Mantle cell lymphoma (MCL) is a mature B-cell lymphoma that can present with lymphadenopathy, peripheral blood involvement, and/or extranodal involvement. MCL can involve any portion of the gastrointestinal system and may present as lymphomatous intestinal polyposis. It is often a very aggressive disease.
It is characterized by a CCND1 translocation, t(11;14)(q13;q32), in more than 95% of cases.
MCL makes up 3%-10% of non-Hodgkin lymphomas and occurs in older individuals (median age of 60-70 years) with a male predominance. Most patients present with advanced (stage III or IV) disease. Symptoms may include fatigue, fever, night sweats, unintentional weight loss, lymphadenopathy, hepatosplenomegaly, and/or abdominal discomfort.
Mantle cell lymphoma
Alerts and Notices
Synopsis
Codes
ICD10CM:
C83.10 – Mantle cell lymphoma, unspecified site
SNOMEDCT:
443487006 – Mantle cell lymphoma
C83.10 – Mantle cell lymphoma, unspecified site
SNOMEDCT:
443487006 – Mantle cell lymphoma
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Other B-cell lymphomas – nodal marginal zone lymphoma, extranodal marginal zone lymphoma, splenic marginal zone lymphoma, chronic lymphocytic leukemia, follicular lymphoma, lymphoplasmacytic lymphoma
- Splenomegaly can occur with other hematologic disorders including leukemia and other myeloproliferative neoplasms, as well as nonmalignant causes (autoimmune or infection).
- Infectious mononucleosis (Epstein-Barr virus [EBV] infection)
- Other infectious lymphadenitis (eg, cat-scratch fever)
- Other systemic or indolent infections that present with B symptoms (eg, endocarditis, osteomyelitis, tuberculosis)
- Other cancers (carcinoma, melanoma, sarcoma)
Best Tests
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Management Pearls
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Therapy
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Drug Reaction Data
Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.
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References
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Last Reviewed:08/07/2018
Last Updated:07/20/2022
Last Updated:07/20/2022