Search Database ICD-O-3 Code Lists
This neoplasm is not reportable.

Name

Hydroa vacciniforme like 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
This neoplasm is reportable for primary sites C700-C729, C751-C753 only.

Abstractor Notes

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.

Alternate Names

Classic HV
EBV-associated HV-like lymphoproliferative disorder
Severe HV

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.

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-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)

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

Recurrent skin lesions for 10-15 years before progression to systemic involvement

Epidemiology and Mortality

Age: mainly children and adolescents
Country: Asia, Native Americans from Central and South America, Mexico
Survival: Once becomes systemic, patients usually die of infectious complications

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 T- and NK-cell neoplasms
Pages: 360-362

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