Splenic marginal zone lymphoma pathophysiology
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [5]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [6]
Overview
Genetics
- Clonal rearrangements of the immunoglobulin genes (heavy and light chains) [1]
- Deletion 7q21-32 is seen in 40% of SMZL patients
- Translocations of the CDK6 gene located at 7q21 have also been reported[2]
Immunophenotype
| Antigen | Splenic marginal zone lymphoma |
|---|---|
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Positive | |
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Positive | |
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Negative | |
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Negative | |
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Negative | |
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Negative | |
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Negative |
Microscopic Pathology
- Splenic marginal zone lymphoma (SMZL) is a lymphoma comprised of B-cells that replace the normal architecture of the white pulp of the spleen.
- The neoplastic cells are both small lymphocytes and larger, transformed blasts, and they invade the mantle zone of splenic follicles and erode the marginal zone, ultimately invading the red pulp of the spleen.
- The neoplastic cells circulating in the peripheral blood are termed villious lymphocytes due to their characteristic appearance.[3]
- Other features that may been seen include sinus invasion, epithelial histocytes, and plasmacytic differentiation of neoplastic cells.
- Aside from the uniform involvement of the spleen, the bone marrow is frequently positive in patients with splenic marginal zone lymphoma. Nodal and extranodal involvement are rare.[3]
- Splenic Hilar Lymph Nodes
- Involved hilar lymph nodes adjacent to the spleen show an effaced architecture without preservation of the marginal zone seen in the spleen.[3]
- Bone Marrow Biopsy
- Splenic marginal zone lymphoma in bone marrow displays a nodular pattern with morphology similar to what is observed in the splenic hilar lymph nodes.[4]
References
- ↑ [1] 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
- ↑ [2] 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
- ↑ 3.0 3.1 3.2 [3] Jaffe E.S., Harris N.L., Stein H., Vardiman J.W. (eds): World Health Organization Classification of Tumors. Pathology and Genetics of Tumours of Haemopoietic and Lymphoid Tissues. IARC Press: Lyon 2001
- ↑ [4] Franco V, Florena AM, Campesi G. "Intrasinusoidal bone marrow infiltration: a possible hallmark of splenic lymphoma." Histopathology. 1996 Dec;29(6):571-5. PMID: 8971565