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Evidence that prefibrotic myelofibrosis is aligned along a clinical and biological continuum featuring primary

Giovanni Barosi1, Vittorio Rosti, Elisa Bonetti

  • 1Laboratory of Clinical Epidemiology and Centre for the Study of Myelofibrosis, IRCCS Policlinico S. Matteo Foundation, Pavia, Italy. barosig@smatteo.pv.it

Plos One
|April 27, 2012
PubMed
Abstract

Insights

Prefibrotic myelofibrosis (pre-MF) is an indolent form of primary myelofibrosis (PMF). This study shows pre-MF fits into a disease continuum, impacting prognosis and classification of PMF.

Area of Science:

  • Hematology
  • Oncology
  • Pathology

Background:

  • The classification of prefibrotic myelofibrosis (pre-MF) within primary myelofibrosis (PMF) lacks robust supporting evidence.
  • Understanding the relationship between pre-MF and fibrotic PMF is crucial for accurate diagnosis and prognosis.

Purpose of the Study:

  • To investigate if pre-MF represents a distinct entity or lies on a clinical and biological continuum with fibrotic primary myelofibrosis (PMF).
  • To analyze clinical and biological characteristics across different stages of bone marrow fibrosis in PMF patients.

Main Methods:

  • A cohort of 683 consecutive patients diagnosed with PMF according to WHO criteria was analyzed.
  • Patients were categorized into pre-MF, early fibrotic PMF, and advanced fibrotic PMF based on bone marrow fibrosis.
  • Clinical data, laboratory parameters, cytogenetics, and survival outcomes were compared across these categories.

Main Results:

  • Prefibrotic myelofibrosis (pre-MF) exhibited distinct characteristics including female predominance, younger age, higher hemoglobin and platelet counts, and lower white blood cell counts compared to fibrotic PMF.
  • A continuous spectrum was observed with increasing age, circulating CD34+ cells, LDH levels, and chromosomal abnormalities, while hemoglobin, CXCR4 expression, and cholesterol decreased along the continuum of bone marrow fibrosis.
  • Survival varied significantly, with 10-year survival rates of 98% for pre-MF, 81% for early fibrotic PMF, and 56% for advanced fibrotic PMF.

Conclusions:

  • Prefibrotic myelofibrosis (pre-MF) is an indolent presentation of primary myelofibrosis (PMF).
  • The findings support a continuum model for PMF, integrating pre-MF and its fibrotic stages.
  • This perspective necessitates improved prognostic models for primary myelofibrosis to better predict disease progression and outcomes.

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