Background

MCL is an uncommon subtype of B-cell non-Hodgkin lymphomas defined by the overexpression of the cyclin D1 (CCND1) gene due to a (1,14) translocation.

Depending on the number of morphologic variables, this condition may be difficult to diagnose, while some cases are straightforward.

It normally follows an aggressive clinical course, whereas the description is of an indolent form of leukemia.

Epidemiology

Mantle Cell Lymphoma has a low frequency of one case per 200,000 individuals annually.

MCL accounts for around 5% of non-Hodgkin lymphomas, and it is three times more prevalent in men than in women.

Anatomy

Pathophysiology

MCL is distinguished by the chromosomal translocation t(11;14) (q13:q32), which results in the juxtaposition of the cyclin D1 region and the immunoglobulin heavy chain gene locus.

This results in constitutive production of cyclin D1 (CCND1), which plays a crucial role in tumor cell proliferation via disruption of the cell cycle, epigenetic regulation, and chromosomal instability.

In rare cases where this translocation is absent, CCND2 or 3 translocations may be present. Based on the cell of origin that connects to clinical symptoms, hypothesized models of molecular subtypes have been presented.

Classical MCL or aggressive MCL arises from naive B cells that have none or minimal iGVH mutations and express SOX 11. SOX 11 is a neuronal transcription factor that reportedly inhibits the final development of B cells.

The indolent type of MCL originates from antigen-exposed B cells that have undergone IGVH somatic hypermutations. They are often SOX 11-negative and genetically stable B cells.

Etiology

MCL is not a very common condition, but some families might be predisposed to a high incidence of this disease.

Genetics

Prognostic Factors

Clinical History

Physical Examination

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Medication

Future Trends

Media Gallary

References

https://www.ncbi.nlm.nih.gov/books/NBK536985/