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Mantle Cell Lymphoma

Aggressive B-cell lymphoma marked by t(11;14) cyclin D1 overexpression, often presenting at advanced stage with bone marrow and GI involvement.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Hematoloji department. Book Appointment →

What is Mantle Cell Lymphoma?

Mantle cell lymphoma (MCL) is a distinct subtype of B-cell non-Hodgkin lymphoma accounting for 5–7% of cases. It arises from naive pre-germinal center B cells of the mantle zone and is defined by the t(11;14)(q13;q32) translocation that overexpresses cyclin D1. Median age at diagnosis is approximately 65–68 years with strong male predominance.

Variants include classical (aggressive) MCL and a leukemic non-nodal indolent subtype expressing low levels of SOX11. Blastoid and pleomorphic histologies indicate aggressive behavior. Important prognostic factors include MIPI score, Ki-67 proliferation index, TP53 mutation/deletion (high-risk), and complex karyotype. CNS involvement occurs in 5–10% with worse outcomes.

Frontline therapy is divided by fitness: younger fit patients receive intensive cytarabine-containing induction (Nordic, R-DHAP, hyper-CVAD) followed by autologous stem cell transplantation and rituximab maintenance; older patients receive bendamustine plus rituximab (BR) or R-CHOP with maintenance rituximab. Relapsed/refractory disease responds to BTK inhibitors (ibrutinib, acalabrutinib, zanubrutinib), CAR T-cell therapy (brexucabtagene autoleucel), venetoclax combinations, and bispecific antibodies (glofitamab).

Symptoms

Generalized lymphadenopathy
Splenomegaly (often massive)
Bone marrow involvement with peripheral blood lymphocytosis
GI tract involvement (multiple lymphomatous polyposis, colon, ileum)
B symptoms (fever, night sweats, weight loss)
Hepatomegaly
Fatigue, anemia
CNS involvement (5–10%)
Tumor lysis at presentation
Compressive symptoms
GI bleeding from polyposis
Waldeyer ring involvement
Skin lesions (rare)
Pleural effusion
Asymptomatic incidental finding

Risk Factors

Male sex (3:1)
Age 60–70
Caucasian ethnicity
t(11;14) cyclin D1 translocation
SOX11 expression
TP53 mutation/deletion
Complex karyotype
Family history of B-cell malignancy
Pesticide exposure (farming)
Immunosuppression (less specific)
Hair dye, organic solvents (debated)
Smoking (weak)
Autoimmune disease (weak)
Idiopathic in most
No reliable lifestyle prevention

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Generalized lymphadenopathy with constitutional symptoms
  • Massive splenomegaly
  • GI symptoms with weight loss and anemia
  • Lymphocytosis with abnormal lymphocytes
  • B symptoms
  • Cytopenias
  • CNS symptoms in MCL patient
  • Family history of lymphoma with adenopathy
  • Recurrent infections in patient on therapy
  • Symptoms suggesting transformation

Treatment Methods

01
Excisional or core needle biopsy with immunohistochemistry (CD5, CD20, CD23-, cyclin D1, SOX11)
02
FISH for t(11;14) when histology is suggestive
03
TP53 mutation testing and FISH for 17p deletion
04
Ki-67 proliferation index quantification
05
Bone marrow biopsy and PET-CT for staging
06
GI evaluation (endoscopy and colonoscopy with biopsy)
07
Lumbar puncture in high-risk presentation (blastoid, high MIPI, CNS symptoms)
08
MIPI and MIPI-c risk stratification
09
Watchful waiting for asymptomatic non-nodal indolent variant (SOX11 negative, low MIPI)
10
Younger fit patients: Nordic regimen (alternating R-maxi-CHOP/R-cytarabine) plus ASCT and rituximab maintenance
11
Alternative R-DHAP or hyper-CVAD plus ASCT
12
Older or unfit patients: bendamustine plus rituximab (BR) or R-CHOP with maintenance rituximab
13
TRIANGLE trial supports addition of ibrutinib to induction
14
Relapsed disease: BTK inhibitor (ibrutinib, acalabrutinib, zanubrutinib)
15
Brexucabtagene autoleucel CAR T-cell therapy for refractory disease
16
Venetoclax plus ibrutinib (chemo-free) in TP53-mutated disease
17
Allogeneic transplantation in young patients with multiply relapsed disease
18
CNS prophylaxis in high-risk patients
19
Long-term surveillance for relapse and second malignancies
20
Antimicrobial prophylaxis (PJP, antiviral) during therapy

Which Department to Visit?

You can visit our Hematoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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