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This neoplasm is not reportable.
Name
Monoclonal immunoglobulin deposition disease
ICD-O-3 Morphology
9769/1: Immunoglobulin deposition disease
Effective
2001 and later
Reportability
This neoplasm is not reportable
Primary Site(s)
See Abstractor Notes
This neoplasm is reportable for primary sites C700-C729, C751-C753 only.
Alternate Names
Heavy chain deposition disease (HCDD)
Immunoglobulin deposition disease
Light and heavy chain deposition disease (LHCDD)
Light chain deposition disease (LCDD)
Monoclonal immunoglobulin deposition disease (MIDD)
Primary amyloidosis
Randall disease
Systemic light chain disease
Definition
The monoclonal immunoglobulin (Ig) deposition diseases are closely related disorders characterized by visceral and soft tissue deposition of aberrant Ig, resulting in compromised organ function.
There are two major categories of monoclonal Ig deposition diseases: primary amyloidosis and light chain and heavy chain disposition diseases. These disorders appear to be chemically different manifestations of similar pathological processes, resulting in clinically similar conditions
Primary amyloidosis
A plasma cell or (rarely) a lymphoplasmacytic neoplasm in which the monoclonal plasma cells secrete intact or fragments of abnormal immunoglobulin light chains that deposit in various tissues and form a beta-pleated sheet structure (amyloid sheet chain).
Light chain and heavy chain deposition diseases
Plasma cell or (rarely) lymphoplasmacytic neoplasm that secrete an abnormal light or (less often) have chain, or both, which deposit in tissues, causing organ dysfunction, but do not form amyloid beta-pleated sheets. These disorders comprise:
1) Light chain deposition disease (LCDD)
2) Heavy chain deposition disease (HCDD)
3) Light and heavy chain deposition disease (LHCDD)
There are two major categories of monoclonal Ig deposition diseases: primary amyloidosis and light chain and heavy chain disposition diseases. These disorders appear to be chemically different manifestations of similar pathological processes, resulting in clinically similar conditions
Primary amyloidosis
A plasma cell or (rarely) a lymphoplasmacytic neoplasm in which the monoclonal plasma cells secrete intact or fragments of abnormal immunoglobulin light chains that deposit in various tissues and form a beta-pleated sheet structure (amyloid sheet chain).
Light chain and heavy chain deposition diseases
Plasma cell or (rarely) lymphoplasmacytic neoplasm that secrete an abnormal light or (less often) have chain, or both, which deposit in tissues, causing organ dysfunction, but do not form amyloid beta-pleated sheets. These disorders comprise:
1) Light chain deposition disease (LCDD)
2) Heavy chain deposition disease (HCDD)
3) Light and heavy chain deposition disease (LHCDD)
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-9 Codes
238.79 Other lymphatic and hematopoietic tissues
277.30 Amyloidosis, unspecified
Corresponding ICD-10 Codes
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)
E85.9 Amyloidosis, unspecified (effective October 01, 2015)
Sources
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Mature B-cell neoplasms
Pages: 254-256
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Mature B-cell neoplasms
Pages: 254-256
International Classification of Diseases for Oncology, Third Edition, Second Revision. Geneva: World Health Organization, 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
National Cancer Institute
Section: General Information About Plasma Cell Neoplasms
Pages: https://www.cancer.gov/types/myeloma/hp/myeloma-treatment-pdq
Section: General Information About Plasma Cell Neoplasms
Pages: https://www.cancer.gov/types/myeloma/hp/myeloma-treatment-pdq
