Name

Systemic EBV-positive T-cell lymphoma of childhood

ICD-O-3 Morphology

9724/3: Systemic EBV-positive T-cell lymphoproliferative disease of childhood
Effective 2010 and later

Reportable

for cases diagnosed 2010 and later

Primary Site(s)

See Module 7
Most common sites of involvement: liver, spleen, lymph nodes, bone marrow, skin and lung.

Grade

Not Applicable

Module Rule

None

Alternate Names

Chronic active EBV infection of T- and NK-cell type, systemic form
Fatal EBV-associated hemophagocytic syndrome
Fulminant EBV+ T-cell LPD of childhood
Fulminant hemophagocytic syndrome in children
Severe CAEBV
Sporadic fatal infectious mononucleosis (FIM)
Systemic EBV+ T-cell LPD of childhood

Definition

Systemic EBV-positive T-cell lymphoma of childhood is a life-threatening illness of children and young adults, characterized by a clonal proliferation of EBV-infected T cells with an activated cytotoxic phenotype. It can occur shortly after primary acute EBV infection or in the setting of chronic active EBV infection (CAEBV).

Chronic active EBV infection of T- and NK-cell type, systemic form is a systemic EBV-positive polyclonal, oligoclonal, or )often) monoclonal lymphoproliferative disorder characterized by fever, persistent hepatitis, hepatosplenomegaly, and lymphadenopathy, which shows varying degrees of clinical severity depending on the immune response and the EBV viral load.

Abstractor Notes

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

Systemic EBV-positive T-cell lymphoproliferative disease of childhood 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 9725/3: Hydroa vacciniforme-like lymphoma)

Most cases have a fulminant clinical course with multiple organ failure and sepsis, resulting in death, usually within days or weeks of diagnosis. Some cases have a subacute course of several months to a year.

Definitive Diagnostic Methods

FISH
Genetic testing
Histologic confirmation
Immunophenotyping

Genetics Data

TR genes are clonally rearranged
Type A EBV

Immunophenotyping

CD2 positive
CD3 positive
CD4 positive
CD8 positive
CD56 negative
EBER positive
TIA1 positive phenotype

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Immunotherapy

Transformations to

There are no known transformations

Transformations from

There are no known transformations

Corresponding ICD-9 Codes

202.9 Other and unspecified malignant neoplasms

Corresponding ICD-10 Codes

C96.7 Other specified malignant neoplasms of lymphoid, hematopoietic and related tissue

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

C96.Z Other specified malignant neoplasms of lymphoid, hematopoietic and related tissue (effective October 01, 2015)
C96.9 Malignant neoplasm of lymphoid, hematopoietic and related tissue, unspecified (effective October 01, 2015)

Signs and Symptoms

Abnormal liver functions tests
Coagulopathy
Hemophagocytic syndrome
Liver failure
Pancytopenia

Diagnostic Exams

Progression and Transformation

None

Epidemiology and Mortality

Age: children and young adults
Country: Japan, Taiwan, Mexico
Sex: no male or female predominance
Survival: rapid progression with multiple organ failure, sepsis and death within days to weeks

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: 355-360

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
Glossary