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HAEM5:Monoclonal immunoglobulin deposition disease (view source)

Revision as of 17:03, 4 December 2023

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<blockquote class='blockedit'>{{Box-round|title=HAEM5 Conversion Notes|This page was converted to the new template on 2023-11-30. The original page can be found at [[HAEM4:Light Chain and Heavy Chain Deposition Disease]].

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<blockquote class='blockedit'>{{Box-round|title=HAEM5 Conversion Notes|This page was converted to the new template on 2023-12-04. The original page can be found at [[HAEM4:Light Chain and Heavy Chain Deposition Disease]].

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==Primary Author(s)*==

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==Cancer Category / Type==

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*[https://ccga.io/index.php/Mature_B-Cell_Neoplasms Mature B-cell neoplasms]

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*[https://ccga.io/index.php/HAEM4:Mature_B-Cell_Neoplasms Mature B-cell neoplasms]

==Cancer Sub-Classification / Subtype==

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*[https://ccga.io/index.php/Monoclonal_Immunoglobulin_Deposition_Diseases Monoclonal immunoglobulin deposition disease]

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*[https://ccga.io/index.php/HAEM4:Monoclonal_Immunoglobulin_Deposition_Diseases Monoclonal immunoglobulin deposition disease]

==Definition / Description of Disease==

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*Systemic disorder with non-amyloid deposits of monoclonal immunoglobulin (Ig) in various organs

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*Underlying diseases are [[Plasma Cell Neoplasms]] (PCN, >95%) or lymphoplasmacytic neoplasms (2-3%)

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*Underlying diseases are [[HAEM4:Plasma Cell Neoplasms]] (PCN, >95%) or lymphoplasmacytic neoplasms (2-3%)

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*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)

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*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==

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==Morphologic Features==

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*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~~]]

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*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

*Deposition of Ig is mostly found on renal biopsies but can be observed in bone marrow and other tissues

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<blockquote class='blockedit'>{{Box-round|title=v4:Immunophenotype|The content below was from the old template. Please incorporate above.}}

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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" />:

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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

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*Findings are similar to those of the underlying condition (e.g., [[Plasma Cell Neoplasms|Plasma Cell Neoplasm]], [[Lymphoplasmacytic ~~Lymphoma~~]])

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*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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<blockquote class='blockedit'>{{Box-round|title=v4:Gene Mutations (SNV/INDEL)|The content below was from the old template. Please incorporate above.}}

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*Findings are similar to those of the underlying condition (e.g., [[Plasma Cell Neoplasms|Plasma Cell Neoplasm]], [[Lymphoplasmacytic ~~Lymphoma~~]])

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*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==

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*Findings are similar to those of the underlying condition (e.g., [[Plasma Cell Neoplasms|Plasma Cell Neoplasm]], [[Lymphoplasmacytic ~~Lymphoma~~]])

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*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==

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<blockquote class='blockedit'>{{Box-round|title=v4:Genes and Main Pathways Involved|The content below was from the old template. Please incorporate above.}}

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*Findings are similar to those of the underlying condition (e.g., [[Plasma Cell Neoplasms|Plasma Cell Neoplasm]], [[Lymphoplasmacytic ~~Lymphoma~~]])

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*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==

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*[[Monoclonal Immunoglobulin Deposition Diseases]]

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*[[HAEM4:Monoclonal Immunoglobulin Deposition Diseases]]

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*[[~~Primary Amyloidosis~~]]

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*[[HAEM5:Immunoglobulin-related (AL) amyloidosis]]

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*[[Plasma ~~Cell Myeloma~~|Plasma Cell Myeloma]]

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*[[HAEM5:Plasma cell myeloma / multiple myeloma|Plasma Cell Myeloma]]

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*[[Non-IgM ~~Monoclonal Gammopathy~~ of ~~Undetermined Signiﬁcance~~]]

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*[[HAEM5:Non-IgM monoclonal gammopathy of undetermined significance]]

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