[7] In Western countries MCL accounts for around 7% of adult non-Hodgkin's lymphomas, with between 4 and 8 per cases per million diagnosed each year.

[10] This type of mantle cell lymphoma is associated with a more indolent, asymptomatic and slowly progressive course, however malignant transformation to aggressive forms is possible.

[11][7] Mantle cell lymphoma has been reported in rare cases to be associated with severe allergic reactions to mosquito bites.

These reactions involve extensive allergic reactions to mosquito bites which range from greatly enlarged bite sites that may be painful and involve necrosis to systemic symptoms (e.g. fever, swollen lymph nodes, abdominal pain, and diarrhea), or, in extremely rare cases, to life-threatening anaphylaxis.

Cells affected by MCL proliferate in a nodular or diffuse pattern with two main cytologic variants, typical or blastic.

Blastic (aka blastoid) variants have intermediate to large-sized cells with finely dispersed chromatin, and are more aggressive in nature.

Biopsy of the involved tissues (such as the lymph nodes, bone marrow, gastrointestinal tract, spleen or other areas) shows the characteristic histopathologic changes of MCL.

[7] In the nodular type, there are large nodules of MCL cells in the lymph node with no germinal centers observed.

[7] Histologically, the lymphoma cells in classic MCL are characterized as small to medium lymphocytes with scant cytoplasm and clumped chromatin with prominent nuclear clefts and the nucleoli are not visible.

[18] MRI of the brain and the spine are performed in cases of MCL with suspected central nervous system involvement.

A stronger chemotherapy with greater side effects (mostly hematologic) is HyperCVAD, often given in the hospital setting, with rituximab and generally to fitter patients (some of which are over 65).

A relatively new regimen that uses old medications is PEP-C, which includes relatively small, daily doses of prednisone, etoposide, procarbazine, and cyclophosphamide, taken orally, has proven effective for relapsed patients.

[25] Another approach involves using very high doses of chemotherapy, sometimes combined with total body irradiation (TBI), in an attempt to destroy all evidence of the disease.

A presentation at the December 2007 American Society of Hematology (ASH) conference by Christian Geisler, chairman of the Nordic Lymphoma Group[26] claimed that according to trial results, mantle cell lymphoma is potentially curable with very intensive chemo-immunotherapy followed by a stem cell transplant, when treated upon first presentation of the disease.

[27][28] These results seem to be confirmed by a large trial of the European Mantle Cell Lymphoma Network indicating that induction regimens containing monoclonal antibodies and high dose cytarabine followed by autologous stem cell transplantation should become the standard of care of MCL patients up to approximately 65 years of age.

[29][30] A study released in April 2013 showed that patients with previously untreated indolent lymphoma, bendamustine plus rituximab can be considered as a preferred first-line treatment approach to R-CHOP because of increased progression-free survival and fewer toxic effects.

[31] Immune-based therapy is dominated by the use of the rituximab monoclonal antibody, sold under the trade name Rituxan (or as Mabthera in Europe and Australia).

Rituximab may have good activity against MCL as a single agent, but it is typically given in combination with chemotherapies, which prolongs response duration.

[33][34][35] Two Bruton tyrosine kinase inhibitors (BTKi), one In November 2013, ibrutinib (brand name Imbruvica, Pharmacyclics LLC) and one in October 2017, acalabrutinib (brand name Calquence, AstraZeneca Pharmaceuticals LP) were approved in the United States for treating mantle cell lymphoma.

[5] In November 2019, zanubrutinib (Brukinsa) was approved in the United States with an indication for the treatment of adults with mantle cell lymphoma who have received at least one prior therapy.

[38] Brexucabtagene autoleucel (Tecartus) was approved for medical use in the United States in July 2020, with an indication for the treatment of adults with relapsed or refractory mantle cell lymphoma.

[42] Each dose of brexucabtagene autoleucel is a customized treatment created using the recipient's own immune system to help fight the lymphoma.

Bruton Tyrosine Kinase (BTK) inhibitors in the relapse setting have all demonstrated survival advantage in clinical trials (Wang et al., 2013; Eskelund et al., 2016; Rule et al., 2016).

Prognosis for individuals with MCL is problematic and indexes do not work well because most patients present at the advanced stage disease.

[46] The Mantle Cell Lymphoma International Prognostic Index (MIPI) was derived from a data set of 455 advanced stage MCL patients treated in series of clinical trials in Germany/Europe.

It has been suggested that in time, some non-blastic MCL transforms to blastic; however, this model has the assumption that increasing genetic alterations lead to the loss of cell cycle control, the higher proliferation rate, and thus to blastoid features.

But blastoid features are frequently seen at initial presentation in some patients, whereas other cases remain morphologically stable classical MCL throughout the duration of the disease.

[medical citation needed] Testing for high levels of lactate dehydrogenase in patients with non-Hodgkin's lymphoma is useful because it is released when body tissues break down for any reason.