the side of his neck. It has been there for six weeks. It does not hurt. He has had sore throats before; this feels different, but not alarming. At a polyclinic in Accra, he receives antibiotics. The lump remains. Three months later, at a district hospital in Tamale, a doctor examines the node — rubbery, persistent, in the cervical chain — and asks about weight loss and drenching night sweats. He has lost weight without trying. The doctor does not aspirate with a fine needle and send him home. An excisional biopsy is arranged. Histology, immunohistochemistry, and flow cytometry return a diagnosis: diffuse large B-cell lymphoma. That delay — treating a painless node as infection — is not unusual. It is, in many ways, the story
of non-Hodgkin’s lymphoma.

What Non-Hodgkin’s Lymphoma Actually Is

Non-Hodgkin’s lymphoma (NHL) is a broad group of cancers arising from the lymphatic system — particularly from lymphocytes, the white blood cells responsible for immune defence. It is one of the most common haematological malignancies worldwide, affecting both adults and children, though more frequent in older adults. Unlike Hodgkin’s lymphoma, NHL is not a single disease but a collection of distinct lymphoid malignancies — uncontrolled proliferation of B lymphocytes, T lymphocytes, or natural killer (NK) cells in lymph nodes or extranodal tissues. It includes all lymphomas that do not show the classical Reed-Sternberg cells of Hodgkin’s lymphoma. Subtypes vary in behaviour, treatment response, and prognosis. Examples of subtypes include:
 Diffuse large B-cell lymphoma (DLBCL)

 Follicular lymphoma
 Burkitt lymphoma
 Mantle cell lymphoma
 Marginal zone lymphoma
 Peripheral T-cell lymphoma
 Cutaneous T-cell lymphoma
The disease may develop in lymph nodes or in extranodal tissues such as the stomach, intestines, skin, brain, bone marrow, and spleen. Some forms progress slowly over years; others are aggressive and grow rapidly. Advances in diagnostic methods, chemotherapy, immunotherapy, and targeted treatment have significantly improved outcomes.

Causes and Who Is at Risk
The exact cause is not fully understood, but several factors increase risk:
Genetic mutations — changes in DNA within lymphocytes cause abnormal growth and resistance to cell death. Examples include t(14;18) translocation in follicular lymphoma, c-MYC translocation in Burkitt lymphoma, and BCL6 abnormalities in diffuse large B-cell lymphoma.
Immunodeficiency states — HIV/AIDS, congenital immunodeficiency disorders, post-organ transplant immunosuppression, and long-term use of immunosuppressive drugs.
Autoimmune diseases — chronic immune stimulation may predispose to lymphoma: rheumatoid arthritis, Sjögren syndrome, systemic lupus erythematosus, Hashimoto thyroiditis.

Infections linked to specific subtypes:
 Epstein-Barr virus (EBV) — Burkitt lymphoma, some aggressive lymphomas
 Helicobacter pylori — gastric MALT lymphoma
 Hepatitis C virus — marginal zone lymphoma
 Human T-cell leukemia virus type 1 (HTLV-1) — adult T-cell lymphoma
 Human herpesvirus 8 (HHV-8) — primary effusion lymphoma

Environmental and occupational exposure — pesticides, herbicides, benzene, radiation, certain industrial chemicals.

Age and gender — incidence increases with age; slight male predominance in many subtypes.Family history — a close relative with lymphoma or haematological cancer may slightly increase risk.
There is no guaranteed prevention, but risk may be reduced by HIV prevention and treatment, avoiding unnecessary chemical exposure, managing autoimmune diseases, treating chronic infections such as H. pylori, and regular medical review of persistent lymph node swelling.

How NHL Develops: From Mutation to Mass
NHL develops when lymphocytes undergo malignant transformation. Normally, lymphocytes grow, mature, perform immune functions, and die in an organised manner. In NHL, mutations disrupt this balance:

1. Genetic damage — DNA mutations or chromosomal translocations occur in B-cells, T-cells, or NK cells
2. Loss of growth control — mutated cells multiply uncontrollably and evade apoptosis (programmed cell death)
3. Clonal expansion — the abnormal cell reproduces identical malignant cells, forming a clone
4. Tissue infiltration — cancer cells accumulate in lymph nodes, bone marrow, spleen, liver, gastrointestinal tract, skin, and central nervous system
5. Immune dysfunction — normal lymphocyte production is impaired, leading to recurrent infections and reduced immunity
6. Organ damage — large tumour masses or diffuse infiltration interfere with organ function

Indolent, Aggressive, and Highly Aggressive: Three Speeds
NHL can be broadly divided into:
Indolent (slow growing) — follicular lymphoma, small lymphocytic lymphoma, marginal zone
lymphoma. These may remain stable for years but can relapse repeatedly.
Aggressive (fast growing) — diffuse large B-cell lymphoma, mantle cell lymphoma, peripheral T-cell lymphoma. These require urgent treatment.
Highly aggressive — Burkitt lymphoma, lymphoblastic lymphoma. Rapid progression but often responsive to intensive therapy.

What to Look For: Signs and Symptoms
Clinical presentation depends on subtype, site, and stage.
Lymphadenopathy — the most common feature
Enlarged lymph nodes, usually painless, commonly in the neck, axilla, or groin. Nodes may be firm, rubbery, and persistent. This is the presentation that sends too many patients home with antibiotics when biopsy is what they need.

Constitutional (B) symptoms — systemic disease
 Unexplained fever
 Drenching night sweats
 Weight loss (more than 10% body weight in 6 months)
Other features

 Fatigue and weakness from anaemia, cytokine release, or chronic disease
 Recurrent infections from impaired immune function
 Hepatosplenomegaly — abdominal fullness from enlarged liver or spleen
 Mediastinal mass — cough, chest pain, dyspnoea, superior vena cava obstruction
Extranodal symptoms
 Gastrointestinal — abdominal pain, nausea, vomiting, bowel obstruction, GI bleeding
 CNS — headache, seizures, weakness, personality changes
 Skin — nodules, plaques, itching
 Bone marrow — pallor, easy bruising, bleeding, infections

When to Suspect NHL — and What to Order
A doctor should consider NHL when a patient presents with:
 Persistent painless lymph node enlargement
 Unexplained weight loss
 Night sweats
 Fever of unknown origin
 Hepatosplenomegaly
 Recurrent infections
 Unexplained cytopenias
 Mass lesions in extranodal sites

Prompt biopsy is essential. This cannot be overstated.
Take a full history — symptom duration, B symptoms, exposure, HIV risk, autoimmune disease, drug use — and examine all lymph node regions, liver, spleen, skin, and neurology.
Laboratory investigations
Test Role
Complete blood count (CBC) May reveal anaemia, leukopenia, leukocytosis, thrombocytopenia
Peripheral blood film May show abnormal lymphoid cells
ESR / CRP Markers of inflammation
LDH Often elevated in aggressive disease and high tumour burden

Uric acid May be elevated due to rapid cell turnover
Liver function tests Assess liver involvement and chemotherapy

readiness

Renal function tests Important before treatment
Viral screening HIV, hepatitis B, hepatitis C

Tissue diagnosis — the gold standard

Excisional lymph node biopsy is the gold standard investigation. A whole node is removed for histology, immunohistochemistry, flow cytometry, and cytogenetics. Fine needle aspiration alone is usually insufficient.
Bone marrow aspiration and trephine biopsy are used for staging and marrow involvement.
Imaging and special tests
Chest X-ray (mediastinal mass), CT of neck/chest/abdomen/pelvis (staging), PET-CT (extent and response), MRI (CNS, spine, soft tissue), lumbar puncture if CNS involvement suspected, and molecular studies for genetic abnormalities and prognosis.

A note for practice in Ghana
Excisional biopsy and full immunophenotyping may not be available at every CHPS compound or district hospital. If you have a persistent painless node with B symptoms or extranodal signs, do not settle for repeated courses of antibiotics without tissue diagnosis. Refer to a centre with surgical biopsy capacity and haematology/oncology support. Teaching hospitals in Accra, Kumasi, Tamale, and other regional hubs are where the diagnostic pathway completes. NHIS may cover aspects of investigation and treatment — verify current benefit packages with your facility’s claims desk, but do not let financial uncertainty delay referral when clinical suspicion is high.

Staging: Ann Arbor
The Ann Arbor staging system is commonly used:
 Stage I — single lymph node region or single extranodal site
 Stage II — two or more lymph node regions on same side of diaphragm
 Stage III — both sides of diaphragm involved
 Stage IV — disseminated extranodal disease (bone marrow, liver, CNS)
Suffixes: A = no B symptoms; B = fever, sweats, weight loss.
The International Prognostic Index (IPI) helps risk stratification. Poor prognostic factors include advanced age, high LDH, poor performance status, advanced stage, and multiple extranodal sites.

Complications: Disease and Treatment
Disease-related complications
Bone marrow failure (severe anaemia, infection, bleeding), organ compression (airways, ureters, blood vessels, spinal cord), CNS spread, intestinal obstruction or perforation with abdominal lymphoma, hypercalcaemia in some subtypes, and tumour lysis syndrome — rapid cell destruction causing hyperuricaemia, hyperkalaemia, hyperphosphataemia, and renal failure.

Treatment-related complications
Neutropenic sepsis (medical emergency after chemotherapy), cardiotoxicity from drugs such as doxorubicin, infertility after chemotherapy/radiotherapy, and secondary malignancies long- term.

Treatment: Individualised and Urgent When Aggressive
Treatment depends on histological subtype, stage, age, performance status, organ function, and presence of symptoms. Management is best coordinated by a haematologist/oncologist.
Supportive care is essential in all patients: nutrition, pain control, hydration, infection prevention, psychological support, transfusion if needed.
Watchful waiting suits selected indolent lymphomas without symptoms — monitor until treatment is necessary.
Chemotherapy is the mainstay for many forms. R-CHOP — rituximab, cyclophosphamide, doxorubicin, vincristine, prednisolone — is common for diffuse large B-cell lymphoma; other regimens depend on subtype.
Immunotherapy (rituximab, a monoclonal antibody against CD20 on B-cells, often combined with chemotherapy), targeted therapy (ibrutinib, acalabrutinib, venetoclax, lenalidomide for relapsed or specific subtypes), radiotherapy (localised early-stage disease, bulky masses, palliation), stem cell transplant (autologous or allogeneic for relapsed or high-risk disease), and
CAR T-cell therapy (for some refractory B-cell lymphomas) round out modern options.

Managing acute complications
 Tumour lysis syndrome — IV fluids, allopurinol, rasburicase, electrolyte correction
 Febrile neutropenia — immediate IV antibiotics, cultures, supportive care
 Spinal cord compression — emergency steroids, MRI, radiotherapy or surgery

Prognosis and What Patients Should Know
Outcome depends on subtype and stage. Indolent lymphomas may be chronic but manageable for years; aggressive lymphomas can often be cured if treated early. Modern combined therapy makes many forms highly treatable — but only after biopsy confirms the diagnosis. If a neck lump has persisted for weeks without biopsy, you are not being difficult for asking again. Bring a timeline of symptoms. Ask explicitly about excisional biopsy and referral. Clinicians: a rubbery cervical node with weight loss deserves tissue, not reassurance alone.

Key Takeaways
 NHL is a diverse group of lymphoid malignancies — not one disease

 Persistent painless lymphadenopathy is the classic presentation; B symptoms suggest systemic disease
 Excisional biopsy is the gold standard — fine needle aspiration alone is usually insufficient
 Staging uses Ann Arbor; IPI helps prognostic stratification
 R-CHOP is a cornerstone regimen for DLBCL; treatment must be subtype-specific
 Aggressive lymphomas require urgent referral; indolent forms may be watched initially
 Early biopsy and accurate staging significantly improve survival

Success in NHL is often partial at first — a confirmed diagnosis, a staging scan, a treatment plan that matches the subtype. That partial success begins with the biopsy nobody wanted to delay.

For specialist haematology/oncology care in Ghana, ask your primary clinician about referral to a teaching hospital or accredited cancer centre. If you are a health worker and want to discuss a suspected case, document the node characteristics and B symptoms, arrange excisional biopsy, and refer without delay when suspicion is high.

Medical disclaimer: This article is for general health education only. It does not replace
examination, diagnosis, or treatment by a qualified doctor. If you have a persistent lump in your
neck, armpit, or groin — especially with fever, night sweats, or unexplained weight loss —
please seek care at your nearest health facility.