Myeloid neoplasms with PDGFRB rearrangement

Name
Myeloid neoplasms with PDGFRB rearrangement
ICD-O-3 Morphology
9966/3
Effective 2010 and later
Reportable
for cases diagnosed 2010 and later
Primary Site(s)
C421

Help me code for dx year :

Grade
Code grade specified by pathologist. If no grade specified, code 9
Module Rule
None
Alternate Names
Chronic myelomonocytic leukemia with eosinophilia associated with t(5;12)
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 occurs in association with rearrangement of PDGFRB at 5q31-33. Usually there is (5;12)(q31-33;p12) with formation of an ETV6-PDGFRB fusion gene. The features are most consistent with CMML, but some patients have been characterized as atypical chronic myeloid leukemia.
Abstractor Notes
(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010 see code 9960/3.) Myeloid neoplasms with PDGFRB rearrangement is a myeloproliferative neoplasm associated with t(5;12)(q31-33;p12). It is a multisystem disorder. The peripheral blood and bone marrow are always involved. The spleen is enlarged in majority of patients. Skin infiltration may be present and some have cardiac damage leading to cardiac failure. Serum tryptase may be mildly or moderately elevated. The white cell count is increased. There may be anemia and thrombocytopenia. This neoplasm is considerably more common in men and has a wide age range (8-12 years) with the peak incidence being in middle-aged adults. Median age of onset is in the late 49's. Patients have splenomegaly with hepatomegaly being present in a majority. A good majority of patients treated with imatinib have been responsive.
Definitive Diagnostic Methods
Bone marrow biopsy
Cytochemistry
FISH
Genetic testing
Immunophenotyping
Genetics Data
ETV6-PDGFRB fusion gene
t(5;12)(q31-33;p12)
Immunophenotyping
CD2+
CD25+
Treatments
Chemotherapy
Other RX
Transformations to
No Transformations
Transformations from
No 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