Essential thrombocythaemia with mutation in MPL: Clinicopathological correlation and comparison with JAK 2V617F-mutated and CALR- mutated genotypes

Alberto Alvarez-Larran, Daniel Martínez, Leonor Arenillas, Ariadna Rubio, Eduardo Arellano-Rodrigo, Juan Carlos Hernández Boluda, Natalia Papaleo, Gonzalo Caballero, Clara Martínez, Francisca Ferrer-Marín, María Isabel Mata, Manuel Pérez-Encinas, María Antonia Durán, José María Alonso, Gonzalo Carreño-Tarragona, Juan Manuel Alonso, Soledad Noya, Elena Magro, Raúl Pérez, Mónica López-GuerraIrene Pastor-Galán, Francisco Cervantes, Carlos Besses, Luis Colomo, María Rozman

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    © 2018 Article author(s) (or their employer(s) unless otherwise stated in the text of the article). All rights reserved. Aim To characterise the clinical and histological features of MPL-mutated essential thrombocythaemia (ET). Patients and methods Bone marrow biopsies of 175 patients with ET were centrally reviewed according to the 2016 WHO classification, including 42 cases with MPL mutation, 98 JAK2V617F-mutated and 35 CALR-mutated. Clinical and histological features were compared among the three genotypes included in the current 2016 WHO classification and among the different types of MPL mutations. Results Patients with MPL-mutated ET were significantly older than those with the other genotypes. Haematological values at diagnosis were similar among MPL-mutated and CALR-mutated ET, with both genotypes showing higher platelet counts and lower haemoglobin values than ET with JAK2V617F genotype. In the bone marrow, the median number of megakaryocytes was higher in MPL and CALR than in JAK2V617F genotype (16, 19 and 14 megakaryocytes per ×20 power field, respectively, p=0.004). Histological features of prefibrotic myelofibrosis were rarely observed in MPL genotype, whereas sinusoidal hyperplasia, dense clusters of megakaryocytes and reticulin fibrosis were more frequent in CALR-mutated ET, with 11% of such cases fulfilling WHO 2016 histological criteria of prefibrotic myelofibrosis. With a median follow-up of 3.5 years, no significant differences were seen among genotypes regarding survival, vascular complications or myelofibrotic transformation. There were no significant differences in the clinical data or in the histological characteristics depending on the type of MPL mutation. Conclusion MPL and CALR ET genotypes share clinical and histological characteristics. In contrast to CALR genotype, features of prefibrotic myelofibrosis are uncommon in MPL-mutated ET.
    Original languageEnglish
    Pages (from-to)975-980
    JournalJournal of Clinical Pathology
    Publication statusPublished - 1 Nov 2018


    • diagnostic hematology
    • essential thrombocythemia
    • genetics
    • histopathology
    • myeloproliferative neoplasms


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