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<blockquote class='blockedit'>{{Box-round|title=HAEM5 Conversion Notes|This page was converted to the new template on 2023-11-03. The original page can be found at [[HAEM4:Light Chain and Heavy Chain Deposition Disease]].

<blockquote class='blockedit'>{{Box-round|title=HAEM5 Conversion Notes|This page was converted to the new template on 2023-12-07. The original page can be found at [[HAEM4:Light Chain and Heavy Chain Deposition Disease]].

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==Primary Author(s)*==

==Primary Author(s)*==

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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==

*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

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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

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==Individual Region Genomic Gain/Loss/LOH==

==Individual Region Genomic Gain / Loss / LOH==

Put your text here and fill in the table <span style="color:#0070C0">(''Instructions: Includes aberrations not involving gene fusions. Can include references in the table. Can refer to CGC workgroup tables as linked on the homepage if applicable.'') </span>

Put your text here and fill in the table <span style="color:#0070C0">(''Instructions: Includes aberrations not involving gene fusions. Can include references in the table. Can refer to CGC workgroup tables as linked on the homepage if applicable.'') </span>

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*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]])

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==Gene Mutations (SNV/INDEL)==

==Gene Mutations (SNV / INDEL)==

Put your text here and fill in the table <span style="color:#0070C0">(''Instructions: This table is not meant to be an exhaustive list; please include only genes/alterations that are recurrent and common as well either disease defining and/or clinically significant. Can include references in the table. For clinical significance, denote associations with FDA-approved therapy (not an extensive list of applicable drugs) and NCCN or other national guidelines if applicable; Can also refer to CGC workgroup tables as linked on the homepage if applicable as well as any high impact papers or reviews of gene mutations in this entity.'') </span>

Put your text here and fill in the table <span style="color:#0070C0">(''Instructions: This table is not meant to be an exhaustive list; please include only genes/alterations that are recurrent and common as well either disease defining and/or clinically significant. Can include references in the table. For clinical significance, denote associations with FDA-approved therapy (not an extensive list of applicable drugs) and NCCN or other national guidelines if applicable; Can also refer to CGC workgroup tables as linked on the homepage if applicable as well as any high impact papers or reviews of gene mutations in this entity.'') </span>

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*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]])

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==Epigenomic Alterations==

==Epigenomic Alterations==

*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==

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*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]])

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==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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