HAEM4:Light Chain and Heavy Chain Deposition Disease: Difference between revisions
Bailey.Glen (talk | contribs) Created page with "==Primary Author(s)*== Chen Yang, MD, PhD, University of Michigan __TOC__ ==Cancer Category/Type== *[https://ccga.io/index.php/Mature_B-Cell_Neoplasms Mature B-cell neopla..." |
Bailey.Glen (talk | contribs) No edit summary |
||
| (One intermediate revision by the same user not shown) | |||
| Line 1: | Line 1: | ||
{{DISPLAYTITLE:Light Chain and Heavy Chain Deposition Disease}} | |||
<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]]. | |||
}}</blockquote> | |||
==Primary Author(s)*== | ==Primary Author(s)*== | ||
| Line 7: | Line 12: | ||
==Cancer Category/Type== | ==Cancer Category/Type== | ||
*[https://ccga.io/index.php/Mature_B-Cell_Neoplasms Mature B-cell neoplasms] | *[https://ccga.io/index.php/HAEM4:Mature_B-Cell_Neoplasms Mature B-cell neoplasms] | ||
==Cancer Sub-Classification / Subtype== | ==Cancer Sub-Classification / Subtype== | ||
*[https://ccga.io/index.php/Monoclonal_Immunoglobulin_Deposition_Diseases Monoclonal immunoglobulin deposition disease] | *[https://ccga.io/index.php/HAEM4:Monoclonal_Immunoglobulin_Deposition_Diseases Monoclonal immunoglobulin deposition disease] | ||
==Definition / Description of Disease== | ==Definition / Description of Disease== | ||
| Line 17: | Line 22: | ||
*Systemic disorder with non-amyloid deposits of monoclonal immunoglobulin (Ig) in various organs | *Systemic disorder with non-amyloid deposits of monoclonal immunoglobulin (Ig) in various organs | ||
*Underlying diseases are [[Plasma Cell Neoplasms]] (PCN, >95%) or lymphoplasmacytic neoplasms (2-3%) | *Underlying diseases are [[HAEM4:Plasma Cell Neoplasms]] (PCN, >95%) or lymphoplasmacytic neoplasms (2-3%) | ||
*20-35% have non-smoldering [[Plasma | *20-35% have non-smoldering [[HAEM5:Plasma cell myeloma / multiple myeloma]] (PCM), and rest (65-75%) have smoldering plasma cell myeloma or Monoclonal Gammopathy of Uncertain Significance (MGUS)/monoclonal gammopathy of renal significance (MGRS) | ||
==Synonyms / Terminology== | ==Synonyms / Terminology== | ||
| Line 71: | Line 76: | ||
==Morphologic Features== | ==Morphologic Features== | ||
*Most cases are associated with PCM or MGUS<ref name=":0" />, and rarely with [[Lymphoplasmacytic | *Most cases are associated with PCM or MGUS<ref name=":0" />, and rarely with [[HAEM5:Lymphoplasmacytic lymphoma]], marginal zone lymphoma, or [[HAEM5:Chronic lymphocytic leukaemia/small lymphocytic lymphoma]] | ||
*The smooth, ribbon-like linear Ig deposits consist of Congo red-negative amorphous eosinophilic material that is non-amyloid and non-fibrillary | *The smooth, ribbon-like linear Ig deposits consist of Congo red-negative amorphous eosinophilic material that is non-amyloid and non-fibrillary | ||
*Deposition of Ig is mostly found on renal biopsies but can be observed in bone marrow and other tissues | *Deposition of Ig is mostly found on renal biopsies but can be observed in bone marrow and other tissues | ||
| Line 78: | Line 83: | ||
==Immunophenotype== | ==Immunophenotype== | ||
Findings are similar to those of the underlying condition (e.g., [[Plasma Cell Neoplasms|Plasma Cell Neoplasm]], [[Lymphoplasmacytic | Findings are similar to those of the underlying condition (e.g., [[HAEM4:Plasma Cell Neoplasms|Plasma Cell Neoplasm]], [[HAEM5:Lymphoplasmacytic lymphoma]]). Additional findings may include the following<ref name=":2" /><ref name=":4" />: | ||
*The plasma cells in bone marrow may exhibit an aberrant kappa/lambda ratio | *The plasma cells in bone marrow may exhibit an aberrant kappa/lambda ratio | ||
| Line 101: | Line 106: | ||
==Genomic Gain/Loss/LOH== | ==Genomic Gain/Loss/LOH== | ||
*Findings are similar to those of the underlying condition (e.g., [[Plasma Cell Neoplasms|Plasma Cell Neoplasm]], [[Lymphoplasmacytic | *Findings are similar to those of the underlying condition (e.g., [[HAEM4:Plasma Cell Neoplasms|Plasma Cell Neoplasm]], [[HAEM5:Lymphoplasmacytic lymphoma]]) | ||
==Gene Mutations (SNV/INDEL)== | ==Gene Mutations (SNV/INDEL)== | ||
*Findings are similar to those of the underlying condition (e.g., [[Plasma Cell Neoplasms|Plasma Cell Neoplasm]], [[Lymphoplasmacytic | *Findings are similar to those of the underlying condition (e.g., [[HAEM4:Plasma Cell Neoplasms|Plasma Cell Neoplasm]], [[HAEM5:Lymphoplasmacytic lymphoma]]) | ||
==Epigenomics (Methylation)== | ==Epigenomics (Methylation)== | ||
*Findings are similar to those of the underlying condition (e.g., [[Plasma Cell Neoplasms|Plasma Cell Neoplasm]], [[Lymphoplasmacytic | *Findings are similar to those of the underlying condition (e.g., [[HAEM4:Plasma Cell Neoplasms|Plasma Cell Neoplasm]], [[HAEM5:Lymphoplasmacytic lymphoma]]) | ||
==Genes and Main Pathways Involved== | ==Genes and Main Pathways Involved== | ||
*Findings are similar to those of the underlying condition (e.g., [[Plasma Cell Neoplasms|Plasma Cell Neoplasm]], [[Lymphoplasmacytic | *Findings are similar to those of the underlying condition (e.g., [[HAEM4:Plasma Cell Neoplasms|Plasma Cell Neoplasm]], [[HAEM5:Lymphoplasmacytic lymphoma]]) | ||
==Diagnostic Testing Methods== | ==Diagnostic Testing Methods== | ||
| Line 143: | Line 148: | ||
==Links== | ==Links== | ||
*[[Monoclonal Immunoglobulin Deposition Diseases]] | *[[HAEM4:Monoclonal Immunoglobulin Deposition Diseases]] | ||
*[[ | *[[HAEM5:Immunoglobulin-related (AL) amyloidosis]] | ||
*[[Plasma | *[[HAEM5:Plasma cell myeloma / multiple myeloma|Plasma Cell Myeloma]] | ||
*[[Non-IgM | *[[HAEM5:Non-IgM monoclonal gammopathy of undetermined significance]] | ||
<br /> | <br /> | ||
| Line 155: | Line 160: | ||
==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]] | |||