Myeloid and lymphoid neoplasms with PDGFRA rearrangement

Name
Myeloid and lymphoid neoplasms with PDGFRA rearrangement
ICD-O-3 Morphology
9965/3
Effective 2010 and later
Reportable
for cases diagnosed 2010 and later
Primary Site(s)
C421

Help me code for diagnosis year :

Grade
Code grade specified by pathologist. If no grade specified, code 9
Module Rule
None
Alternate Names
Chronic eosinophilic leukemia with FIP1L1-PDGFRA
Myeloproliferative variant of the hypereosinophilic syndrome
Definition
One of three myeloproliferative and lymphoid neoplasms associated with rearrangement of PDGFRA, PDGFRB and FGFR1. All result from formation of a fusion gene encoding an aberrant tyrosine kinase. This disease is associated with PDGFRA rearrangement that is associated with FIP1L1-PDGFRA formed as a result of a cryptic deletion at 4q12. Presentation is generally as chronic eosinophilic leukemia (CEL) but can be acute myeloid leukemia (AML) as well.
Abstractor Notes
(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010 see code 9960/3.) This is a multisystem disorder. The peripheral blood and bone marrow are always involved. Tissue infiltration by eosinophils can lead to tissue damage in the heart, lungs, central and peripheral nervous system, skin and GI tract. Spleen is enlarged in the majority of cases.
Definitive Diagnostic Methods
Bone marrow
FISH
Genetic testing
Immunophenotyping
Genetics Data
FIP1L1-PDGFRA fusion gene resulting from cryptic del (4) (q12)
Immunophenotyping
CD23+
CD25+
CD69+
Treatments
Chemotherapy
Other treatment
Transformations to
There are no known transformations
Transformations from
There are no known transformations
Corresponding ICD-9 Codes
205.8 Other myeloid leukemia
Corresponding ICD-10 Codes
C92.7 Other myeloid leukemia
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C92.Z Other myeloid leukemia
Signs and Symptoms
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None