Splenic marginal zone lymphoma
Splenic marginal zone lymphoma | |
---|---|
Specialty | Hematology, oncology |
Splenic marginal zone lymphoma (SMZL) is a type of
Cause
The cell of origin is postulated to be a post-germinal center B-cell with an unknown degree of differentiation.[1] SMZL is a form of cancer known to be associated with Hepatitis C virus infection.[citation needed]
Molecular biology
Immunophenotype
Antigen | Status |
---|---|
CD20 | Positive |
CD79a
|
Positive |
CD5 | Negative |
CD10
|
Negative |
CD23 | Negative |
CD43 | Negative |
cyclin D1 | Negative |
The relevant markers that define the immunophenotype for SMZL are shown in the adjacent table.[2] [3] The lack of
Genetics
Clonal rearrangements of the
Diagnosis
Circulating lymphoma cells are sometimes present in peripheral blood, and they occasionally show short villi at the poles of cells and plasmacytoid differentiation.[1][8]
Prognosis
Three-quarters of patients survive five or more years; more than half of patients with SMZL survive more than a decade after diagnosis.[12]
Patients who have a hemoglobin level of less than 12 g/dL, a lactate dehydrogenase level higher than normal, and/or a blood serum albumin levels of less than 3.5 g/dL are likely to have more an aggressive disease course and a shorter survival.[12] However, even high-risk patients have even odds of living for five years after diagnosis.[12]
Some genetic mutations, such as mutations in NOTCH2, are also correlated with shorter survival.[citation needed]
Epidemiology
Less than 1% of all lymphomas are splenic marginal zone lymphomas[13] and it is postulated that SMZL may represent a large fraction of unclassifiable CD5- chronic lymphocytic leukemias.[1] The typical patient is over the age of 50, and gender preference has been described.[9]
Synonyms
Under older classification systems, the following names were used:[1]
Classification system | Name |
---|---|
Rappaport | well-differentiated lymphocytic lymphoma |
Lukes-Collins | small lymphocytic lymphoma |
Working Formulation | small lymphocytic lymphoma |
FAB | splenic lymphoma with circulating villous lymphocytes |
See also
References
- ^ ISBN 978-92-832-2411-2.)
{{cite book}}
: CS1 maint: multiple names: authors list (link - ^
Isaacson PG, Matutes E, Burke M, Catovsky D (1 December 1994). "The histopathology of splenic lymphoma with villous lymphocytes". Blood. 84 (11): 3828–34. PMID 7949139.
- PMID 8123845.
- ^
Savilo E, Campo E, Mollejo M, et al. (July 1998). "Absence of cyclin D1 protein expression in splenic marginal zone lymphoma". Mod. Pathol. 11 (7): 601–6. PMID 9688179.
- PMID 9635678.
- ^
Corcoran MM, Mould SJ, Orchard JA, et al. (November 1999). "Dysregulation of cyclin dependent kinase 6 expression in splenic marginal zone lymphoma through chromosome 7q translocations". Oncogene. 18 (46): 6271–7. PMID 10597225.
- S2CID 37364398.
- PMID 3497180.
- ^ PMID 10706860.
- ^ PMID 7573673.
- PMID 6638043.
- ^ S2CID 13024127.
- PMID 9704731. Archived from the originalon 2013-04-15.