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This neoplasm is not reportable.
Name
KSHV/HHV8-positive germinotropic lymphoproliferative disorder
Reportability
This neoplasm is not reportable
Abstractor Notes
For cases diagnosed prior to 2010-2020, see code 9738/3.
KSHV/HHV8-positive germinotropic lymphoproliferative disorder is part of the Mature B-cell neoplasms lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B15)
Per the IARC/WHO Committee for ICD-O and included in the WHO Tumors of Hematopoietic and Lymphoid Tissues, Revised 4th Ed, Volume 2, this histology is now a /1. This change will go into effect for 2021+.
Per the 5th edition of the Hematolymphoid Tumors blue book, 9738/1 is no longer used for this terminology. Although the definition is in the 5th edition, it no longer is assigned to an ICD-O code.
This disorder presents with localized and sometime multifocal lymph node involvement in otherwise healthy individuals.
KSHV/HHV8-positive germinotropic lymphoproliferative disorder is part of the Mature B-cell neoplasms lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B15)
Per the IARC/WHO Committee for ICD-O and included in the WHO Tumors of Hematopoietic and Lymphoid Tissues, Revised 4th Ed, Volume 2, this histology is now a /1. This change will go into effect for 2021+.
Per the 5th edition of the Hematolymphoid Tumors blue book, 9738/1 is no longer used for this terminology. Although the definition is in the 5th edition, it no longer is assigned to an ICD-O code.
This disorder presents with localized and sometime multifocal lymph node involvement in otherwise healthy individuals.
Alternate Names
HHV8-positive germinotropic lymphoproliferative disorder (GLPD)
Definition
Kaposi sarcoma–associated herpesvirus / human herpesvirus 8 (KSHV/HHV8)-positive germinotropic lymphoproliferative disorder (KSHV/HHV8-positive GLPD) is characterized by KSHV/HHV8-positive and usually EBV-positive large atypical lymphoid cells that predominantly colonize germinal centres. These lesions are usually polyclonal and classically arise in elderly, immunocompetent patients. (WHO 5th edition)
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
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)
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: KSHV/HHV8-associated B-cell lymphoid proliferations and lymphomas
Pages: Part B: 547-548
Section: KSHV/HHV8-associated B-cell lymphoid proliferations and lymphomas
Pages: Part B: 547-548
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
