Non-Hodgkin’s Lymphoma

“Disorder caused by neoplastic proliferation of lymphocytes, without the presence of Reed-Sternberg cells (large mononuclear neoplastic cells that fuse – “owl eye” appearance)“

Risk Factors

• Family History

Aetiology

• Immunodeficiency (drugs, HIV)
• Congenital
• HTLV-1 virus
• H.pylori
• Toxins

Pathophysiology

• Malignant transformations usually due to errors in gene arrangements of immunoglobulins or T cell receptors. There are B cell (more common) and T cell lymphomas:
  • B cell lymphomas (in order of increasing aggression)
    1. Lymphoplasmacytic: indolent, the involvement of bone marrow, lymph nodes, spleen
    2. Follicular: slow growing
    3. Marginal zone: most common, associated with mucosa-associated lymphoid tissue (MALT) in cases of chronic inflammation of stomach lining (chronic H.pylori infection)
    4. Diffuse large B cell lymphoma: more common, aggressive
    5. Mantel cell: aggressive
    6. Burkitt: highly aggressive, characterised by “starry sky appearance”
  • T cell lymphomas:
    • Adult T cell lymphoma
    • Mycosis fungoides: T cell lymphoma of the skin, resembles a fungal infection

Clinical Presentation

• Painless lymphadenopathy (75%)
• Extranodal disease (25%): skin infections, sore throat, gastric cancer symptoms with systemic feature (MALT), fatigue, motor/sensory deficits, recurrent infections, easy bruising

Investigations

• Bloods: FBC, U+E, LFTs (increase in LDH = worst prognosis as it reflects an increase in cell turnover)
• Marrow and nodal biopsy for classification
• CT, PET scan

Management

• Low grade: if no symptoms, no treatment may be necessary. However if symptoms present: radiotherapy + chemotherapy (Chlorambucil, Alpha-interferon, Rituximab, Bendamustine
• High grade: Chemotherapy (R-CHOP regime – Rituximab, Cyclophosphamide, Hydroxydaunorubicin, Vincristine/Oncovin, Prednisolone)