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HAEM4:Light Chain and Heavy Chain Deposition Disease (view source)
Revision as of 16:34, 4 December 2023
, 16:34, 4 December 2023no edit summary
==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==
*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 Cell Myeloma]] (PCM), and rest (65-75%) have smoldering plasma cell myeloma or Monoclonal Gammopathy of Uncertain Significance (MGUS)/monoclonal gammopathy of renal significance (MGRS)
*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==
==Morphologic Features==
==Morphologic Features==
*Most cases are associated with PCM or MGUS<ref name=":0" />, and rarely with [[Lymphoplasmacytic Lymphoma]], marginal zone lymphoma, or [[Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma]]
*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
==Immunophenotype==
==Immunophenotype==
Findings are similar to those of the underlying condition (e.g., [[Plasma Cell Neoplasms|Plasma Cell Neoplasm]], [[Lymphoplasmacytic Lymphoma]]). Additional findings may include the following<ref name=":2" /><ref name=":4" />:
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
==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 Lymphoma]])
*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 Lymphoma]])
*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 Lymphoma]])
*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 Lymphoma]])
*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==
==Links==
==Links==
*[[Monoclonal Immunoglobulin Deposition Diseases]]
*[[HAEM4:Monoclonal Immunoglobulin Deposition Diseases]]
*[[Primary Amyloidosis]]
*[[HAEM5:Immunoglobulin-related (AL) amyloidosis]]
*[[Plasma Cell Myeloma|Plasma Cell Myeloma]]
*[[HAEM5:Plasma cell myeloma / multiple myeloma|Plasma Cell Myeloma]]
*[[Non-IgM Monoclonal Gammopathy of Undetermined Significance]]
*[[HAEM5:Non-IgM monoclonal gammopathy of undetermined significance]]
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