Splenic Marginal Zone Lymphoma

Splenic Marginal Zone Lymphoma


Clinical features

§        Splenomegaly

§        Peripheral blood villous lymphocytes

§        BM normally involved, but peripheral lymphadenopathy not common



§        Spleen

§        Small lymphocytes surround and replace germinal centres with effacement of the normal follicle mantle

§        Red pulp is always infiltrated

§        Bone marrow

§        Nodal

§        Peripheral blood

§        Short polar villi

§        May resemble LPLs



§        Positive for SIg

§        Absence of CD5 and CD43 helps exclude CLL and MCL

§        Absence of CD103 helps exclude HCL

§        Absence of CD10 helps exclude follicular



§        Clinical course is indolent

§        Splenectomy is treatment of choice

Mantle cell lymphoma


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

§   Incurable but relatively aggressive – median survival 3 years


Sites of involvement

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

§   Most cases of lymphomatous polyposis represent MCL


Clinical Features

§   Most present with stage III / IV

§   A lymphoblastoid form has been recognised

§   IgM gammopathy in 25%



§        Lymph nodes

§        Diffuse infiltration but may show vaguely nodular of 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, B cell antigens, CD20, Surface IgM

§   Negative:CD10, Bcl6, CD23



§   T(11;14) = cyclin D1 (nearly all)



§   Fludarabine

§   Fludarabine in combination

§   R CHOP  but most patients recur within 24 months

§   Some benefit from splenectomy

§   Autologous transplant not thought to be curative

§   Allografting has shown promise but most patients ineligible

§   Cyclin D1 inhibitors – flavopiridol

§   Bortezomib