Systemic EBV-positive T-cell lymphoproliferative disease of childhood

Name
Systemic EBV-positive T-cell lymphoproliferative disease of childhood
ICD-O-3 Morphology
9724/3
Effective 2010 and later
Reportable
for cases diagnosed 2010 and later
Primary Site(s)
See Abstractor Notes and Module 7

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Grade
5 - T-cell
Module Rule
None
Alternate Names
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
This neoplasm of childhood is 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). It has a rapid progression with mulitple organ failure, sepsis and death, usually from days to weeks. This entity shows some overlapping clinicopathologic features with aggressive NK-cell leukemia.
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. (The other is hydroa vacciniforme-like lymphoma, 9725/3.) Both occur with increased frequency in Asians and in Native Americans from Central and South America and Mexico.

Systemic EBV-positive T-cell lymphoproliferative disease of childhood is a systemic disease of children and young adults. The most commonly involved sites are the liver and the spleen, followed by lymph nodes, bone marrow, skin and lung. The etiology of this neoplasm is not known, but the association with primary EBV infection and the racial predisposition strongly suggests a genetic defect in the immune response to EBV. Previously healthy patients present with acute onset of fever and general malaise suggestive of acute viral respiratory illness. Within a period of weeks to months, the patients develop hepatosplenomegaly and liver failure, sometimes accompanied by lymphadenopathy. Most cases have a fulminant clinical course 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
Genetics testing
Histologic confirmation
Immunophenotype
Genetics Data
Monoclonally rearranged T-cell receptor (TCR) genes
Type A EBV
Immunophenotyping
CD2+
CD3+
CD4+
CD8+
CD56-
EBER+
TIA1+
Treatments
Chemotherapy
Immunotherapy
Stem cell transplant
Transformations to
No Transformations
Transformations from
No 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 (effective October 1, 2015 U.S. only)
C96.Z Other specified malignant neoplasms of lymphoid, hematopoietic and related tissue
Signs and Symptoms
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None