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Leuk Res. 2015 Jul;39(7):684-8. doi: 10.1016/j.leukres.2015.04.004. Epub 2015 Apr 17.

Limitations of fibrosis grade as diagnostic criteria for post polycythemia vera and essential thrombocytosis myelofibrosis.

Author information

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.

KEYWORDS:

Essential thrombocytosis; Myelofibrosis; Myeloproliferative; Polycythemia; Primary

PMID:
25922307
DOI:
10.1016/j.leukres.2015.04.004
[Indexed for MEDLINE]

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