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| {{Infobox_Disease |
| | '''For patient information, click [[Non-Hodgkin lymphoma (patient information)|here]]''' |
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| ICDO = 9689/3 |
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| '''For patient information, click [[Splenic marginal zone lymphoma (patient information)|here]]''' | |
| {{Splenic marginal zone lymphoma}} | | {{Splenic marginal zone lymphoma}} |
| {{CMG}} | | {{CMG}}; {{AE}} {{Affan}}, {{AS}} |
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| | {{SK}} SMZL, SLVL, Splenic marginal zone B-cell lymphoma, Splenic B-cell marginal zone lymphoma Splenic lymphoma with circulating villous lymphocytes, splenic lymphoma with villous lymphocytes, |
| | ==[[Splenic marginal zone lymphoma overview|Overview]]== |
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| | ==[[Splenic marginal zone lymphoma historical perspective|Historical Perspective]]== |
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| ===ICD-O Code=== | | ==[[Splenic marginal zone lymphoma classification|Classification]]== |
| 9689/3<ref name="who1"/>
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| ===Definition=== | | ==[[Splenic marginal zone lymphoma pathophysiology|Pathophysiology]]== |
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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 causes|Causes]]== |
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| ==Epidemiology== | | ==[[Splenic marginal zone lymphoma differential diagnosis|Differentiating Splenic marginal zone lymphoma from other Diseases]]== |
| Less than 1% of all lymphomas are splenic marginal zone lymphomas<ref name="arm1"> [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=9704731&query_hl=5&itool=pubmed_ExternalLink]
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| Armitage JO, Weisenburger DD. "New approach to classifying non-Hodgkin's lymphomas: clinical features of the major histologic subtypes. Non-Hodgkin's Lymphoma Classification Project." '''J Clin Oncol.''' 1998 Aug;16(8):2780-95. PMID: 9704731</ref> and it is postulated that SMZL may represent a large fraction of unclasssifiable CD5- chronic lymphocytic leukemias.<ref name="who1"/> The typical patient is over the age of 50, and gender preference has been described.<ref name="ber1">
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| [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=10706860&query_hl=7&itool=pubmed_ExternalLink]
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| Berger F, Felman P, Thieblemont C, Pradier T, Baseggio L, Bryon PA, Salles G, Callet-Bauchu E, Coiffier B. "Non-MALT marginal zone B-cell lymphomas: a description of clinical presentation and outcome in 124 patients." '''Blood'''. 2000 Mar 15;95(6):1950-6. PMID: 10706860</ref>
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| ==Clinical Features== | | ==[[Splenic marginal zone lymphoma epidemiology and demographics|Epidemiology and Demographics]]== |
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| ===Etiology=== | | ==[[Splenic marginal zone lymphoma risk factors|Risk Factors]]== |
| The cell of origin is postulated to be a post-germinal center B-cell with an unknown degree of differentiation.<ref name="who1"/>
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| ===Clinical Presentation=== | | ==[[Splenic marginal zone lymphoma screening|Screening]]== |
| With splenic involvement a ''sine qua non'' for SMZL, [[splenomegaly]] is seen in almost all patients, commonly without lymphadenopathy.<ref name="who1"/>
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| ===Laboratory Findings=== | | ==[[Splenic marginal zone lymphoma natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
| Autoimmune thrombocytopenia and [[anemia]] sometimes seen in patients with SMZL. Circulating villous lymphocytes are sometimes observed in peripheral [[blood]] samples.<ref name="who1"/> A [[monoclonal]] [[paraprotein]] is detected in a third of patients without [[hypergammaglobulinemia]] or hyperviscosity.<ref name="ber1"/><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]
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| Mollejo M, Menarguez J, Lloret E, Sanchez A, Campo E, Algara P, Cristobal E, Sanchez E, Piris MA.
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| "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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| PMID: 7573673</ref>
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| ===Sites of Involvement=== | | ==Diagnosis== |
| Aside from the uniform involvement of the [[spleen]], the [[bone marrow]] is frequently positive in patients with SMZL. Nodal and extranodal involvement are rare.<ref name="who1"/>
| | [[Splenic marginal zone lymphoma diagnostic study of choice|Diagnostic study of choice]] | [[Splenic marginal zone lymphoma staging|Staging]] | [[Splenic marginal zone lymphoma history and symptoms|History and Symptoms]] | [[Splenic marginal zone lymphoma physical examination|Physical Examination]] | [[Splenic marginal zone lymphoma laboratory findings|Laboratory Findings]] | [[Splenic marginal zone lymphoma biopsy|Biopsy]] | [[Splenic marginal zone lymphoma CT|CT]] | [[Splenic marginal zone lymphoma MRI|MRI]] | [[Splenic marginal zone lymphoma ultrasound|Ultrasound]] | [[Splenic marginal zone lymphoma other imaging findings|Other Imaging Findings]] | [[Splenic marginal zone lymphoma other diagnostic studies|Other Diagnostic Studies]] |
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| ==Morphology== | | ==Treatment== |
| | [[Splenic marginal zone lymphoma medical therapy|Medical Therapy]] | [[Splenic marginal zone lymphoma surgery|Surgery]] | [[Splenic marginal zone lymphoma cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Splenic marginal zone lymphoma future or investigational therapies|Future or Investigational Therapies]] |
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| ===Spleen=== | | ==Case Studies== |
| 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"/><ref name="jaf1">
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| [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=6638043&query_hl=11&itool=pubmed_ExternalLink]
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| 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</ref> 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.
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| ===Splenic Hilar Lymph Nodes===
| | [[Splenic marginal zone lymphoma case study one|Case #1]] |
| 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"/>
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| ===Bone Marrow Biopsy===
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| SMZL 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]
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| 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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| ===Peripheral Blood===
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| Circulating [[lymphoma]] cells are sometimes present in peripheral [[blood]], and they occasionally show short villi at the poles of cells and plasmacytoid differentiation.<ref name="mel1">
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| [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=3497180&query_hl=15&itool=pubmed_ExternalLink]
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| 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."
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| '''J Clin Pathol.''' 1987 Jun;40(6):642-51. PMID: 3497180</ref>
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| ==Molecular Findings==
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| ===Immunophenotype===
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| {| class="wikitable" style="width: 20em; text-align: left; font-size: 100%;"
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| ! '''Antigen'''
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| | '''Status'''
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| |-
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| ! [[CD20]]
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| | Positive
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| |-
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| ! [[CD79a]]
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| | Positive
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| |-
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| ! [[CD5]]
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| | Negative
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| |-
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| ! [[CD10]]
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| | Negative
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| |-
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| ! [[CD23]]
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| | Negative
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| |-
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| ! [[CD43]]
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| | Negative
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| |-
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| ! [[cyclin D1]]
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| | Negative
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| |-
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| |}
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| The relevant markers that define the immunophenotype for SMZL are shown in the table to the right.<ref name="isa1">
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| [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=7949139&query_hl=17&itool=pubmed_ExternalLink]
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| 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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| [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=8123845&query_hl=20&itool=pubmed_ExternalLink]
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| 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."
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| '''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">
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| [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=9688179&query_hl=22&itool=pubmed_ExternalLink]
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| 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."
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| '''Mod Pathol'''. 1998 Jul;11(7):601-6. PMID: 9688179</ref>
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| ===Genetics===
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| 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]
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| 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">
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| [http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=10597225&query_hl=28&itool=pubmed_ExternalLink]
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| 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>
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| ==References==
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| <div class="references-small"><references/></div>
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| {{Hematological malignancy histology}} | | {{Hematological malignancy histology}} |
| | | [[Category:Disease]] |
| [[Category:Hematology|Splenic marginal zone lymphoma]] | | [[Category:Hematology|Splenic marginal zone lymphoma]] |
| [[Category:Types of cancer|Splenic marginal zone lymphoma]] | | [[Category:Types of cancer|Splenic marginal zone lymphoma]] |
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