HAEM4:Monoclonal Immunoglobulin Deposition Diseases

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Primary Author(s)*

Chen Yang, MD, PhD

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General Disease Overview / Description of Cancer Category

  • The major classification group of Monoclonal Immunoglobulin Deposition Diseases, as described in the revised 4th edition of the WHO, includes the categories: HAEM5:Immunoglobulin-related (AL) amyloidosis and HAEM5:Monoclonal immunoglobulin deposition disease
  • The monoclonal immunoglobulin (Ig) deposition diseases are closely related disorders characterized by deposition of aberrant Ig (mostly light chain and rarely heavy chain) in various tissues, leading to organ dysfunction[1]
  • Underlying disorders are plasma cell neoplasms or rarely lymphoplasmacytic neoplasm
  • Many patients at diagnosis have not developed overt myeloma or lymphoma
  • Even with low clonal neoplastic burden, systemic Ig deposition often results in aggressive disease progression and organ failure if untreated
  • While secreted monoclonal Ig products are soluble in most patients, some are amyloidogenic, and (more rarely) some are prone to form non-amyloid deposits
  • There are two major types of monoclonal Ig deposition diseases: primary amyloidosis, or Ig light chain (AL) amyloidosis; and light chain and heavy chain disposition diseases
  • Ig Heavy chain (AH) amyloidosis[2][3][4] and heavy-and-light-chain (AH/AL) amyloidosis are two rare forms of monoclonal Ig-related amyloidosis[5][6]

WHO Classification Pages (Includes Links to Content)

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References


  1. ↑ McKenna RW, et al., (2017). Plasma cell neoplasms: Monoclonal immunoglobulin deposition diseases, in World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, Revised 4th edition. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Arber DA, Hasserjian RP, Le Beau MM, Orazi A, and Siebert R, Editors. IARC Press: Lyon, France, p254-256.
  2. ↑ Eulitz, M.; et al. (1990-09). "Immunoglobulin heavy-chain-associated amyloidosis". Proceedings of the National Academy of Sciences of the United States of America. 87 (17): 6542–6546. doi:10.1073/pnas.87.17.6542. ISSN 0027-8424. PMC 54572. PMID 2118650. Check date values in: |date= (help)CS1 maint: PMC format (link)
  3. ↑ Solomon, A.; et al. (1994-02). "Primary amyloidosis associated with a novel heavy-chain fragment (AH amyloidosis)". American Journal of Hematology. 45 (2): 171–176. doi:10.1002/ajh.2830450214. ISSN 0361-8609. PMID 8141123. Check date values in: |date= (help)
  4. ↑ Miyazaki, Daigo; et al. (2008-06). "AH amyloidosis associated with an immunoglobulin heavy chain variable region (VH1) fragment: a case report". Amyloid: The International Journal of Experimental and Clinical Investigation: The Official Journal of the International Society of Amyloidosis. 15 (2): 125–128. doi:10.1080/13506120802006229. ISSN 1744-2818. PMID 18484339. Check date values in: |date= (help)
  5. ↑ Nasr, Samih H.; et al. (2013-03). "The diagnosis and characteristics of renal heavy-chain and heavy/light-chain amyloidosis and their comparison with renal light-chain amyloidosis". Kidney International. 83 (3): 463–470. doi:10.1038/ki.2012.414. ISSN 1523-1755. PMID 23302715. Check date values in: |date= (help)
  6. ↑ Chaulagain, Chakra P.; et al. (2020-11). "How We Manage Systemic Immunoglobulin Heavy Chain Amyloidosis (AH Amyloidosis) and Immunoglobulin Heavy-and-Light-Chain Amyloidosis (AH/AL Amyloidosis)". Clinical Lymphoma, Myeloma & Leukemia. 20 (11): e826–e831. doi:10.1016/j.clml.2020.06.017. ISSN 2152-2669. PMID 32703752 Check |pmid= value (help). Check date values in: |date= (help)

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