B lymphoblastic leukemia/lymphoma with t(1;19)(q23;p13.3); E2A-PBX1 (TCF3-PBX1)

Name
B lymphoblastic leukemia/lymphoma with t(1;19)(q23;p13.3); E2A-PBX1 (TCF3-PBX1)
ICD-O-3 Morphology
9818/3
Effective 2010 and later
Reportable
for cases diagnosed 2010 and later
Primary Site(s)
See Module 4: Rules PH7, PH8
Usually presents as a leukemia. Lymphomatous presentation is rare.

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Grade
6 - B-cell
Module Rule
Module 4: PH7, PH8
Alternate Names
None
Definition
This is a neoplasm of lymphoblasts committed to the B lineage with a t(1;19) translocation between the E2A(TCF3) gene on chromosome 19 and the PBX1 gene on chromosome 1.
Abstractor Notes
(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010 see codes: 9728/3 [lymphoma] or 9836/3 [leukemia].)
Definitive Diagnostic Methods
Bone marrow biopsy
Genetic testing
Immunophenotyping
Genetics Data
E2A-PBX1 translocation
Immunophenotyping
CD9+
CD10+ cytoplasmic heavy chain-positive pre-B-cell phenotype
CD19+
CD34-
Treatments
Chemotherapy
Stem cell transplant
Transformations to
There are no known transformations
Transformations from
There are no known transformations
Corresponding ICD-9 Codes
204.8 Other lymphoid leukemia
Corresponding ICD-10 Codes
C91.7 Other lymphoid leukemia
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C91.Z Other lymphoid leukemia
Signs and Symptoms
Anemia
Arthralgias
Bone pain
Hepatomegaly
Lymphoadenopathy
Splenomegaly
Diagnostic Exams
CT (CAT) scan
Complete blood count (CBC)
Cytogenetic analysis
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
MRI (magnetic resonance imaging)
PET (positron emission tomography) scan
Recurrence and Metastases
None
Epidemiology and Mortality
Age: more common in children, can occur in adults
Incidence: 6% of cases of B-ALL
Survival: poor prognosis, but changing due to more modern intensive therapy