The course of monoclonal ‘villous’ lymphocytosis over 15 years of follow-up: progression to SLVL or spontaneous clinical but not molecular remission

Case Details

Disease Location

Spleen

Personal Characteristics

Between 61-67 -year-old 66% chance female

Clinical Characteristics

Mvl diagnosis initial cyclical lymphocyte fluctuations were initially observed (min 4.8 and max 13.6 x 10^9/l) at 15 year follow-up pcr analysis demonstrated the persistence of a circulating residual b-cell clone with the same igh gene rearrangement as that detected at diagnosis, serum was screened for hepatitis c mrna and was negative.

Remission Characteristics

Progressive decrease in the lymphocyte count was noted starting from the 4th year of follow-up onward lymphocyte decrease was slow, taking about 4 years to reach <4 x 10^9/l. Starting at the sixth year of follow-up, villous cells were no longer detectable in the peripheral blood smear. Lymphocyte count was constantly <4 x 10^9/l, and <2 x 10^9/l from the 11th year. After 15 years of follow-up there is no abnormal physical finding related to mvl or slvl and no morphological or immunophenotypic abnormality to supporight the diagnosis of a b-cell lymphoproliferative disease.

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

None reported

Non-Clinical Treatment

None reported