The Course Of Monoclonal ‘villous’ Lymphocytosis Over 15 Years Of Follow-up: Progression To Slvl Or Spontaneous Clinical But Not Molecular Remission
Bassan, R., Spinelli, O., Rambaldi, A., & Barbui, T. (2003). The course of monoclonal 'villous' lymphocytosis over 15 years of follow-up: progression to SLVL or spontaneous clinical but not molecular remission. Leukemia, 17(11), 2243–2244. https://doi.org/10.1038/sj.leu.2403127
View Original Source →Case Details
Disease Location
Spleen
Personal Characteristics
Between 61-67 -year-old 66% chance female
Clinical Characteristics
Initial diagnosis of isolated monoclonal 'villous' lymphocytosis (mvl) as either preclinical or or a purely lymphocytosis variant of splenic lymphoma with villous lymphocytes (slvl) or splenic marginal zone lymphoma (smzl). Transition from mvl to aggressive form of slvl was seen in one patient after a progression-free interval of 7 years after 6 years of observation, slvl occured. There was increased splenomegaly (from the tip to 4cm below coastal margin) associated with osteoarticular pain and the appearance of a serum m band (IGM type, 0.7 g/dl), mild anemia (9.5 g/dl), and thrombocytopenia (89 x 10^9/l). Lymphocyte count showed a cyclical pattern (min 6.9 and max 24.1 x 10^9/l), with periods that were not well defined but ranged from 6-9 months. Lymphocyte cycles were less well characterized due to infrequent outpatient visits since 3rd to 4th year at the time of last exam, 70% of circulating leukocytes were villous cells and retained their original immunophenotype of clonal cd11c+, 25-, 23-, 38-, and 5- b cells
Remission Characteristics
Progression-free interval of 7 years at age 82 remains in relatively good clinical standing (15 years after initial mvl diagnosis) regression hypothesis is enforced by the results obtained in gastric b-cell lymphoma-associated helicobacter pylori infection where clinical but not molecular remissions are achieved (reference in article)
Treatment & Mechanisms
Proposed Remission Mechanisms
Activation/suppression of genes regulating the cell cycle, cytokine loops, and cytotoxic t/NK cells may variously affect the proliferative rate of mvl. A combination of strongly suppressive effects might occasionally induce a clonal regression like the one observed in one patient. The possible regression of slvl following treatment of hepatitis c virus point to the biological instability of these clones and to the critical role of extrinsic factors
Clinical Treatment
Low-dose oral chlorambucil for slvl
Non-Clinical Treatment
None reported