This neoplasm is not reportable.

Name

Monoclonal immunoglobulin deposition disease

ICD-O-3 Morphology

9769/1: Immunoglobulin deposition disease
Effective 2001 and later

Not Reportable

This neoplasm is not reportable

Primary Site(s)

See Abstractor Notes

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)
Randall 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)

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

D47.9 Neoplasm of uncertain or unknown behavior of lymphoid, hematopoietic, and related tissue, unspecified
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

International Classification of Diseases for Oncology, Third Edition, First Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2

National Cancer Institute
Section: General Information About Plasma Cell Neoplasms
Pages: https://www.cancer.gov/types/myeloma/hp/myeloma-treatment-pdq
Glossary