Essential thrombocythaemia with mutation in MPL: clinicopathological correlation and comparison with JAK2V617F-mutated and CALR-mutated genotypes
Por:
Alvarez-Larran, A, Martinez, D, Arenillas, L, Rubio, A, Arellano-Rodrigo, E, Boluda, JCH, Papaleo, N, Caballero, G, Martinez, C, Ferrer-Marin, F, Mata, MI, Perez-Encinas, M, Duran, MA, Alonso, JM, Carreno-Tarragona, G, Alonso, JM, Noya, S, Magro, E, Perez, R, Lopez-Guerra, M, Pastor-Galan, I, Cervantes, F, Besses, C, Colomo, L, Rozman, M
Publicada:
1 nov 2018
Resumen:
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 x20 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.
ConclusionMPL and CALR ET genotypes share clinical and histological characteristics. In contrast to CALR genotype, features of prefibrotic myelofibrosis are uncommon in MPL-mutated ET.
Filiaciones:
Alvarez-Larran, A:
Hosp Clin Barcelona, IDIBAPS, Hematol Dept, Barcelona, Spain
Martinez, D:
Hosp Clin Barcelona, IDIBAPS, Pathol Dept, Barcelona, Spain
Arenillas, L:
Univ Autonoma Barcelona, Hosp Mar, Pathol Dept, IMIM, Barcelona, Spain
Rubio, A:
Univ Autonoma Barcelona, Hosp Mar, IMIM, Hematol Dept, Barcelona, Spain
Arellano-Rodrigo, E:
Hosp Clin Barcelona, IDIBAPS, Hemotherapy & Hemostasis Dept, Barcelona, Spain
Boluda, JCH:
Hosp Clin, INCLIVA, Hematol Dept, Valencia, Spain
Papaleo, N:
Univ Autonoma Barcelona, Hosp Mar, Pathol Dept, IMIM, Barcelona, Spain
Caballero, G:
Hosp Miguel Servet, Dept Hematol, Zaragoza, Spain
Martinez, C:
Hosp Santa Creu & Sant Pau, Dept Hematol, Barcelona, Spain
Ferrer-Marin, F:
UCAM, Hosp Morales Messeguer, Hematol & Med Oncol, CIBERER, Murcia, Spain
Mata, MI:
Hosp Costa del Sol, Dept Hematol, Marbella, Spain
Perez-Encinas, M:
Hosp Clin, Dept Hematol, Santiago De Compostela, Spain
Duran, MA:
Hosp Son Espases, Dept Hematol, Palma De Mallorca, Spain
Alonso, JM:
Hosp Palencia, Dept Hematol, Palencia, Spain
Carreno-Tarragona, G:
Hosp 12 Octubre, Dept Hematol, Madrid, Spain
Alonso, JM:
Fdn Jimenez Diaz, Dept Hematol, Madrid, Spain
Noya, S:
Complexo Hosp, Dept Hematol, La Coruna, Spain
Magro, E:
Hosp Principe de Asturias, Dept Hematol, Alcala De Henares, Spain
Perez, R:
Hosp Virgen Arrixaca, Dept Hematol, Murcia, Spain
Lopez-Guerra, M:
Hosp Clin Barcelona, IDIBAPS, Pathol Dept, Barcelona, Spain
Pastor-Galan, I:
Hosp Clin, INCLIVA, Hematol Dept, Valencia, Spain
Cervantes, F:
Hosp Clin Barcelona, IDIBAPS, Hematol Dept, Barcelona, Spain
Besses, C:
Univ Autonoma Barcelona, Hosp Mar, IMIM, Hematol Dept, Barcelona, Spain
Colomo, L:
Univ Autonoma Barcelona, Hosp Mar, Pathol Dept, IMIM, Barcelona, Spain
Rozman, M:
Hosp Clin Barcelona, IDIBAPS, Pathol Dept, Barcelona, Spain
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