K Gowin1, S Verstovsek2, N Daver2, N Pemmaraju2, R Valdez3, H Kosiorek1, A Dueck1, R Mesa4. 1. Mayo Clinic Arizona, Scottsdale, AZ, United States. 2. MD Anderson Cancer Center, Houston, TX, United States. 3. Miraca Life Sciences, Phoenix, AZ, United States. 4. Mayo Clinic Arizona, Scottsdale, AZ, United States. Electronic address: Mesa.ruben@mayo.edu.
Abstract
BACKGROUND: The clinical phenotype of patients with myeloproliferative neoplasms (MPNs) including primary myelofibrosis (PMF), polycythemia vera (PV), and essential thrombocytosis (ET) whom manifest WHO grade 1 marrow fibrosis is poorly defined. Current IWG-MRT criteria require 2+ marrow fibrosis for diagnosis of post PV/ET myelofibrosis (MF). In contrast, the 2008 WHO definition of PMF does not require a minimum fibrosis threshold. METHODS: We retrospectively analyzed the clinical characteristics of 91 MPN patients with 1+ marrow fibrosis. We compared the clinical phenotype of sub threshold fibrosis PV/ET with that manifested by PMF. We applied the IWG-MRT criteria for post-PV/ET MF with the fibrosis component omitted and evaluated for percentage of criteria fulfillment. RESULTS: When IWG-MRT criteria were applied to the PV/ET group, 38/58 (66%) of patients fulfilled criteria for diagnosis of post-PV/ET myelofibrosis except for the 2+ fibrosis requirement. Comparison of sub threshold fibrotic PV/ET clinical phenotype to PMF revealed similar characteristics including heavy symptomatic burden (57% and 52%), presence of splenomegaly (43% and 55%), leukoerythroblastic blood smear (38% and 45%), and median hemoglobin (12.8g/dL and 11.1g/dL). CONCLUSION: MPN progression represents a biological spectrum and definitions of progression in ET/PV may benefit from criteria not restricted by degree of fibrosis.
BACKGROUND: The clinical phenotype of patients with myeloproliferative neoplasms (MPNs) including primary myelofibrosis (PMF), polycythemia vera (PV), and essential thrombocytosis (ET) whom manifest WHO grade 1 marrow fibrosis is poorly defined. Current IWG-MRT criteria require 2+ marrow fibrosis for diagnosis of post PV/ET myelofibrosis (MF). In contrast, the 2008 WHO definition of PMF does not require a minimum fibrosis threshold. METHODS: We retrospectively analyzed the clinical characteristics of 91 MPNpatients with 1+ marrow fibrosis. We compared the clinical phenotype of sub threshold fibrosis PV/ET with that manifested by PMF. We applied the IWG-MRT criteria for post-PV/ET MF with the fibrosis component omitted and evaluated for percentage of criteria fulfillment. RESULTS: When IWG-MRT criteria were applied to the PV/ET group, 38/58 (66%) of patients fulfilled criteria for diagnosis of post-PV/ET myelofibrosis except for the 2+ fibrosis requirement. Comparison of sub threshold fibrotic PV/ET clinical phenotype to PMF revealed similar characteristics including heavy symptomatic burden (57% and 52%), presence of splenomegaly (43% and 55%), leukoerythroblastic blood smear (38% and 45%), and median hemoglobin (12.8g/dL and 11.1g/dL). CONCLUSION:MPN progression represents a biological spectrum and definitions of progression in ET/PV may benefit from criteria not restricted by degree of fibrosis.
Authors: Brady L Stein; Donna M Williams; Christine O'Keefe; Ophelia Rogers; Roxann G Ingersoll; Jerry L Spivak; Amit Verma; Jarek P Maciejewski; Michael A McDevitt; Alison R Moliterno Journal: Haematologica Date: 2011-06-28 Impact factor: 9.941
Authors: John T Reilly; Mary Frances McMullin; Philip A Beer; Nauman Butt; Eibhlean Conneally; Andrew Duncombe; Anthony R Green; N George Michaeel; Marie H Gilleece; Georgina W Hall; Steven Knapper; Adam Mead; Ruben A Mesa; Mallika Sekhar; Bridget Wilkins; Claire N Harrison Journal: Br J Haematol Date: 2012-06-01 Impact factor: 6.998