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

Help me code for diagnosis year :

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).

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. (See also 9725/3: Hydroa vacciniforme-like lymphoma)

Systemic EBV-positive T-cell lymphoproliferative disease of childhood is a systemic disease of children and young adults. The etiology of this neoplasm is not known. A genetic defect in the immune response to EBV is suspected.

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
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
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 (effective October 1, 2015 U.S. only)
C96.Z Other specified malignant neoplasms of lymphoid, hematopoietic and related tissue
Signs and Symptoms
Abnormal EBV serology
Abnormal liver functions tests
Coagulopathy
Hemophagocytic syndrome
Liver failure
Pancytopenia
Diagnostic Exams
CT (CAT) scan
Complete blood count (CBC)
MRI (magnetic resonance imaging)
PET (positron emission tomography) scan
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