Alberto Alvarez-Larran 1 , Daniel Martínez 2 , Leonor Arenillas 3 , Ariadna Rubio 4 , Eduardo Arellano-Rodrigo 5 , Juan Carlos Hernández Boluda 6 , Natalia Papaleo 3 , Gonzalo Caballero 7 , Clara Martínez 8 , Francisca Ferrer-Marín 9 , María Isabel Mata 10 , Manuel Pérez-Encinas 11 , María Antonia Durán 12 , José María Alonso 13 , Gonzalo Carreño-Tarragona 14 , Juan Manuel Alonso 15 , Soledad Noya 16 , Elena Magro 17 , Raúl Pérez 18 , Mónica López-Guerra 2 , Irene Pastor-Galán 6 , Francisco Cervantes 1 , Carlos Besses 4 , Luis Colomo 3 , María Rozman 2 Show Affiliations »
Abstract
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. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.
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. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.
Entities: Disease
Gene
Species
Keywords:
diagnostic hematology; essential thrombocythemia; genetics; histopathology; myeloproliferative neoplasms
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Year: 2018
PMID： 29934356 DOI： 10.1136/jclinpath-2018-205227
Source DB: PubMed Journal: J Clin Pathol ISSN： 0021-9746 Impact factor: 3.411