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%
Morphology
§ 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
Immunophenotype
§ 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
Cytogenetics
§ 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
Treatment
§ 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
