Bone marrow megakaryocytic activation predicts fibrotic evolution of Philadelphia-negative myeloproliferative neoplasms
| Autor: | Valerio De Stefano, Luigi Maria Larocca, Valentina Ranucci, Maurizio Martini, Mattia Schino, Silvia Betti, Elena Rossi, Monica Di Cecca, Patrizia Chiusolo, Vincenzo Fiorentino |
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| Rok vydání: | 2020 |
| Předmět: |
0301 basic medicine
Pathology medicine.medical_specialty early/prefibrotic primary myelofibrosis Article 03 medical and health sciences 0302 clinical medicine Bone Marrow White blood cell Biopsy Megakaryocytic Activation medicine Humans Platelet Myelofibrosis Polycythemia Vera Retrospective Studies Myeloproliferative Disorders medicine.diagnostic_test Settore MED/08 - ANATOMIA PATOLOGICA Essential thrombocythemia business.industry food and beverages Hematology Janus Kinase 2 medicine.disease Emperipolesis 030104 developmental biology medicine.anatomical_structure 030220 oncology & carcinogenesis polycythemia Mutation Philadelphia-negative chronic myeloproliferative neoplasms Bone marrow Differential diagnosis business |
| Zdroj: | Haematologica |
| ISSN: | 1592-8721 |
| Popis: | Philadelphia-negative chronic myeloproliferative neoplasms (MPN) have been traditionally considered as indistinctly slowly progressing conditions; recent evidence proves that a subset of cases have a rapid evolution, so that MPN prognosis needs to be personalized. We identified a new morphological parameter, defined as megakaryocytic activation (M-ACT) based on the coexistence of megakaryocytic emperipolesis, megakaryocytes (MK) cluster formation and evidence of arrangement of collagen fibers around the perimeter of MK. We retrospectively analyzed the bone marrow biopsy of two MPN cohorts of patients with polycythemia (PV) (n=64) and non-PV patients (including essential thrombocythemia, and early/prefibrotic primary myelofibrosis [PMF]) (n=222). M-ACT showed a significant correlation with splenomegaly, white blood cell count, and lactate dehydrogenase serum levels in both groups, with JAK2 V617F allele burden in PV patients, and with CALR mutations, and platelet count in non-PV patients. Progression-free survival, defined as PV-to-secondary MF progression and non-PV-to-overt PMF, was worse in both PV and early/prefibrotic PMF patients with M-ACT in comparison to those without M-ACT (P |
| Databáze: | OpenAIRE |
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