Splenic marginal zone lymphoma pathophysiology: Difference between revisions
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'''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]] [[follicle]]s and erode the [[marginal zone]], ultimately invading the red pulp of the spleen. Frequently, the bone marrow and [[splenic]] [[hilar]] [[lymph nodes]] are involved along with the peripheral [[blood]]. The neoplastic cells circulating in the peripheral blood are termed villious lymphocytes due to their characteristic appearance.<ref name="who1">[http://www.iarc.fr/WHO-BlueBooks/BBwebsite/bb3.html] 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 </ref> | '''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]] [[follicle]]s and erode the [[marginal zone]], ultimately invading the red pulp of the spleen. Frequently, the bone marrow and [[splenic]] [[hilar]] [[lymph nodes]] are involved along with the peripheral [[blood]]. The neoplastic cells circulating in the peripheral blood are termed villious lymphocytes due to their characteristic appearance.<ref name="who1">[http://www.iarc.fr/WHO-BlueBooks/BBwebsite/bb3.html] 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 </ref> | ||
With splenic involvement a ''sine qua non'' for | With splenic involvement a ''sine qua non'' for splenic marginal zone lymphoma, [[splenomegaly]] is seen in almost all patients, commonly without lymphadenopathy.<ref name="who1">[http://www.iarc.fr/WHO-BlueBooks/BBwebsite/bb3.html] 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 </ref> Reactive germinal centers in [[splenic]] [[white pulp]] are replaced by small neoplastic [[lymphocytes]] that efface the mantle zone and ultimately blend in with the [[marginal zone]] with occasional larger neoplastic cells that resemble blasts.<ref name="mol1"> [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=7573673&query_hl=9&itool=pubmed_ExternalLink] | ||
Mollejo M, Menarguez J, Lloret E, Sanchez A, Campo E, Algara P, Cristobal E, Sanchez E, Piris MA. | Mollejo M, Menarguez J, Lloret E, Sanchez A, Campo E, Algara P, Cristobal E, Sanchez E, Piris MA. | ||
"Splenic marginal zone lymphoma: a distinctive type of low-grade B-cell lymphoma. A clinicopathological study of 13 cases." '''Am J Surg Pathol'''. 1995 Oct;19(10):1146-57. | "Splenic marginal zone lymphoma: a distinctive type of low-grade B-cell lymphoma. A clinicopathological study of 13 cases." '''Am J Surg Pathol'''. 1995 Oct;19(10):1146-57. | ||
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==Sites of Involvement== | ==Sites of Involvement== | ||
Aside from the uniform involvement of the [[spleen]], the [[bone marrow]] is frequently positive in patients with | 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.<ref name="who1"/> | ||
===Splenic Hilar Lymph Nodes=== | ===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.<ref name="who1"/> | Involved hilar [[lymph nodes]] adjacent to the [[spleen]] show an effaced architecture without preservation of the [[marginal zone]] seen in the spleen.<ref name="who1"/> | ||
===Bone Marrow Biopsy=== | ===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]].<ref name="fra1"> [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=8971565&query_hl=13&itool=pubmed_ExternalLink] | |||
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</ref> | 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</ref> | ||
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The relevant markers that define the immunophenotype for | The relevant markers that define the immunophenotype for splenic marginal zone lymphoma are shown in the table to the right.<ref name="isa1"> | ||
[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=7949139&query_hl=17&itool=pubmed_ExternalLink] | [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=7949139&query_hl=17&itool=pubmed_ExternalLink] | ||
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</ref><ref name="mat1"> | 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</ref><ref name="mat1"> | ||
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [11]
Overview
Pathophysiology
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. Frequently, the bone marrow and splenic hilar lymph nodes are involved along with the peripheral blood. The neoplastic cells circulating in the peripheral blood are termed villious lymphocytes due to their characteristic appearance.[1]
With splenic involvement a sine qua non for splenic marginal zone lymphoma, splenomegaly is seen in almost all patients, commonly without lymphadenopathy.[1] Reactive germinal centers in splenic white pulp are replaced by small neoplastic lymphocytes that efface the mantle zone and ultimately blend in with the marginal zone with occasional larger neoplastic cells that resemble blasts.[2][3] The red pulp is always involved, with both nodules of larger neoplastic cells and sheets of the small neoplastic lymphocytes. Other features that may been seen include sinus invasion, epithelial histocytes, and plasmacytic differentiation of neoplastic cells.
The cell of origin is postulated to be a post-germinal center B-cell with an unknown degree of differentiation.[1]
Sites of Involvement
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.[1]
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.[1]
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]
Peripheral Blood
Circulating lymphoma cells are sometimes present in peripheral blood, and they occasionally show short villi at the poles of cells and plasmacytoid differentiation.[5]
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 splenic marginal zone lymphoma are shown in the table to the right.[6][7] 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.[8]
Genetics
Clonal rearrangements of the immunoglobulin genes (heavy and light chains) are frequently seen.[9] The deletion 7q21-32 is seen in 40% of SMZL patients, and translocations of the CDK6 gene located at 7q21 have also been reported.[10]
References
- ↑ 1.0 1.1 1.2 1.3 1.4 [1] 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
- ↑ [2] Mollejo M, Menarguez J, Lloret E, Sanchez A, Campo E, Algara P, Cristobal E, Sanchez E, Piris MA. "Splenic marginal zone lymphoma: a distinctive type of low-grade B-cell lymphoma. A clinicopathological study of 13 cases." Am J Surg Pathol. 1995 Oct;19(10):1146-57. PMID: 7573673
- ↑ [3] Jaffe ES, Costa J, Fauci AS, Cossman J, Tsokos M. "Malignant lymphoma and erythrophagocytosis simulating malignant histiocytosis." Am J Med. 1983 Nov;75(5):741-9. PMID: 6638043
- ↑ [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
- ↑ [5] Melo JV, Hegde U, Parreira A, Thompson I, Lampert IA, Catovsky D. "Splenic B cell lymphoma with circulating villous lymphocytes: differential diagnosis of B cell leukaemias with large spleens." J Clin Pathol. 1987 Jun;40(6):642-51. PMID: 3497180
- ↑ [6] 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
- ↑ [7] 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
- ↑ [8] 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
- ↑ [9] 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
- ↑ [10] 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