Morphologic and cytogenetic differences between post-polycythemic myelofibrosis and primary myelofibrosis in fibrotic stage

Leonardo Boiocchi, Susan Mathew, Umberto Gianelli, Alessandra Iurlo, Tommaso Radice, Sharon Barouk-Fox, Daniel M. Knowles, Attilio Orazi

Research output: Contribution to journalArticle

Abstract

Polycythemia vera and primary myelofibrosis share a propensity to progress toward a myelofibrotic late stage with overlapping clinical characteristics. Bone marrow features potentially useful for distinguishing the two entities have not been thoroughly investigated and, currently, clinical history is used for purposes of disease classification. This study describes in detail the morphologic features of 23 cases of post-polycythemic myelofibrosis and 15 cases of primary myelofibrosis with a similar degree of fibrosis, from two large medical centers. Cytogenetic results were available in 19 post-polycythemic myelofibrosis and in 13 primary myelofibrosis cases. JAK2 status and follow-up information was available in all cases. Cellularity was increased in both groups, but more so in post-polycythemic myelofibrosis than in primary myelofibrosis. In post-polycythemic myelofibrosis, most megakaryocytes retained polycythemia vera-like features including normally folded and/or hyperlobulated nuclei devoid of severe maturation defects; only in a few cases were rare tight clusters present. In primary myelofibrosis cases, megakaryocytes showed pronounced anomalies, including increased nuclear:cytoplasmic ratio, abnormal clumping of chromatin and frequent tight clustering. No differences in blast number (

Original languageEnglish
Pages (from-to)1577-1585
Number of pages9
JournalModern Pathology
Volume26
Issue number12
DOIs
Publication statusPublished - Dec 2013

Keywords

  • Bone marrow biopsy
  • Cytogenetics
  • Myeloproliferative neoplasms
  • Post-polycythemic myelofibrosis
  • Primary myelofibrosis

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

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