Myeloid and lymphoid neoplasms with FGFR1 abnormalities

ICD-O-3 Morphology
9967/3: Myeloid and lymphoid neoplasms with FGFR1 abnormalities
Effective 2010 and later
for cases diagnosed 2010 and later
Primary Site(s)
Primary site must be bone marrow (C421)

Not Applicable
Module Rule
Alternate Names
8p11 myeloproliferative syndrome
One of three myeloproliferative 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 derived from a pluripotent hematopoietic stem cell. Patients can present as lymphoma with mainly lymph node involvement, or with myeloproliferative features, such as splenomegaly, AML or myeloid sarcoma.
Abstractor Notes
(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010, see code 9960/3.)

Myeloid and lymphoid neoplasms associated FGFR1 abnormalities primarily involve the bone marrow, peripheral blood, lymph nodes, liver and spleen. Lymphadenopathy is the result of infiltration by lymphoblasts or myeloid cells.

There is no established tyrosine kinase inhibitor therapy for MPN with FGFR1 rearrangement.

Interferon has induced a cytogenetic response in several patients. Until specific therapy is developed, hematopoietic stem cell transplantation should be considered even in those who present in chronic phase.
Definitive Diagnostic Methods
Bone marrow biopsy
Genetic testing
Polymerase chain reaction (PCR)
Genetics Data
Stem cell transplant
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 (U.S. only)
C92.Z Other myeloid leukemia (effective October 01, 2015)
Signs and Symptoms
Lymph node enlargement
Night sweats
Weight loss
Progression and Transformation
Epidemiology and Mortality
Age: 32 years median age (3-84 age range)
Sex: moderate male predominance
Survival: currently poor, specific therapy still being developed