HAEM4:Waldenstrom Macroglobulinemia: Difference between revisions
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{{DISPLAYTITLE:Waldenstrom Macroglobulinemia}} | |||
<blockquote class='blockedit'>{{Box-round|title=PREVIOUS EDITION|This page from the 4th edition of Haematolymphoid Tumours is being updated. See 5th edition [[HAEM5:Table_of_Contents|Table of Contents]]. | |||
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==Primary Author(s)*== | ==Primary Author(s)*== | ||
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==Definition / Description of Disease== | ==Definition / Description of Disease== | ||
Waldenström macroglobulinemia (WM) is a clinicopathological entity characterized by presence of [[Lymphoplasmacytic | Waldenström macroglobulinemia (WM) is a clinicopathological entity characterized by presence of [[HAEM5:Lymphoplasmacytic lymphoma|Lymphoplasmacytic lymphoma]] (LPL) associated with any level of monoclonal immunoglobulin M (IgM) in the serum. | ||
==Synonyms / Terminology== | ==Synonyms / Terminology== | ||
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==Sites of Involvement== | ==Sites of Involvement== | ||
*By definition, diagnosis of Waldenström macroglobulinemia requires bone marrow involvement by [[Lymphoplasmacytic | *By definition, diagnosis of Waldenström macroglobulinemia requires bone marrow involvement by [[HAEM5:Lymphoplasmacytic lymphoma|Lymphoplasmacytic lymphoma]]. | ||
*Lymphadenopathy and hepatosplenomegaly are more common at the time of relapse (up to 50%) rather than at the time of initial presentation (20%). <ref>{{Cite journal|last=Shaheen|first=Saad P.|last2=Talwalkar|first2=Sameer S.|last3=Lin|first3=Pei|last4=Medeiros|first4=L. Jeffrey|date=2012-01|title=Waldenström macroglobulinemia: a review of the entity and its differential diagnosis|url=https://pubmed.ncbi.nlm.nih.gov/22156831|journal=Advances in Anatomic Pathology|volume=19|issue=1|pages=11–27|doi=10.1097/PAP.0b013e31824019d0|issn=1533-4031|pmid=22156831}}</ref> | *Lymphadenopathy and hepatosplenomegaly are more common at the time of relapse (up to 50%) rather than at the time of initial presentation (20%). <ref>{{Cite journal|last=Shaheen|first=Saad P.|last2=Talwalkar|first2=Sameer S.|last3=Lin|first3=Pei|last4=Medeiros|first4=L. Jeffrey|date=2012-01|title=Waldenström macroglobulinemia: a review of the entity and its differential diagnosis|url=https://pubmed.ncbi.nlm.nih.gov/22156831|journal=Advances in Anatomic Pathology|volume=19|issue=1|pages=11–27|doi=10.1097/PAP.0b013e31824019d0|issn=1533-4031|pmid=22156831}}</ref> | ||
*Peripheral blood may have lymphocytosis with small circulating neoplastic cells with condensed chromatin and inconspicuous nucleoli. Rouleaux formation is also observed. <ref name=":5" /> | *Peripheral blood may have lymphocytosis with small circulating neoplastic cells with condensed chromatin and inconspicuous nucleoli. Rouleaux formation is also observed. <ref name=":5" /> | ||
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==Morphologic Features== | ==Morphologic Features== | ||
*Bone marrow involvement by [[Lymphoplasmacytic | *Bone marrow involvement by [[HAEM5:Lymphoplasmacytic lymphoma|lymphoplasmacytic lymphoma]] is characterized by an interstitial, diffuse, or nodular patterns of infiltration. <ref name=":1" /> | ||
*Tumor burden of the bone marrow is variable with a wide range of percentage of involvement (5-95%). <ref name=":6" /> | *Tumor burden of the bone marrow is variable with a wide range of percentage of involvement (5-95%). <ref name=":6" /> | ||
*Paratrabecular infiltration is composed of nodular aggregates or single cells along the bone trabeculae with peritrabecular fibrosis and is seen in 95% of cases. <ref name=":4">{{Cite journal|last=Bassarova|first=Assia|last2=Trøen|first2=Gunhild|last3=Spetalen|first3=Signe|last4=Micci|first4=Francesca|last5=Tierens|first5=Anne|last6=Delabie|first6=Jan|date=2015-06|title=Lymphoplasmacytic lymphoma and marginal zone lymphoma in the bone marrow: paratrabecular involvement as an important distinguishing feature|url=https://pubmed.ncbi.nlm.nih.gov/25972321|journal=American Journal of Clinical Pathology|volume=143|issue=6|pages=797–806|doi=10.1309/AJCP6ZODWV1CIDME|issn=1943-7722|pmid=25972321}}</ref> | *Paratrabecular infiltration is composed of nodular aggregates or single cells along the bone trabeculae with peritrabecular fibrosis and is seen in 95% of cases. <ref name=":4">{{Cite journal|last=Bassarova|first=Assia|last2=Trøen|first2=Gunhild|last3=Spetalen|first3=Signe|last4=Micci|first4=Francesca|last5=Tierens|first5=Anne|last6=Delabie|first6=Jan|date=2015-06|title=Lymphoplasmacytic lymphoma and marginal zone lymphoma in the bone marrow: paratrabecular involvement as an important distinguishing feature|url=https://pubmed.ncbi.nlm.nih.gov/25972321|journal=American Journal of Clinical Pathology|volume=143|issue=6|pages=797–806|doi=10.1309/AJCP6ZODWV1CIDME|issn=1943-7722|pmid=25972321}}</ref> | ||
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==Notes== | ==Notes== | ||
<nowiki>*</nowiki>Primary authors will typically be those that initially create and complete the content of a page. If a subsequent user modifies the content and feels the effort put forth is of high enough significance to warrant listing in the authorship section, please contact the CCGA coordinators (contact information provided on the homepage). Additional global feedback or concerns are also welcome. | <nowiki>*</nowiki>Primary authors will typically be those that initially create and complete the content of a page. If a subsequent user modifies the content and feels the effort put forth is of high enough significance to warrant listing in the authorship section, please contact the CCGA coordinators (contact information provided on the homepage). Additional global feedback or concerns are also welcome. | ||
[[Category:HAEM4]] [[Category:DISEASE]] | |||