Splenic marginal zone lymphoma
| Splenic marginal zone lymphoma | |
| ICD-O: | 9689/3 |
|---|---|
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [6]
Molecular Findings
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 table to the right.[1][2] The lack of CD5 expression is helpful in the discrimination between SMZL and chronic lymphocytic leukemia/small lymphocytic lymphoma, and the lack of CD10 expression argues against follicular lymphoma. Mantle cell lymphoma is excluded due to the lack of CD5 and cyclin-D1 expression.[3]
Genetics
Clonal rearrangements of the immunoglobulin genes (heavy and light chains) are frequently seen.[4] The deletion 7q21-32 is seen in 40% of SMZL patients, and translocations of the CDK6 gene located at 7q21 have also been reported.[5]
References
- ↑ [1] Isaacson PG, Matutes E, Burke M, Catovsky D. "The histopathology of splenic lymphoma with villous lymphocytes." Blood. 1994 Dec 1;84(11):3828-34. PMID: 7949139
- ↑ [2] Matutes E, Morilla R, Owusu-Ankomah K, Houlihan A, Catovsky D. "The immunophenotype of splenic lymphoma with villous lymphocytes and its relevance to the differential diagnosis with other B-cell disorders." Blood. 1994 Mar 15;83(6):1558-62. PMID: 8123845
- ↑ [3] Savilo E, Campo E, Mollejo M, Pinyol M, Piris MA, Zukerberg LR, Yang WI, Koelliker DD, Nguyen PL, Harris NL. "Absence of cyclin D1 protein expression in splenic marginal zone lymphoma." Mod Pathol. 1998 Jul;11(7):601-6. PMID: 9688179
- ↑ [4] Dunn-Walters DK, Boursier L, Spencer J, Isaacson PG. "Analysis of immunoglobulin genes in splenic marginal zone lymphoma suggests ongoing mutation." Hum Pathol. 1998 Jun;29(6):585-93. PMID: 9635678
- ↑ [5] Corcoran MM, Mould SJ, Orchard JA, Ibbotson RE, Chapman RM, Boright AP, Platt C, Tsui LC, Scherer SW, Oscier DG. "Dysregulation of cyclin dependent kinase 6 expression in splenic marginal zone lymphoma through chromosome 7q translocations." Oncogene. 1999 Nov 4;18(46):6271-7. PMID: 10597225