This neoplasm is not reportable.

Name

HHV8-positive germinotropic lymphoproliferative disorder

ICD-O-3 Morphology

9738/1: HHV8-positive germinotrophic lymphoproliferative disorder
Effective 2021 and later

Reportability

This neoplasm is not reportable

Primary Site(s)

No primary site specified
This neoplasm is reportable for primary sites C700-C729, C751-C753 only.

Abstractor Notes

For cases diagnosed prior to 2010-2020, see code 9738/3.

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+.

This disorder presents with localized and sometime multifocal lymph node involvement in otherwise healthy individuals.

Alternate Names

GLPD

Definition

HHV8-positive germinotropic lymphoproliferative disorder (GLPD) is a monotypic HHV8-positive lymphoproliferative lesion that usually occurs in HIV-negative individuals. HHV8 plasmablasts partially or completely replace germinal centers. The plasmablasts show either kappa or lambda light chain restriction but are polyclonal or oligoclonal. Coinfection with EBV is characteristic.

Genetics Data

This data item does not apply

Immunophenotyping

This data item does not apply

Corresponding ICD-9 Codes

238.79 Other lymphatic and hematopoietic tissues

Corresponding ICD-10 Codes

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

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: 328-329

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
Glossary