Makindo Medical Notes

Related Subjects: |Hodgkin Lymphoma |Non Hodgkin Lymphoma |Diffuse large B-cell lymphoma |Intravascular large B-cell lymphoma |Mantle cell lymphoma |Marginal Zone Lymphoma |Gastric (MALT) Lymphoma |Primary CNS Lymphoma (PCNSL) |Burkitt's lymphoma |Follicular Lymphoma

🩸 Non-Hodgkin Lymphoma (NHL) is a heterogeneous group of blood cancers involving lymphoid cells. Unlike Hodgkin lymphoma, NHL includes many subtypes, most of which are of B-cell origin. Rituximab (anti-CD20) is effective in most B-cell NHLs. Elevated LDH is often a marker of fast-growing/aggressive disease.

📖 About

• 90% of lymphomas are NHL; 10% are Hodgkin lymphoma (HL).
• Malignant disorder of B, T or NK lymphocytes in lymphoid tissue.
• Peak incidence: Adults 20–40 and also later in life (>60).
• Associated with viral infections and immunosuppression.

🧾 Types of NHL

• B-cell lymphomas (~85% of cases):
  • Diffuse Large B-Cell Lymphoma (DLBCL): most common aggressive NHL.
  • Follicular Lymphoma: common indolent subtype, graded 1–3, associated with t(14;18).
  • CLL/SLL: indolent; affects blood + nodes.
  • Mantle Cell Lymphoma: aggressive, associated with t(11;14).
  • Burkitt Lymphoma: highly aggressive; linked to EBV and c-myc translocation t(8;14).
• T-cell lymphomas (rarer):
  • Peripheral T-Cell Lymphoma (PTCL).
  • Cutaneous T-Cell Lymphoma (CTCL): Mycosis fungoides, Sézary syndrome.
  • Angioimmunoblastic T-Cell Lymphoma (AITL).
  • Anaplastic Large Cell Lymphoma (ALCL): systemic or cutaneous.

⚠️ Risk Factors

• 🛡️ Immunodeficiency: HIV, post-transplant, congenital syndromes.
• 🤒 Autoimmune disease: RA, SLE, Sjögren, psoriasis, Crohn’s.
• 💊 Therapy-related: methotrexate, TNF-inhibitors, immunosuppressants.
• 🦠 Infections:
  • EBV → Burkitt, CNS lymphoma, PTLD.
  • HTLV-1 → Adult T-cell lymphoma.
  • H. pylori → gastric MALT.
  • HCV → splenic marginal zone lymphoma.
  • Borrelia → cutaneous marginal zone lymphoma.
  • HHV8 → Castleman’s, effusion lymphoma.
• 🧬 Genetics / Translocations:
  • t(8;14) Burkitt → c-myc.
  • t(14;18) Follicular → bcl-2 overexpression.
  • t(11;14) Mantle cell → cyclin D1.
  • t(2;5) ALCL.
  • bcl-6 mutations in DLBCL.
• Other: ↑ Age, Male sex, Chemicals (e.g. Agent Orange, pesticides), previous chemo/radiotherapy.

🧑‍⚕️ Clinical Features

• 🔺 B symptoms: fever, night sweats, weight loss.
• Persistent, painless lymphadenopathy.
• Extranodal disease common (GI, CNS, skin, marrow).
• Hepatosplenomegaly, ascites.
• Skin lesions (CTCL).
• Cytopenias: anaemia, thrombocytopenia, lymphocytosis.
• Neurological symptoms if CNS/spinal involvement.

📍 Clinical Classification

• Indolent NHL: slow-growing, median survival ~10 yrs.
  • CLL/SLL
  • Follicular lymphoma
  • Waldenström macroglobulinaemia
  • Marginal zone lymphoma (incl. gastric MALT)
  • Cutaneous T-cell lymphoma (Mycosis fungoides)
• Aggressive NHL: potentially curable, rapid progression.
  • DLBCL (commonest)
  • Burkitt lymphoma
  • Mantle cell lymphoma
  • Peripheral T-cell lymphoma
  • Primary mediastinal B-cell lymphoma
  • Sezary syndrome (advanced CTCL)

📊 Staging (Ann Arbor)

🧪 Investigations

• Bloods: FBC, U&E, LFT, LDH, ESR, urate.
• Lymph node excision biopsy (gold standard).
• Bone marrow aspiration & trephine.
• Immunophenotyping (flow cytometry, IHC).
• Cytogenetics (translocations).
• Imaging: CT/PET-CT for staging; MRI spine if cord compression suspected.
• LP if CNS disease suspected.
• MUGA/Echo before anthracyclines.
• HIV test if risk factors.

🏃 Performance Status (ECOG)

• 0 = Fully active
• 1 = Restricted strenuous activity
• 2 = Ambulatory >50% of day, unable to work
• 3 = Limited self-care, >50% bedbound
• 4 = Completely bedbound

💊 Management

• Indolent NHL
  • Stage I–II: RT alone.
  • Advanced: CVP-R (cyclophosphamide, vincristine, prednisone, rituximab) × 8 cycles → maintenance rituximab for 2 yrs.
  • Relapse: second-line chemo ± stem cell transplant.
• Aggressive NHL (e.g. DLBCL)
  • Stage I–II: R-CHOP × 3 cycles ± RT.
  • Stage III–IV: R-CHOP × 6 cycles, assess with PET-CT.
  • Residual/bulky disease: RT, intrathecal chemo if CNS risk.
  • Relapse: salvage (GDP-R, R-ICE) + autologous stem cell transplant if fit.
• Highly Aggressive NHL
  • Burkitt lymphoma → intensive multi-agent chemo ± CNS prophylaxis.
  • ALL/lymphoblastic lymphoma → treated like acute leukaemia.

📚 References

• NICE CKS: Lymphoma
• Cancer Research UK
• Oxford Handbook of Clinical Medicine (UK context)