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Clinical manifestations, pathologic features, and diagnosis of mantle cell lymphoma

Arnold S Freedman, MD
Jon C Aster, MD
Section Editor
Andrew Lister, MD, FRCP, FRCPath, FRCR
Deputy Editor
Alan G Rosmarin, MD


Mantle cell lymphoma (MCL) is one of the mature B cell non-Hodgkin lymphomas (NHL) [1-5]. (See "Classification of the hematopoietic neoplasms".) While it is often discussed together with the clinically indolent forms of NHL, its behavior is more often that of an aggressive disease.

MCL has been previously referred to as intermediate lymphocytic lymphoma, mantle zone lymphoma, centrocytic lymphoma, and lymphocytic lymphoma of intermediate differentiation [6,7]. In the Rappaport and Working Formulation classifications, the majority of cases of diffuse small cleaved cell lymphomas on re-review were found to be MCLs; however, cases were also found in the small lymphocytic and follicular categories [2]. These misclassifications stemmed from cases with ambiguous morphologies, emphasizing the importance of immunophenotyping in the diagnosis of MCL. (See 'Immunophenotype' below.)

The epidemiology, clinical presentation, pathology, diagnosis, and prognosis of MCL will be discussed here. The pathobiology and treatment of this NHL variant are presented separately. (See "Initial treatment of mantle cell lymphoma" and "Pathobiology of mantle cell lymphoma".)


Mantle cell lymphoma comprises about 7 percent of adult non-Hodgkin lymphomas in the United States and Europe with an incidence of approximately 4 to 8 cases per million persons per year [3,8-12]. Incidence increases with age and appears to be increasing overall in the United States [13]. Approximately three-quarters of patients are male and Caucasians are affected almost twice as frequently as Blacks. Median age at diagnosis is 68 years.


Mantle cell lymphoma (MCL) is thought to have two distinct cellular origins, each giving rise to different forms of the disease [14]:


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