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== Pathophysiology ==
== 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]] [[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>
==Molecular Findings==
===Immunophenotype===
{| class="wikitable" style="width: 20em; text-align: left; font-size: 100%;"
|-
! '''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.<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]
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">
[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=8123845&query_hl=20&itool=pubmed_ExternalLink]
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</ref> 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.<ref name="sav1">
[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=9688179&query_hl=22&itool=pubmed_ExternalLink]
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</ref>
===Genetics===
Clonal rearrangements of the [[immunoglobulin]] genes (heavy and light chains) are frequently seen.<ref name="dun1">[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=9635678&query_hl=24&itool=pubmed_ExternalLink]
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</ref> The deletion 7q21-32 is seen in 40% of SMZL patients, and translocations of the CDK6 gene  located at 7q21 have also been reported.<ref name="cor1">
[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=10597225&query_hl=28&itool=pubmed_ExternalLink]
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</ref>
==References==
==References==
{{reflist|2}}
{{reflist|2}}

Revision as of 15:46, 18 September 2012

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [7]

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]

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.[2][3] 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.[4]

Genetics

Clonal rearrangements of the immunoglobulin genes (heavy and light chains) are frequently seen.[5] The deletion 7q21-32 is seen in 40% of SMZL patients, and translocations of the CDK6 gene located at 7q21 have also been reported.[6]

References

  1. ↑ [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. ↑ [2] 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
  3. ↑ [3] 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
  4. ↑ [4] 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
  5. ↑ [5] 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
  6. ↑ [6] 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

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