Mantle cell lymphoma

Mantle cell lymphoma 

§   Falls in between aggressive and indolent combining the worst features of both

§   Incurable but relatively aggressive – median survival 4-5 years


Sites of involvement

§   LNs, spleen, marrow, GI tract, Waldeyer’s ring, lungs, CNS, prostate, skin

§   Most cases of lymphomatous polyposis represent MCL


Clinical Features

§   Most (>90%) present with stage III / IV

       Median age 60-65 years, male predominance

Bulk disease at presentation, B symptoms less common, 30% lymphocytosis present

§   A lymphoblastoid form (blastoid variant) has been recognized. Other variants include classical (87%), small cell and pleomorphic variant.

       20% have an indolent form (survival 5-12 years)

§   IgM gammopathy in 25%



§        Lymph nodes

§        Diffuse infiltration but may show vaguely nodular mantle zone growth pattern

§        Hyalinized small vessels are common

§        Scattered epitheliod histiocytes = starry sky appearance

§        Small cleaved lymphocytes which may be confused for SLL/CLL



§   Positive: CD5, FMC7, CD43, Bcl 2, CD20, Surface IgM

§   Negative:CD10, Bcl6, CD23

(5-10% aberrant phenotype , CD5 –ve)

-      SOX11 highly expressed in classical variant



§   T(11;14) (q13;q32)= cyclin D1 (coded by CCND1 gene at 11q13)

-         secondary cytogenetic abnormalities common – poor prognosis

-         Rare cyclin D1 negative cases which express cyclin d2/d3


§   R + HD Cytarabine with Autograft in 1st remission for younger patients

§   R chemo (eg FCR, RCVP, R bendamustine, R chlorambucil) in older, less fit patients

-    IFRT in early stage disease

§   Maintenance R not recommended outside clinical trial

§   Assess response with conventional CT

§   Allografting has shown promise but most patients ineligible

§   RIC alloSCT should be considered in 2nd remission. 1st line is an option, consider trials where possible

§   Bortezomib – consider at relapse

Many thanks to Lewis Vanhinsbergh for updating this