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This neoplasm is not reportable.
Name
Hydroa vaccinoforme lymphoproliferative disorder
ICD-O-3 Morphology
9725/1: Hydroa vacciniforme-like lymphoproliferative disorder
Effective
2021 and later
Reportability
This neoplasm is not reportable
Primary Site(s)
No primary site specified
Cutaneous primary sites only (C440-C449, C510-C512, C518-C519, C600-C602, C608-C609, C632)
This hematopoietic disease is not reportable for the U.S.
Canada, refer to your specific province for reportability requirements.
This hematopoietic disease is not reportable for the U.S.
Canada, refer to your specific province for reportability requirements.
Abstractor Notes
Hydroa vaccinoforme lymphoproliferative disorder is part of the Mature T-cell and NK-cell neoplasms lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B20)
This histology was previous defined as 9725/3, Hydro vacciniforme-like lymphoma for years 2010-2020. If this diagnosis occurs within years 2010-2020, see 9725/3 and report the case. If diagnosed 2021+, it is not reportable.
In the classic form HVD, sun-exposed skin is involved. In the systemic form, there is often involvement of non–sun-exposed skin as well, in addition to multiorgan involvement (e.g. liver, spleen, lymph nodes, and very rarely, lung)
Systemic chronic active EBV disease involves multiple sites, most commonly the spleen and the liver, followed by the bone marrow, lymph nodes, skin, and lungs.
This histology was previous defined as 9725/3, Hydro vacciniforme-like lymphoma for years 2010-2020. If this diagnosis occurs within years 2010-2020, see 9725/3 and report the case. If diagnosed 2021+, it is not reportable.
In the classic form HVD, sun-exposed skin is involved. In the systemic form, there is often involvement of non–sun-exposed skin as well, in addition to multiorgan involvement (e.g. liver, spleen, lymph nodes, and very rarely, lung)
Systemic chronic active EBV disease involves multiple sites, most commonly the spleen and the liver, followed by the bone marrow, lymph nodes, skin, and lungs.
Alternate Names
Hydroa vacciniforme-like lymphoma (see 9725/3 prior to 2021)
Hydroa vaccinoforme-like lymphoproliferative disorder
Hydroa vacciniforme lymphoproliferative disorder, classic (Classic HV-LPD)
Systemic chronic active EBV-positive disease
Definition
Hydroa vaccinoforme- (HV) like lymphoproliferative disorder is a chronic EBV-positive lymphoproliferative disorder of childhood, associated with a risk of developing systemic lymphoma. HV-like lymphoproliferative disorder is primarily a cutaneous disorder of polyclonal or (most often) monoclonal T cells or NK cells, which a broad spectrum of clinical aggressiveness and usually a long clinical course. As the disease progresses, patients develop severe and extensive skin lesions and systemic symptoms including fever, hepatosplenomegaly, and lymphadenopathy.
Classic HV, severe HV, and HV-like T-cell lymphoma constitute a continuous spectrum of EBV-associated HV-like lymphoproliferative disorder.
Classic HV, severe HV, and HV-like T-cell lymphoma constitute a continuous spectrum of EBV-associated HV-like lymphoproliferative disorder.
Definitive Diagnostic Methods
This data item does not apply
Genetics Data
This data item does not apply
Immunophenotyping
This data item does not apply
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)
Signs and Symptoms
Fever
Hepatosplenomegaly
Lymphadenopathy
Papulovesicular eruption resulting in ulceration and scarring
Wasting
Diagnostic Exams
Blood chemistry studies
Bone marrow aspiration and biposy
CT (CAT) scan
Cytogenetic analysis
Flow cytometry
Immunohistochemistry
PET (positron emission tomography) scan
Progression and Transformation
Epidemiology and Mortality
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: Mature T- and NK-cell neoplasms
Pages: Part B: 774-776
Section: Mature T- and NK-cell neoplasms
Pages: Part B: 774-776
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
