[ Home Page ] [ Site Map ]

Mantle Cell Lymphoma

CLINICAL FEATURES

• Presents with advanced stage disease
• Bone marrow and peripheral blood involved in almost every case
• Extranodal involvement is frequent and may be the presenting feature; usually gastro-intestinal tract (multiple lymphomatous polyposis)
• Some patients present with lymphocytosis although most have nodal disease on further investigation.

LABORATORY DIAGNOSIS

A) Morphology

• Monomorphic population of small to intermediate sized cells with irregular nuclei
• May have a nodular or diffuse growth pattern. In some cases there may be fragments of residual normal germinal centre
• A blastic variant is described which consists of larger lymphoid cells with a higher rate of cell proliferation.

Intestinal MCL - CD20 immunoperoxidase staining of Peyer's patches.	Involvement of peripheral blood and bone marrow is a very frequent finding in mantle cell lymphoma

B) Immunophenotype

• moderate to strong sIgM and sIgD, CD19⁺, CD20⁺, CD5⁺, CD10^-, CD23^-, bcl-1⁺
• Only a few cases show evidence of somatic hypermutation indicating a pre-germinal centre origin⁴³

Three-colour flow cytometric analysis of a typical case of mantle cell lymphoma. There is strong co-expression of CD5 and CD19. CD23 is absent. The other main features are moderate to strong expression of sIg (IgD and IgM) with CD19 and CD20, and absence of CD10.

CYTOGENETICS

• The t(11;14) is found in almost all cases of mantle zone lymphoma using FISH based techniques
• The translocation results in aberrant expression of bcl-1, a key cell cycle regulatory protein. However, this has only a weak relationship to cell proliferation which is often relatively low
• The demonstration of t(11:14) or bcl-1 are essential for the diagnosis of MCL
• Other abnormalities found include 3q+ and 11q-

OUTCOME AND THERAPY

• The median overall survival for patients with mantle cell lymphoma is around 40-50 months with progression-free survival of 20 months
• Complete remission is uncommon. 30-60% achieve a partial response.
• There are no clear data on optimal treatment or indeed evidence that treatment is effective in most cases
• Suggested benefits for high dose therapy have not been proven
• The large cell variant is associated with short survival.

[ Home Page ] [ Site Map ]