Splenic lymphoma with villous lymphocytes (SLVL)
نویسندگان
چکیده
Phenotype/cell stem origin Light chain restriction surface immunoglobulin. Most cases express IgM and IgD. B-cells express CD19+, CD20+, CD22+, CD24+, CD79b+, FMC7+ and DBA44+. Lack expression of CD5 (85%), CD10, CD23, CD103 and CD123. Epidemiology In 1987, the term SLVL was introduced; 1-2% of nonHodgkin lymphomas; occurs in the elderly (med 70 yrs); sex ratio 2M/1F. Clinics Splenomegaly without hepatomegaly nor enlarged lymph nodes; monoclonal Ig in a third of cases, autoimmune phenomena in 10% of patients, transformation to high grade lymphoma in 10% of cases. Pathology Spleen. Nodular replacement of the white pulp with a central core of small lymphocytes and larger cells in the peripheral marginal zone. Invasion of the splenic red pulp is inconstant. Bone marrow morphology showing intrasinusoidal lymphoma cells. Treatment Only in symptomatic patients: splenectomy or chemotherapy with purine analogues. Antiviral therapy (IFN) in patients with SLVL and hepatitis C.
منابع مشابه
Severe T-mediated bone marrow aplasia in a patient with splenic lymphoma with villous lymphocytes (SLVL) previously treated with fludarabine regimen.
Splenic Lymphoma with villous lymphocytes (SLVL) is a B cell chronic lymphoproliferative disorder defined in the WHO classification as the leukemic form of splenic marginal zone lymphoma. Autoimmune diseases or second neoplasms in patients affected by B cell chronic lymphoproliferative disorders are well known conditions after fludarabine containing regimens (above all in the case of Chronic Ly...
متن کامل[Splenic lymphoma with circulating villous lymphocytes].
Splenic lymphoma with circulating villous lymphocytes (SLVL) is a special kind of lymphoproliferative disease characterized by specific clinical, haematological, histomorphological and immunophenotypic features that make it different from hairy cell leukaemia, prolymphocytic leukaemia and non-Hodgkin lymphoma. Four patients suffering from SLVL, 3 men and 1 woman, 62 years in average (range 55-6...
متن کاملDeletion mapping on the long arm of chromosome 7 in splenic lymphoma with villous lymphocytes.
Splenic lymphoma with villous lymphocytes (SLVL) is a low-grade lymphoproliferative disorder characterized by splenomegaly and circulating villous lymphocytes in the peripheral blood. It is considered to be the leukemic form of splenic marginal zone lymphoma (SMZL). The genetic basis of this lymphoma type remains unknown. Conventional cytogenetic studies have identified frequent structural abno...
متن کاملThe immunophenotype of splenic lymphoma with villous lymphocytes and its relevance to the differential diagnosis with other B-cell disorders.
Splenic lymphoma with villous lymphocytes (SLVL) is a low-grade disorder that regularly presents with peripheral blood involvement. We describe the immunophenotype of the circulating cells from 100 SLVL patients whose disease has been characterized on clinical, morphologic, and histologic grounds. Cells from all cases expressed B-cell antigens (CD19 and CD37) and/or HLA-Dr and showed light chai...
متن کاملSplenic Lymphoma with an Incidental Hemolytic Anemia: A Rare Association
Splenic lymphoma with villous lymphocytes (SLVL) is a rare malignancy that comprises less than 1% of lymphoid neoplasms, characterized by the presence of small atypical lymphocytes in the peripheral blood and bone marrow and splenic infiltration in the white and red pulp. It must be distinguished from other chronic lymphoproliferative disorders that present with a similar clinical and hematolog...
متن کامل