TY - JOUR
T1 - Chronic myeloid leukaemia with extreme thrombocytosis at presentation: Incidence, clinical findings and outcome
AU - Sora', Federica
AU - Iurlo, Alessandra
AU - Sica, Simona
AU - Latagliata, Roberto
AU - Annunziata, Mario
AU - Galimberti, Sara
AU - Castagnetti, Fausto
AU - Pregno, Patrizia
AU - Sgherza, Nicola
AU - Celesti, Francesca
AU - Bocchia, Monica
AU - Gozzini, Antonella
AU - Fava, Carmen
AU - Cattaneo, Daniele
AU - Crugnola, Monica
AU - Montefusco, Enrico
AU - Mauro, Endri
AU - Capodanno, Isabella
AU - Breccia, Massimo
PY - 2017
Y1 - 2017
N2 - Chronic myeloid leukaemia (CML) usually presents with marked leucocytosis, but rare cases may present with an isolated, marked thrombocytosis, defined as a platelet count >1000 × 109/l (Turakhia et al, 2016).
The natural history and the optimal treatment of this entity are not well characterized. We have previously reported our monocentric experience (Sorà et al, 2014); based on these results, we evaluated the incidence, clinical characteristics and outcome of this CML variant in a large series of patients from 16 different Italian haematological centres.
From January 2002 to December 2015, 87 of 1591 patients with extreme thrombocytosis were identified, with an estimated incidence of 5·5%. Patient characteristics are shown in Table 1. Coagulation tests were available in 59/87 patients (67·8%), of which only three (5%) had an abnormally prolonged activated partial thromboplastin time. These three patients were investigated for the presence of a non-specific inhibitor, such as antiphospholipid antibodies, and the levels of coagulation factors. In one patient, anti-cardiolipin antibodies were also detected and in another patient a protein C deficiency was identified. However, only a few patients had undergone a full investigation for congenital thrombophilia, including F2 (prothrombin) G20210A mutation and F5 R506Q (Factor V Leiden).
AB - Chronic myeloid leukaemia (CML) usually presents with marked leucocytosis, but rare cases may present with an isolated, marked thrombocytosis, defined as a platelet count >1000 × 109/l (Turakhia et al, 2016).
The natural history and the optimal treatment of this entity are not well characterized. We have previously reported our monocentric experience (Sorà et al, 2014); based on these results, we evaluated the incidence, clinical characteristics and outcome of this CML variant in a large series of patients from 16 different Italian haematological centres.
From January 2002 to December 2015, 87 of 1591 patients with extreme thrombocytosis were identified, with an estimated incidence of 5·5%. Patient characteristics are shown in Table 1. Coagulation tests were available in 59/87 patients (67·8%), of which only three (5%) had an abnormally prolonged activated partial thromboplastin time. These three patients were investigated for the presence of a non-specific inhibitor, such as antiphospholipid antibodies, and the levels of coagulation factors. In one patient, anti-cardiolipin antibodies were also detected and in another patient a protein C deficiency was identified. However, only a few patients had undergone a full investigation for congenital thrombophilia, including F2 (prothrombin) G20210A mutation and F5 R506Q (Factor V Leiden).
KW - Chronic myeloid leukaemia
KW - Euro score
KW - Hematology
KW - Sokal score
KW - Thrombo-haemorrhagic risk
KW - Thrombocytosis
KW - Chronic myeloid leukaemia
KW - Euro score
KW - Hematology
KW - Sokal score
KW - Thrombo-haemorrhagic risk
KW - Thrombocytosis
UR - http://hdl.handle.net/10807/101084
UR - http://www.blackwellpublishing.com/journals/bjh
U2 - 10.1111/bjh.14553
DO - 10.1111/bjh.14553
M3 - Article
SN - 0007-1048
SP - 10
EP - 12
JO - British Journal of Haematology
JF - British Journal of Haematology
ER -