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Br J Haematol ; 192(6): 988-996, 2021 03.
Article in English | MEDLINE | ID: mdl-32745264

ABSTRACT

The present study assessed the criteria for initiating cytoreduction and response to conventional therapies in 1446 patients with essential thrombocythemia (ET), 267 (17%) of which were CALR-mutated. In low risk patients, time from diagnosis to cytoreduction was shorter in CALR-positive than in the other genotypes (2·8, 3·2, 7·4 and 12·5 years for CALR, MPL, JAK2V617F and TN, respectively, P < 0·0001). A total of 1104 (76%) patients received cytoreductive treatment with hydroxycarbamide (HC) (n = 977), anagrelide (n = 113), or others (n = 14). The estimated cumulative rates of complete haematological response (CR) at 12 months were 40 % and 67% in CALR and JAK2V617F genotypes, respectively. Median time to CR was 192 days for JAK2V617F, 343 for TN, 433 for MPL, and 705 for CALR genotypes (P < 0·0001). Duration of CR was shorter in CALR-mutated ET than in the remaining patients (P = 0·003). In CALR-positive patients, HC and anagrelide had similar efficacy in terms of response rates and duration. CALR-mutated patients developed resistance/intolerance to HC more frequently (5%, 23%, 27% and 15% for JAK2V617F, CALR, MPL and TN, respectively; P < 0·0001). In conclusion, conventional cytoreductive agents are less effective in CALR-mutated ET, highlighting the need for new treatment modalities and redefinition of haematologic targets for patients with this genotype.


Subject(s)
Calreticulin/genetics , Genotype , Hydroxyurea/administration & dosage , Mutation, Missense , Quinazolines/administration & dosage , Registries , Thrombocythemia, Essential , Adolescent , Adult , Aged , Aged, 80 and over , Amino Acid Substitution , Child , Female , Follow-Up Studies , Humans , Janus Kinase 2/genetics , Male , Middle Aged , Spain , Thrombocythemia, Essential/drug therapy , Thrombocythemia, Essential/genetics
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