B lymphoblastic leukemia/lymphoma with t(5;14)(q31;q32); IL3-IGH

Name
B lymphoblastic leukemia/lymphoma with t(5;14)(q31;q32); IL3-IGH
ICD-O-3 Morphology
9817/3
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
A neoplasm of lymphoblasts committed to the B lineage in which the blasts harbor a translocation between the IL3 gene and the IGH@ gene, resulting in a variable eosinophilia.

This diagnosis may be made based on immunophenotypic and genetic findings even if the bone marrow blast count is low.
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
Functional rearrangememt between the IL3 gene on chromosome 5 and the IGH@ gene on chromosome 14
Overexpression of IL3 gene
Immunophenotyping
CD10+
CD19+
Treatments
Chemotherapy
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: occurs in children and adults
Incidence: <1% of ALL
Survival: too few cases to be certain, although thought to be similar to other cases of ALL