Name

Hydroa vacciniforme-like lymphoma

ICD-O-3 Morphology

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

Reportable

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.

Grade

Not Applicable

Module Rule

None

Alternate Names

Hydroa vaccinoforme-like lymphoproliferative disorder

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.

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: http://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq#section/_1 or http://www.cancer.gov/types/lymphoma/patient/adult-nhl-treatment-pdq#section/_129

Definitive Diagnostic Methods

FISH
Genetic testing
Histologic confirmation
Immunophenotyping

Genetics Data

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

Immunophenotyping

CD4 positive
CD8 positive
CD56 expression

Treatments

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
Wasting

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

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, First Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2

National Cancer Institute
Section: General Information About Adult Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
Glossary