Splenic marginal zone lymphoma: Difference between revisions

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	Splenic marginal zone lymphoma;
ICD-O:	9689/3

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Revision as of 16:54, 18 September 2012

For patient information, click here

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [6]

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.^[1]^[2] 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.^[3]

Genetics

Clonal rearrangements of the immunoglobulin genes (heavy and light chains) are frequently seen.^[4] The deletion 7q21-32 is seen in 40% of SMZL patients, and translocations of the CDK6 gene located at 7q21 have also been reported.^[5]

References

↑ [1] 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
↑ [2] 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
↑ [3] 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
↑ [4] 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
↑ [5] 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

Template:WikiDoc Sources

[isa1-1] [1] 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

[mat1-2] [2] 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

[sav1-3] [3] 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

[dun1-4] [4] 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

[cor1-5] [5] 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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-==Morphology==
-===Spleen===
-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">
-[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=6638043&query_hl=11&itool=pubmed_ExternalLink]
-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.
-===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"/>
-===Bone Marrow Biopsy===
-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]
-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>
-===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.<ref name="mel1">
-[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=3497180&query_hl=15&itool=pubmed_ExternalLink]
-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</ref>
 ==Molecular Findings==

v t e Myeloid Hematological malignancy/leukemia histology (ICD-O 9590-9989, C81-C96, 200-208)
CFU-GM/ and other granulocytes	Template:Navbox subgroup
MEP	Template:Navbox subgroup
CFU-Mast	Mastocytoma (Mast cell leukemia, Mast cell sarcoma, Systemic mastocytosis)
Multiple/unknown	AML (Acute panmyelosis with myelofibrosis, Myeloid sarcoma) · MP (Myelofibrosis) · Acute biphenotypic leukaemia
See also hematology, lymphoid malignancy