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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]]. 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. <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>There are no established causes for splenic marginal zone lymphoma. Splenic marginal zone lymphoma must be differentiated from other diseases such as  [[chronic lymphocytic leukemia]],  [[follicular lymphoma]], and [[mantle cell lymphoma]].<ref name=cancer.gov> Splenic marginal zone lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf57e3e27c3994bd5327/. Accessed on January 14, 2016</ref> The incidence of splenic marginal zone lymphoma increases with age; the median age at diagnosis is 50 years.  Splenic marginal zone lymphoma affects men and women equally.<ref name="ber1">  
'''Splenic marginal zone lymphoma''' (SMZL) is a [[lymphoma]] comprised of [[B-cells]] that replace the normal architecture of the [[white pulp]] of the [[spleen]]. 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. <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>There are no established causes for splenic marginal zone lymphoma. Splenic marginal zone lymphoma must be differentiated from other diseases such as  [[chronic lymphocytic leukemia]],  [[follicular lymphoma]], and [[mantle cell lymphoma]].<ref name=cancer.gov> Splenic marginal zone lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf57e3e27c3994bd5327/. Accessed on January 14, 2016</ref> The incidence of splenic marginal zone lymphoma increases with age; the median age at diagnosis is 50 years.  Splenic marginal zone lymphoma affects men and women equally.<ref name="ber1">  
[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=10706860&query_hl=7&itool=pubmed_ExternalLink]
[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=10706860&query_hl=7&itool=pubmed_ExternalLink]
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>
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> There are no established risk factors for splenic marginal zone lymphoma. According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for splenic marginal zone  lymphoma.<ref> Recommendations. U.S Preventive Services Task Force. http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=SPLENIC+MARGINAL+ZONE+LYMPHOMA Accessed on December 23, 2015</ref> Low [[Hemoglobin]] levels, high [[lactate dehydrogenase]] levels, low blood serum [[albumin]] levels,  and  genetic mutations such as mutations in ''NOTCH2'' are associated with poor prognosis among patients with splenic marginal zone lymphoma. According to the Lugano classification, there are four stages of splenic marginal zone lymphoma based on the number of nodes and extranodal involvement. The most common symptoms of splenic marginal zone lymphoma include [[fever]], [[weight loss]], skin rash, [[night sweats]], chest pain, [[abdominal pain]], [[bone pain]], and painless swelling in the neck, axilla, groin, thorax, and abdomen. Common physical examination findings of splenic marginal zone lymphoma include [[fever]], [[rash]], [[ulcer]], [[splenomegaly]], chest tenderness, abdomen tenderness, bone tenderness, [[Lymphadenopathy|peripheral lymphadenopathy]], and [[Lymphadenopathy|central lymphadenopathy]].<ref name= seer>Splenic marginal zone lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf57e3e27c3994bd5327/ Accessed on December 22, 2015 </ref>
 
 


==Pathophysiology==
==Pathophysiology==
Line 19: Line 17:
[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=10706860&query_hl=7&itool=pubmed_ExternalLink]
[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=10706860&query_hl=7&itool=pubmed_ExternalLink]
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>
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>
==Risk Factors==
There are no established risk factors for splenic marginal zone lymphoma.
==Screening==
According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for splenic marginal zone  lymphoma.<ref> Recommendations. U.S Preventive Services Task Force. http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=SPLENIC+MARGINAL+ZONE+LYMPHOMA Accessed on December 23, 2015</ref>
==Prognosis==
Low [[Hemoglobin]] levels, high [[lactate dehydrogenase]] levels, low blood serum [[albumin]] levels,  and  genetic mutations such as mutations in ''NOTCH2'' are associated with poor prognosis among patients with splenic marginal zone lymphoma.
==Staging==
According to the Lugano classification, there are four stages of splenic marginal zone lymphoma based on the number of nodes and extranodal involvement. 
==Symptoms==
The most common symptoms of splenic marginal zone lymphoma include [[fever]], [[weight loss]], skin rash, [[night sweats]], chest pain, [[abdominal pain]], [[bone pain]], and painless swelling in the neck, axilla, groin, thorax, and abdomen.
==Physical Examination==
Common physical examination findings of splenic marginal zone lymphoma include [[fever]], [[rash]], [[ulcer]], [[splenomegaly]], chest tenderness, abdomen tenderness, bone tenderness, [[Lymphadenopathy|peripheral lymphadenopathy]], and [[Lymphadenopathy|central lymphadenopathy]].<ref name= seer>Splenic marginal zone lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf57e3e27c3994bd5327/ Accessed on December 22, 2015 </ref>


==References==
==References==

Revision as of 13:49, 18 January 2016

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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

Splenic marginal zone lymphoma (SMZL) is a lymphoma comprised of B-cells that replace the normal architecture of the white pulp of the spleen. 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. [1]There are no established causes for splenic marginal zone lymphoma. Splenic marginal zone lymphoma must be differentiated from other diseases such as chronic lymphocytic leukemia, follicular lymphoma, and mantle cell lymphoma.[2] The incidence of splenic marginal zone lymphoma increases with age; the median age at diagnosis is 50 years. Splenic marginal zone lymphoma affects men and women equally.[3] There are no established risk factors for splenic marginal zone lymphoma. According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for splenic marginal zone lymphoma.[4] Low Hemoglobin levels, high lactate dehydrogenase levels, low blood serum albumin levels, and genetic mutations such as mutations in NOTCH2 are associated with poor prognosis among patients with splenic marginal zone lymphoma. According to the Lugano classification, there are four stages of splenic marginal zone lymphoma based on the number of nodes and extranodal involvement. The most common symptoms of splenic marginal zone lymphoma include fever, weight loss, skin rash, night sweats, chest pain, abdominal pain, bone pain, and painless swelling in the neck, axilla, groin, thorax, and abdomen. Common physical examination findings of splenic marginal zone lymphoma include fever, rash, ulcer, splenomegaly, chest tenderness, abdomen tenderness, bone tenderness, peripheral lymphadenopathy, and central lymphadenopathy.[5]

Pathophysiology

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. [1]

Causes

There are no established causes for splenic marginal zone lymphoma.

Differential Diagnosis

Splenic marginal zone lymphoma must be differentiated from other diseases such as chronic lymphocytic leukemia, follicular lymphoma, and mantle cell lymphoma.[2]

Epidemiology and demographics

The incidence of splenic marginal zone lymphoma increases with age; the median age at diagnosis is 50 years. Splenic marginal zone lymphoma affects men and women equally.[3]

Risk Factors

There are no established risk factors for splenic marginal zone lymphoma.

Screening

According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for splenic marginal zone lymphoma.[6]

Prognosis

Low Hemoglobin levels, high lactate dehydrogenase levels, low blood serum albumin levels, and genetic mutations such as mutations in NOTCH2 are associated with poor prognosis among patients with splenic marginal zone lymphoma.

Staging

According to the Lugano classification, there are four stages of splenic marginal zone lymphoma based on the number of nodes and extranodal involvement.

Symptoms

The most common symptoms of splenic marginal zone lymphoma include fever, weight loss, skin rash, night sweats, chest pain, abdominal pain, bone pain, and painless swelling in the neck, axilla, groin, thorax, and abdomen.

Physical Examination

Common physical examination findings of splenic marginal zone lymphoma include fever, rash, ulcer, splenomegaly, chest tenderness, abdomen tenderness, bone tenderness, peripheral lymphadenopathy, and central lymphadenopathy.[5]


References

  1. ↑ 1.0 1.1 [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. ↑ 2.0 2.1 Splenic marginal zone lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf57e3e27c3994bd5327/. Accessed on January 14, 2016
  3. ↑ 3.0 3.1 [2] 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
  4. ↑ Recommendations. U.S Preventive Services Task Force. http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=SPLENIC+MARGINAL+ZONE+LYMPHOMA Accessed on December 23, 2015
  5. ↑ 5.0 5.1 Splenic marginal zone lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf57e3e27c3994bd5327/ Accessed on December 22, 2015
  6. ↑ Recommendations. U.S Preventive Services Task Force. http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=SPLENIC+MARGINAL+ZONE+LYMPHOMA Accessed on December 23, 2015

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