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
🧬 The classical hallmark of Mantle Cell Lymphoma is the chromosomal translocation t(11;14), leading to dysregulation of cyclin D1 and uncontrolled B-cell proliferation.
ℹ️ About
- 🎗️ Aggressive B-cell non-Hodgkin lymphoma (NHL), accounting for ~6% of NHL cases
- 👨 Male predominance: Male:Female ≈ 3:1
- 📈 Median age at diagnosis: 60–65 years
🧬 Aetiology
- ⚡ Overexpression of cyclin D1 → drives cell cycle progression
- 🔗 Usually caused by translocation t(11;14)(q13;q32) between chromosomes 11 and 14
- 📌 Sometimes cyclin D1-negative → cyclin D2/D3 overexpression may substitute
🩺 Clinical Features
- 🔻 "B symptoms": fever, night sweats, weight loss, anorexia
- 🤢 GI involvement: nausea, abdominal pain, bloating (“lymphomatous polyposis” of the gut)
- 🗣️ Fullness/discomfort from enlarged tonsils, lymphadenopathy
- 🫀 Hepatosplenomegaly, abdominal swelling
- 🩸 Anaemia → fatigue; cytopenias common
- ⚡ Back pain or radicular pain from nodal/organomegaly compressing nerves
🔎 Investigations
- 🩸 FBC: cytopenias (low Hb, WCC, platelets) or leukocytosis
- 🧪 U&E, LFTs, LDH, CRP, ESR
- 📊 Prognostic markers: serum LDH & β2-microglobulin
- 🖥️ Imaging: CT chest/abdomen/pelvis for staging; PET often used
- 🧬 Bone marrow biopsy for staging (common marrow involvement)
🔬 Pathology
- 🔍 Lymph node biopsy: B-cell markers (CD20+, CD5+)
- 🧪 Cyclin D1 overexpression (IHC) = diagnostic hallmark
- 📌 Genetic hallmark: translocation t(11;14)
- Other markers: SOX11 positivity (useful when cyclin D1-negative)
💊 Management
- 🌟 Stage I–II (localised disease): radiotherapy ± chemo-immunotherapy
- 🌟 Advanced, symptomatic disease:
– Immunochemotherapy + Rituximab (anti-CD20)
– Cytarabine-containing regimens (e.g. R-DHAP, R-CHOP + cytarabine) in fit patients
– R-CHOP alone is still widely used standard
- 💉 Bortezomib (proteasome inhibitor) for patients unsuitable for transplantation
- 💊 Ibrutinib (BTK inhibitor) for relapsed/refractory disease
- 🧬 Autologous stem cell transplantation: considered in first remission in eligible patients
- 📑 NICE reviewing bendamustine in combination regimens for MCL
📚 References