HAEM4:Light Chain and Heavy Chain Deposition Disease: Difference between revisions
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==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== | ||
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*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== | ||
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==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 | ||
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==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 | ||
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==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== | ||
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==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]] | ||
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