Search Database ICD-O-3 Code Lists

Name

Systemic EBV-positive T-cell lymphoma of childhood (SEBVTCL)

ICD-O-3 Morphology

9724/3: Systemic EBV-positive T-cell lymphoproliferative disease of childhood
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

Module 7
Systemic disease usually at diagnosis, with involvement of liver, spleen, lymph nodes, bone marrow, skin and lung.
Common metastatic sites: liver, lung, and bone marrow
Spleen involvement is common due to dissemination of disease

See abstractor notes.

Coding Manual: Hematopoietic Coding Manual (PDF)

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 lymphoma of childhood (SEBVTCL) 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)

Patients are EBV+.

This disease is systemic and involves multiple sites. Most common are the spleen, liver, bone marrow, lymph nodes, skin and lungs.

Most cases are reported in Asia.

Diagnostic Confirmation

This histology can be determined by positive histology (including peripheral blood) with or without genetics and/or immunophenotyping. Review the Definitive Diagnostic Methods, Immunophenotyping and Genetics Data sections below, and the instructions in the Hematopoietic Manual for further guidance on assigning Diagnostic confirmation.

Module Rule

None

Alternate Names

Systemic EBV positive T-cell lymphoproliferative disease (LPD) of childhood

Definition

Systemic EBV-positive T-cell lymphoma (SEBVTCL) of childhood is a clonal proliferation of EBV-infected cytotoxic T cells occurring in immunocompetent children and young adults, often associated with haemophagocytic lymphohistiocytosis (HLH), systemic symptoms, multiorgan involvement, and a rapidly fatal course. (WHO 5th edition)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

Type A EBV, either with wildtype LMP1 or variant 30 bp deletion of LMP1

Immunophenotyping

CD2+ (expression/negative)
CD3+ (expression/negative)
CD4+ (expression/negative)
CD8+ (expression/negative)
CD56- (no expression/negative)
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

Same Primaries

Corresponding ICD-10 Codes (Cause of Death codes only)

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)
C84.ZA Other mature T/NK-cell lymphomas (effective October 01, 2024)

Signs and Symptoms

Abnormal EBV serology
Abnormal liver functions tests
Coagulopathy
Fever
General malaise
Hemophagocytic syndrome
Hepatosplenomegaly
Liver failure
Lymphadenopathy
Pancytopenia

Diagnostic Exams

Blood chemistry studies
Bone marrow aspiration and biopsy
CT (CAT) scan
Cytogenetic analysis
Flow cytometry
Immunohistochemistry
Immunophenotyping
PET (positron emission tomography) scan

Progression and Transformation

None

Epidemiology and Mortality

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

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: 777-781

International Classification of Diseases for Oncology, Third Edition, Second Revision. Geneva: World Health Organization, 2011, 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