Splenic Marginal Zone Lymphoma
Clinical features § Splenomegaly § Peripheral blood villous lymphocytes § BM normally involved, but peripheral lymphadenopathy not common
Morphology § 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
Immunophenotype § 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
Treatment § Clinical course is indolent § Splenectomy is treatment of choice
§ 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%
Morphology § 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
Immunophenotype § Positive: CD5, FMC7, CD43, B cell antigens, CD20, Surface IgM § Negative:CD10, Bcl6, CD23
Cytogenetics § T(11;14) = cyclin D1 (nearly all)
Treatment § 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 |
Haemato-oncology > Mature B Cell neoplasms >