Mantle Cell Lymphoma

At a Glance

Mantle cell lymphoma is a small B-cell lymphoma that is clinically more aggressive than other small B-cell non-Hodgkin lymphomas. It commonly presents at a higher stage, including bone marrow and peripheral blood involvement. It is a disease of adults and is more common in men.

What Tests Should I Request to Confirm My Clinical Dx? In addition, what follow-up tests might be useful?

Mantle cell lymphoma is usually diagnosed by biopsy of lymph node or bone marrow. Other commonly affected sites are the gastrointestinal (GI) tract (multiple lymphomatous polyposis) and Waldeyer’s ring. Many patients also have circulating lymphoma cells in their peripheral blood. Mantle cell lymphoma is classically a diffuse infiltrate of monotonous small hyperchromatic lymphocytes. Immunohistochemical staining showing CD20+ small lymphocytes positive for CD5 and cyclin D1 (also called BCL-1) is diagnostic of mantle cell lymphoma.

Flow cytometry is very helpful in identifying the monoclonal B-cell population. The characteristic immunophenotype of the monoclonal B-cells of mantle cell lymphoma is CD45+, CD19+, CD20+, CD5+, CD10- CD23-, and FMC7+. Cytogenetic karyotype and fluorescence in situ hybridization (FISH) studies can be very helpful. The presence of t(11;14) (q13;q32) between IgH and CCND1 is diagnostic of this lymphoma and is present in nearly every case. Molecular polymerase chain reaction (PCR) studies to look for IgH gene rearrangement are usually not performed but would show a clonal B-cell population.

Are There Any Factors That Might Affect the Lab Results? In particular, does your patient take any medications – OTC drugs or Herbals – that might affect the lab results?

Mantle cell lymphoma can sometimes have histologic and immunophenotypic overlap with chronic lymphocytic leukemia/small lymphocytic lymphoma or other small B-cell lymphomas. In any case that is not straightforward, staining for cycling D1 and cytogenetic FISH for t(11; 14) can be performed to look for the characteristic positive results in mantle cell lymphoma. Mantle cell lymphoma can have some morphologic variants that may make diagnosis challenging, such as blastoid, pleomorphic, small cell, or marginal zone-like variants.

What Tests Should I Request to Confirm My Clinical Dx? In addition, what follow-up tests might be useful?

Patients are usually referred to an oncologist after their initial diagnosis for full clinical staging, including lab work, radiology imaging, and a bone marrow biopsy. Because of the more aggressive nature of this lymphoma, it is often treated more intensely than other lymphomas.