Myeloid and lymphoid neoplasms with FGFR1 abnormalities

Name
Myeloid and lymphoid neoplasms with FGFR1 abnormalities
ICD-O-3 Morphology
9967/3
Effective 2010 and later
Reportable
for cases diagnosed 2010 and later
Primary Site(s)
C421

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Grade
Code grade specified by pathologist. If no grade specified, code 9
Module Rule
None
Alternate Names
8p11 myeloproliferative syndrome
8p11 stem cell leukemia/lymphoma syndrome
8p11 stem cell syndrome
Definition
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. Some patients present as lymphoma with mainly lymph node involvement while others present with myeloproliferative features such as splenomegaly and hypermetabolism, and yet others with features of AML or Myeloid sarcoma. Systemic symptoms such as fever, weight loss and night sweats are often present. The prognosis is currently poor. 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
PCR
Genetics Data
Trisomy 21
Immunophenotyping
None
Treatments
Immunotherapy
Stem cell transplant
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