Hydroa vacciniforme-like lymphoma

ICD-O-3 Morphology

9725/3: Hydro vacciniforme-like lymphoma
Effective 2010 and later


for cases diagnosed 2010 and later

Primary Site(s)

C440-C449, C510-C512, C518-C519, C600-C602, C608-C609, C632
Cutaneous (skin) lymphoma condition that primarily affects sun exposed skin, most commonly the face. See Module 7.


Not Applicable

Module Rule


Alternate Names

Hydroa vaccinoforme-like lymphoproliferative disorder


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.

Abstractor Notes

(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010 see code 9702/3).

Hydroa vacciniforme-like lymphoma is one of two major types of Epstein-Barr (EBV) associated T-cell lymphoproliferative disorders that have been reported in the pediatric age group. (See also 9724/3: Systemic EBV-positive T-cell lymphoproliferative disease of childhood)

Systemic spread and mosquito bite allergy/hypersensitivity result in a much more aggressive clinical course.

This disease is treated with UV (ultra-violet light) or PUVA (psoralen plus ultraviolet light). UV and PUVA are both coded as "Other Treatment."

For more information on lymphoma, see the NCI website: or

Definitive Diagnostic Methods

Genetic testing
Histologic confirmation

Genetics Data

Clonal expansion of gamma delta T cells
TR genes are clonally rearranged


CD4 positive
CD8 positive
CD56 expression


Other therapy

Transformations to

There are no known transformations

Transformations from

There are no known transformations

Corresponding ICD-9 Codes

202.8 Other lymphoma

Corresponding ICD-10 Codes

C84.5 Other and unspecified T-cell lymphomas

Corresponding ICD-10-CM Codes (U.S. only)

C84.Z Other mature T/NK-cell lymphomas (effective October 01, 2015)

Signs and Symptoms

Papulovesicular eruption resulting in ulceration and scarring

Diagnostic Exams

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


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, First Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.1 (2011) Morphological Codes

National Cancer Institute
Section: General Information About Adult Non-Hodgkin Lymphoma (NHL)