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This neoplasm is not reportable.
Name
Monoclonal immunoglobulin deposition disease (MIDD)
ICD-O-3 Morphology
9769/1: Immunoglobulin deposition disease
Effective
2001 and later
Reportability
This neoplasm is not reportable
Primary Site(s)
No primary site specified
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: heart, kidneys, liver, soft tissues (tongue, submandibular soft tissues, carpal tunnel, conjunctiva, cornea), gut, peripheral and autonomic nerves, skin, and lymph nodes
Canada, refer to your specific province for reportability requirements.
Most common primary sites: heart, kidneys, liver, soft tissues (tongue, submandibular soft tissues, carpal tunnel, conjunctiva, cornea), gut, peripheral and autonomic nerves, skin, and lymph nodes
Abstractor Notes
Monoclonal immunoglobulin deposition disease (MIDD) is part of the Plasma cell neoplasms and other diseases with paraproteins lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B17)
The heart, kidneys, liver, soft tissues (tongue, submandibular soft tissues, carpal tunnel, conjunctiva, cornea), gut, peripheral and autonomic nerves, skin, and lymph nodes can be involved.
Clues to the diagnosis of AL include multiple organ involvement, with two thirds of patients having more than one organ symptomatically involved with amyloid.
The heart, kidneys, liver, soft tissues (tongue, submandibular soft tissues, carpal tunnel, conjunctiva, cornea), gut, peripheral and autonomic nerves, skin, and lymph nodes can be involved.
Clues to the diagnosis of AL include multiple organ involvement, with two thirds of patients having more than one organ symptomatically involved with amyloid.
Alternate Names
Heavy chain AL amyloidosis
Heavy chain desposition disease (see 9762/3 for 2026+)
Immunoglobulin deposition disease
Immunoglobulin-related amyloidosis (AL amyloidosis), NOS
Light and heavy chain deposition disease (LHCDD)
Light chain amyloidosis
Light chain deposition disease (LCDD)
Localized AL amyloidosis
Primary amyloidosis
Randall-type monoclonal immunoglobulin deposition disease
Systemic AL amyloidosis
Systemic light chain disease
Definition
Immunoglobulin light chain amyloidosis (AL amyloidosis) is defined as extracellular deposition of immunoglobulin light chain–derived linear non-branching fibrils (with a width of 9 nm) produced by clonal plasma cells or B cells causing progressive end-organ dysfunction. (WHO 5th edition)
Genetics Data
This data item does not apply
Immunophenotyping
This data item does not apply
Treatments
Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Corresponding ICD-10 Codes (Cause of Death codes only)
D47.7 Other specified neoplasms of uncertain or unknown behavior of lymphoid, hematopoietic, and related tissue
E85.9 Amyloidosis, unspecified
Corresponding ICD-10-CM Codes (U.S. only)
D47.Z9 Other specified neoplasms of uncertain behavior of lymphoid, hematopoietic and related tissue (effective October 01, 2015)
E85._ Amyloidosis (effective October 01, 2015)
Epidemiology and Mortality
Incidence: 1 case per 100,000 person years
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: Plasma cell neoplasms and other diseases with paraproteins
Pages: Part B: 608-612
Section: Plasma cell neoplasms and other diseases with paraproteins
Pages: Part B: 608-612
International Classification of Diseases for Oncology, 3rd edition (including revisions). Geneva: World Health Organization, 2001, 2011, 2020.
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577
PDQ® Adult Treatment Editorial Board. PDQ Plasma Cell Neoplasms (Including Multiple Myeloma) Treatment. Bethesda, MD: National Cancer Institute. Updated <03/28/2025>. Available at: https://www.cancer.gov/types/myeloma/hp/myeloma-treatment-pdq. Accessed <03/30/2025>. [PMID: 26389362]
Section: Plasma Cell Neoplasms (Including Multiple Myeloma) Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/myeloma/hp/myeloma-treatment-pdq
Section: Plasma Cell Neoplasms (Including Multiple Myeloma) Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/myeloma/hp/myeloma-treatment-pdq
