Lymphoma
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Background
Lymphomas are a group of diseases caused by malignant lymphocytes that accumulate in lymph nodes and other lymphoid tissue, leading to the characteristic clinical feature of lymphadenopathy.The major subdivision of lymphomas is into Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL), based on the histological presence of Reed-Sternberg cells in HL.
Hodgkin Lymphoma (HL)
Hodgkin lymphoma is defined by the presence of Reed–Sternberg cells. It is broadly subclassified into classical Hodgkin lymphoma (approximately 95% of cases, including lymphocyte-rich, nodular sclerosing, mixed cellularity, and lymphocyte-depleted subtypes) and nodular lymphocyte-predominant Hodgkin lymphoma (around 5% of cases), the latter being characterised by the absence of classic Reed–Sternberg cells and the presence of 'popcorn' or lymphocytic and histiocytic (L&H) cells.Incidence
- Can present at any age but rare in children.
- Peak incidence in young adults.
- Bimodal age distribution: first peak in ages 15–35, second peak in ages 55–60
- Male predominance of almost 2:1.
Clinical features
- Lymphadenopathy: typically painless, asymmetrical, firm with discrete superficial lymph node enlargement. Lymphadenopathy usually starts in a single peripheral region then spreads contiguously through the lymphatic system. Retroperitoneal nodes are often involved and typically diagnosed via CT scan.
The lymph nodes involved in HL are typically:
- Cervical: 60–70%
- Axillary: 10–15%
- Inguinal: 6–12%
- Splenomegaly: affects approximately 50% during disease course
- Hepatomegaly
- Mediastinal involvement: up to 10% at presentation; may cause pleural effusions or SVC obstruction.
- Cutaneous involvement: late complication in ~10%.
- Constitutional symptoms (B symptoms - see box below)
B symptoms in Hodgkin Lymphoma
- Fever (~30%)
- Pruritus (~25%)
- Alcohol-induced pain at disease sites
- Weight loss
- night sweats
- fatigue
- weakness
- anorexia
- cachexia
Investigations
- Normochromic normocytic anaemia common; bone marrow involvement rare early on.
- Neutrophilia in one-third; eosinophilia frequent.
- Advanced disease: lymphopenia, impaired cell-mediated immunity.
- Platelets: normal/high early, low later.
- ESR & CRP: usually raised. ESR used for monitoring.
- Serum LDH: raised in 30–40%.
- Diagnosis: excisional lymph node biopsy; multinucleated polypoid Reed-Sternberg cells key for diagnosis.
Staging
The Ann Arbor classification is used to stage both Hodgkin and most non-Hodgkin lymphomas. The Ann Arbor classification with Cotswolds modifications is shown below:
| Stage | Description |
|---|---|
| I | Involvement of a single lymph node region (I) or a single extralymphatic organ/site (IE) |
| II | Involvement of two or more lymph node regions on the same side of the diaphragm (II) or with limited contiguous extralymphatic extension (IIE) |
| III | Involvement of lymph node regions on both sides of the diaphragm (III) which may also be accompanied by spleen involvement (IIIS) or limited contiguous extralymphatic extension (IIIE) |
| IV | Disseminated involvement of one or more extralymphatic organs (e.g. bone marrow, liver, lungs) with or without associated lymph node involvement |
A – No systemic (B) symptoms
B – Presence of systemic symptoms: fever >38°C, drenching night sweats, unexplained weight loss >10% in 6 months
E – Localised extralymphatic involvement contiguous with lymph node disease
S – Spleen involvement
Management
- Treatment choice guided by stage & grade.
- Staging: history, examination, bloods, PET & CT scans.
- Therapy: chemotherapy ± radiotherapy.
- Relapse: chemotherapy ± stem cell transplant.
Prognosis
- Dependent on age, stage, histology.
- 5 year survival >90% for stage 1 and 50-70% in Stage 4.
- ~ 85% cure rate overall.
Non-Hodgkin Lymphoma (NHL)
Background
Non-Hodgkin Lymphoma is a large, heterogeneous group of clonal lymphoid tumours with ~85% being B-cell type and the remaining 15% of T cell or NK-cell origin. NHL has a more variable presentation & natural history than HL. Irregular spread patterns are not uncommon and many present with extra-nodal disease.Clinical features
- Painless, asymmetric superficial lymphadenopathy.
- Constitutional symptoms (B symptoms).
- Oropharyngeal involvement (sore throat, noisy/obstructed breathing).
- Symptoms from cytopenias (anaemia, neutropenia, thrombocytopenia).
- Abdominal disease (hepatosplenomegaly, retroperitoneal/mesenteric node involvement).
- Infiltration of other organs (skin, brain, testis, thyroid).
Investigations
- Definitive diagnosis: whole node excision or trucut biopsy (lymph node or extranodal site), plus immunophenotyping ± genetic analysis.
- Blood tests: FBC, Blood film, ESR or CRP, U&Es, LFTs, LDH (elevated in high-grade disease and has prognostic value), Beta-2 microglobulin (additional prognostic marker), viral serology prior to initiating monoclonal antibody treatment (HIV, hepatitis B & C).
- PET/CT imaging.
Management
- Initial therapy: combination chemotherapy + monoclonal antibody (e.g., rituximab for CD20+ B-cell NHL).
- Emerging therapies may alter future management.
Summary Table: Hodgkin vs Non-Hodgkin Lymphoma
| Feature | Hodgkin Lymphoma | Non-Hodgkin Lymphoma |
|---|---|---|
| Cell origin | B-cell (Reed-Sternberg cells) | B-cell (85%), T/NK-cell (15%) |
| Spread pattern | Contiguous spread | Irregular/non-contiguous spread |
| Extranodal disease | Rare | Common |
| Peak age | Young adults (bimodal distribution) | Older adults (varies by subtype) |
| Male:female ratio | ~2:1 | Variable |
| Prognosis | 85% cure rate | Variable, depends on subtype and stage |