Splenic marginal zone lymphoma pathophysiology
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [3]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [4]
Overview
Genes involved in the pathogenesis of splenic marginal zone lymphoma include immunoglobulin genes and CDK6 gene. On microscopic histopathological analysis, B-cells, villous lymphocytes, and sinus invasion are characteristic findings of splenic marginal zone lymphoma.
Pathophysiology
Pathogenesis
Genetics
Genes involved in the pathogenesis of splenic marginal zone lymphoma include:
- 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 |
Gross and Microscopic Pathology
- Splenic marginal zone lymphoma (SMZL) is a subtype of non-hodgkin's lymphoma comprised of B-cells.[3]
- It tends to involve spleen, bone marrow, liver and blood.[4]
- The microscopic features of these organs are described below:
- Spleen
- Nodular pattern of lymphocytic infiltration of the white pulp involving both mantle ad marginal zone component.[5]
- Biphasic distribution of B cell lymphocytes i.e small cells having dense chromatin and scant cytoplasm surrounded by intermediate size cells with interspersed blast cells.
- Red pulp may also be involved either sparingly or diffusely penetrating the sinuses.
- Bone Marrow:
- Various patterns of Lymphocytic infiltration such as intrasinusoidal, nodular, interstitial and intravascular have been observed.[6]
- Mixed pattern of lymphocytic infiltration with intrasinusoidal being predominant.[7][8]
- Cell population consists of small, intermediate and large lymphoid cells but small cells having large nucleus with dense chromatin and scant cytoplasm are present in abundance.[9]
- Lymph nodes
- Splenic hilar and peripheral lymph nodes may be involved but less frequently as compared to the other organs.
- Micronodular infiltration with no involvement of the sinuses.[10]
- Blood
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
- ↑ Zinzani PL (2012). "The many faces of marginal zone lymphoma". Hematology Am Soc Hematol Educ Program. 2012: 426–32. doi:10.1182/asheducation-2012.1.426. PMID 23233614.
- ↑ Arcaini L, Rossi D, Paulli M (April 2016). "Splenic marginal zone lymphoma: from genetics to management". Blood. 127 (17): 2072–81. doi:10.1182/blood-2015-11-624312. PMID 26989207.
- ↑ Papadaki T, Stamatopoulos K, Belessi C, Pouliou E, Parasi A, Douka V, Laoutaris N, Fassas A, Anagnostopoulos A, Anagnostou D (March 2007). "Splenic marginal-zone lymphoma: one or more entities? A histologic, immunohistochemical, and molecular study of 42 cases". Am. J. Surg. Pathol. 31 (3): 438–46. doi:10.1097/01.pas.0000213419.08009.b0. PMID 17325486.
- ↑ Mohanpuria A, Kumar V, Suteri P, Marwah S, Nigam AS (July 2017). "Important Diagnostic Clues for Diagnosing Splenic Marginal Zone Lymphoma in Absence of Splenic Histology". J Clin Diagn Res. 11 (7): ED15–ED17. doi:10.7860/JCDR/2017/27149.10190. PMC 5583942. PMID 28892912.
- ↑ Iannitto E, Ambrosetti A, Ammatuna E, Colosio M, Florena AM, Tripodo C, Minardi V, Calvaruso G, Mitra ME, Pizzolo G, Menestrina F, Franco V (November 2004). "Splenic marginal zone lymphoma with or without villous lymphocytes. Hematologic findings and outcomes in a series of 57 patients". Cancer. 101 (9): 2050–7. doi:10.1002/cncr.20596. PMID 15389479.
- ↑ Kent SA, Variakojis D, Peterson LC (May 2002). "Comparative study of marginal zone lymphoma involving bone marrow". Am. J. Clin. Pathol. 117 (5): 698–708. doi:10.1309/MECJ-GLK1-WEBW-UEVE. PMID 12090417.
- ↑ Ponzoni M, Kanellis G, Pouliou E, Baliakas P, Scarfò L, Ferreri AJ, Doglioni C, Bikos V, Dagklis A, Anagnostopoulos A, Ghia P, Stamatopoulos K, Papadaki T (November 2012). "Bone marrow histopathology in the diagnostic evaluation of splenic marginal-zone and splenic diffuse red pulp small B-cell lymphoma: a reliable substitute for spleen histopathology?". Am. J. Surg. Pathol. 36 (11): 1609–18. doi:10.1097/PAS.0b013e318271243d. PMID 23073320.
- ↑ Mollejo M, Lloret E, Menárguez J, Piris MA, Isaacson PG (July 1997). "Lymph node involvement by splenic marginal zone lymphoma: morphological and immunohistochemical features". Am. J. Surg. Pathol. 21 (7): 772–80. PMID 9236833.