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WHO ‐histological criteria for myeloproliferative neoplasms: reproducibility, diagnostic accuracy and correlation with gene mutations and clinical outcomes
Author(s) -
AlvarezLarrán Alberto,
Ancochea Agueda,
García Mar,
Climent Fina,
GarcíaPallarols Francesc,
Angona Anna,
Senín Alicia,
Barranco Carlos,
MartínezAvilés Luz,
Serrano Sergio,
Bellosillo Beatriz,
Besses Carlos
Publication year - 2014
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/bjh.12990
Subject(s) - medicine , myelofibrosis , histology , essential thrombocythemia , concordance , bone marrow , polycythemia vera , pathology , polycythaemia , myeloproliferative neoplasm , gastroenterology
Summary Bone marrow histology is included in the diagnostic criteria of myeloproliferative neoplasms ( MPN s). However, some concerns have emerged about its reproducibility. To evaluate the diagnostic accuracy of histology and to assess its correlation with presence of mutations and clinical outcomes, two pathologists reviewed the bone marrow biopsies corresponding to 211 patients with MPN . Despite the low agreement in the evaluation of individual histopathological characteristics, the concordance among pathologists when establishing the diagnosis was good (Kappa index 0·67). The specificity of histology was 100%, 98·5% and 98% in polycythaemia vera ( PV ), essential thrombocythaemia ( ET ) and primary myelofibrosis ( PMF ), respectively, whereas the sensitivity of histological diagnosis was low in PV and ET (32·5% and 54% respectively) and acceptable in PMF (75%). Thirteen out of 146 (9%) patients with clinical ET were diagnosed as prefibrotic PMF . No histological agreement or MPN otherwise unspecified was more frequently observed in JAK 2 V617F‐positive ET than in CALR ‐mutated cases, whereas megakaryocytic abnormalities and prefibrotic PMF were more frequently observed in CALR ‐mutated ET . In conclusion, histological criteria of MPN have a limited diagnostic accuracy due to low sensitivity. Patients with JAK 2 V617F‐positive MPN have a heterogeneous histology while CALR ‐positive ET is associated with megakaryocyte abnormalities and prefibrotic PMF .