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This neoplasm is not reportable.
Name
IgG4-related disease
ICD-O-3 Morphology
9760/1
Effective
2026 and later
Reportability
This neoplasm is not reportable
Primary Site(s)
See Module 7
This hematopoietic disease is not reportable for the U.S. except for primary sites C700-C729, C751-C753.
Canada, refer to your specific province for reportability requirements.
Most common primary sites: pancreas, salivary glands, orbit
Canada, refer to your specific province for reportability requirements.
Most common primary sites: pancreas, salivary glands, orbit
Abstractor Notes
(This code is effective for cases diagnosed 2026 and later.)
IgG4-related disease is part of the Tumor like lesion with B-cell predominance lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B13)
IgG4-RD can affect almost any organ, with the pancreas, salivary glands, and orbit being the most common. Multiorgan involvement, synchronously or metachronously, is typical, with three organs being affected on average.
Disease responsive to steroids; however, relapse is common. Progression of disease leads to organ failure.
IgG4-related disease is part of the Tumor like lesion with B-cell predominance lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B13)
IgG4-RD can affect almost any organ, with the pancreas, salivary glands, and orbit being the most common. Multiorgan involvement, synchronously or metachronously, is typical, with three organs being affected on average.
Disease responsive to steroids; however, relapse is common. Progression of disease leads to organ failure.
Alternate Names
Cold agglutinin disease
Primary cold agglutinin disease
Definition
Immunoglobulin G4–related disease (IgG4-RD) is an immune-mediated disease characterized by mass-forming lesions with a lymphoplasmacytic infiltrate rich in IgG4+ plasma cells and storiform fibrosis, usually with an elevated serum IgG4 titer. (WHO 5th edition).
Cold agglutinin disease (CAD) is an autoimmune haemolytic anaemia mediated by monoclonal cold agglutinins (autoantibodies that agglutinate erythrocytes at an optimum temperature of 0–4 °C) in the absence of any infection or lymphoma. The process is driven by an underlying clonal B-cell proliferation; however, it does not fulfil criteria for a B-cell lymphoma. (WHO 5th edition)
Cold agglutinin disease (CAD) is an autoimmune haemolytic anaemia mediated by monoclonal cold agglutinins (autoantibodies that agglutinate erythrocytes at an optimum temperature of 0–4 °C) in the absence of any infection or lymphoma. The process is driven by an underlying clonal B-cell proliferation; however, it does not fulfil criteria for a B-cell lymphoma. (WHO 5th edition)
Definitive Diagnostic Methods
This data item does not apply
Immunophenotyping
This data item does not apply
Treatments
Immunotherapy
Progression and Transformation
Disease does not transform, but it does increase the risk of carcinoma and lymphoma
Epidemiology and Mortality
Sources
WHO Classification of Tumours Editorial Board. Haematolymphoid tumours. Lyon (France): International Agency for Research on Cancer; 2024. (WHO classification of tumours series, 5th ed.; vol. 11). https://publications.iarc.who.int/637.
Section: B-cell lymphoproliferative disorders and neoplasms
Pages: Part B: 315-318, 599-601
Section: B-cell lymphoproliferative disorders and neoplasms
Pages: Part B: 315-318, 599-601
