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Code grade specified by pathologist. If no grade specified, code 9
8p11 myeloproliferative syndrome
8p11 stem cell leukemia/lymphoma syndrome
8p11 stem cell 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
(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.