CHRONIC MYELOID LEUKEMIA WITH EXTREME THROMBOCYTOCIS AT THE DIAGNOSIS: A NEW SUBSET
(Abstract release date: 05/19/16)
EHA Library. Sora F. 06/09/16; 132649; E1100
Dr. Federica Sora
Contributions
Contributions
Abstract
Abstract: E1100
Type: Eposter Presentation
Background
Chronic myeloid leukemia (CML) is the result of t(9;22) balanced translocation and/or presence of BCR-ABL1 fusion gene. Usually CML presents with marked leukocytosis and only in rare cases CML can present at the diagnosis with an isolated and marked thrombocytosis, defined as platelet count >1000 x 109/L. There are no data on the optimal treatment of these subcategories of “theoretically high risk” patients in the era of tyrosine kinase inhibitors (TKIs).
Aims
To identify a specific subset of CML chronic phase patients with extreme thrombocytosis (platelet count >1000 x 109/L) at diagnosis and to assess clinical outcomes: overall survival, progression free survival and cumulative response in terms of CCyR and/or MMR at 12 and 24 months
Methods
Data were collected and evaluated through the analysis of 1591 CML CP patients registered in 16 Italian Haematological Departments from January 2002 to December 2015
Results
The incidence of extreme thrombocytosis at diagnosis was 5,4% (87/1591). Median age was 59 years (range 18-87), F/M 63/24. Median spleen size below the costal margin was 0 cm (range 0-15), and liver size below the costal margin was 0 cm (range 0-3). Median white blood cell count was 31.600 x 106/microl(range 6.340-390.000), median haemoglobin level was 11,8 g/dl (range 7,7-16,3), and median platelet count was 1466 x 109/L. (range 1054-4720 ). Sokal score was low in 6 patients (6,8%), intermediate in 23 (26,4%) and high in 55 (63,2%). Hasford score was low in 17 patients (22%), intermediate in 22 (28,6%) and high in 38(49,42%) out of 77 evaluable cases. Coagulation tests were available in 59/87 (67,8%), and aPTT was abnormal in only three patients (5%). Bleeding time test was available only in 5 patients and was prolonged in all of them (median value 41 sec1” ). Iron assessment was reported in 56 out of 87 patients and was consistent with iron deficiency in 7 patients (12,5%); assessment of the , JAK2V617F mutation was performed in 43 (49,4%) patients. At diagnosis, 9 out of 87 (10.3%) patients showed haemorragic or thrombotic complications, with only 3 of grade>2 according to the NCI-CTC.In order to reduce severe thrombocytosis, 73/87 patients (83%) received hydroxyurea before starting TKI and in two patients thrombocytapheresis was reported[s1] . As first line TKI 63/87 (72,4%) patients received imatinib, 16 (18,4%) dasatinib and 8 (9,2%) nilotinib.Seventy-seven out of 87 patients were evaluable after 12 months of treatment, and 67 of 77 (87%) obtained cCyR or major molecular response (MMR). Sixty-seven patients were evaluable at 24 months of treatment and 51 out of 67 (76,1%) were in cCyR and/or MMR. After a median follow-up of 66 months (range 3-179), 81/87 (93,1%) patients are still alive. Six patients (6,9%) died: two from myocardial infarction, three from concomitant neoplasia, and one from GvHD.Nine (10,3%) patients developed primary resistance to imatinib and they were shifted to the second line. Eight additional patients developed secondary resistance, mainly after imatinib
Conclusion
The incidence of extreme thrombocytosis in CML chronic phase is rare;this subset of patients shows the following peculiarities: predominance of female sex, high or intermediate risk score with a favourable outcome in terms of both cumulative response rate and survival, low incidence of thrombotic/haemorragic risk[s1] . Due to the inclusion of patients prior to the acquisition of JAK2 V617F in the differential diagnosis of thrombocytosis, only half of the patients were assessed for this mutation
Session topic: E-poster
Keyword(s): Chronic myeloid leukemia, Thrombocytosis
Type: Eposter Presentation
Background
Chronic myeloid leukemia (CML) is the result of t(9;22) balanced translocation and/or presence of BCR-ABL1 fusion gene. Usually CML presents with marked leukocytosis and only in rare cases CML can present at the diagnosis with an isolated and marked thrombocytosis, defined as platelet count >1000 x 109/L. There are no data on the optimal treatment of these subcategories of “theoretically high risk” patients in the era of tyrosine kinase inhibitors (TKIs).
Aims
To identify a specific subset of CML chronic phase patients with extreme thrombocytosis (platelet count >1000 x 109/L) at diagnosis and to assess clinical outcomes: overall survival, progression free survival and cumulative response in terms of CCyR and/or MMR at 12 and 24 months
Methods
Data were collected and evaluated through the analysis of 1591 CML CP patients registered in 16 Italian Haematological Departments from January 2002 to December 2015
Results
The incidence of extreme thrombocytosis at diagnosis was 5,4% (87/1591). Median age was 59 years (range 18-87), F/M 63/24. Median spleen size below the costal margin was 0 cm (range 0-15), and liver size below the costal margin was 0 cm (range 0-3). Median white blood cell count was 31.600 x 106/microl(range 6.340-390.000), median haemoglobin level was 11,8 g/dl (range 7,7-16,3), and median platelet count was 1466 x 109/L. (range 1054-4720 ). Sokal score was low in 6 patients (6,8%), intermediate in 23 (26,4%) and high in 55 (63,2%). Hasford score was low in 17 patients (22%), intermediate in 22 (28,6%) and high in 38(49,42%) out of 77 evaluable cases. Coagulation tests were available in 59/87 (67,8%), and aPTT was abnormal in only three patients (5%). Bleeding time test was available only in 5 patients and was prolonged in all of them (median value 41 sec1” ). Iron assessment was reported in 56 out of 87 patients and was consistent with iron deficiency in 7 patients (12,5%); assessment of the , JAK2V617F mutation was performed in 43 (49,4%) patients. At diagnosis, 9 out of 87 (10.3%) patients showed haemorragic or thrombotic complications, with only 3 of grade>2 according to the NCI-CTC.In order to reduce severe thrombocytosis, 73/87 patients (83%) received hydroxyurea before starting TKI and in two patients thrombocytapheresis was reported[s1] . As first line TKI 63/87 (72,4%) patients received imatinib, 16 (18,4%) dasatinib and 8 (9,2%) nilotinib.Seventy-seven out of 87 patients were evaluable after 12 months of treatment, and 67 of 77 (87%) obtained cCyR or major molecular response (MMR). Sixty-seven patients were evaluable at 24 months of treatment and 51 out of 67 (76,1%) were in cCyR and/or MMR. After a median follow-up of 66 months (range 3-179), 81/87 (93,1%) patients are still alive. Six patients (6,9%) died: two from myocardial infarction, three from concomitant neoplasia, and one from GvHD.Nine (10,3%) patients developed primary resistance to imatinib and they were shifted to the second line. Eight additional patients developed secondary resistance, mainly after imatinib
Conclusion
The incidence of extreme thrombocytosis in CML chronic phase is rare;this subset of patients shows the following peculiarities: predominance of female sex, high or intermediate risk score with a favourable outcome in terms of both cumulative response rate and survival, low incidence of thrombotic/haemorragic risk[s1] . Due to the inclusion of patients prior to the acquisition of JAK2 V617F in the differential diagnosis of thrombocytosis, only half of the patients were assessed for this mutation
Session topic: E-poster
Keyword(s): Chronic myeloid leukemia, Thrombocytosis
Abstract: E1100
Type: Eposter Presentation
Background
Chronic myeloid leukemia (CML) is the result of t(9;22) balanced translocation and/or presence of BCR-ABL1 fusion gene. Usually CML presents with marked leukocytosis and only in rare cases CML can present at the diagnosis with an isolated and marked thrombocytosis, defined as platelet count >1000 x 109/L. There are no data on the optimal treatment of these subcategories of “theoretically high risk” patients in the era of tyrosine kinase inhibitors (TKIs).
Aims
To identify a specific subset of CML chronic phase patients with extreme thrombocytosis (platelet count >1000 x 109/L) at diagnosis and to assess clinical outcomes: overall survival, progression free survival and cumulative response in terms of CCyR and/or MMR at 12 and 24 months
Methods
Data were collected and evaluated through the analysis of 1591 CML CP patients registered in 16 Italian Haematological Departments from January 2002 to December 2015
Results
The incidence of extreme thrombocytosis at diagnosis was 5,4% (87/1591). Median age was 59 years (range 18-87), F/M 63/24. Median spleen size below the costal margin was 0 cm (range 0-15), and liver size below the costal margin was 0 cm (range 0-3). Median white blood cell count was 31.600 x 106/microl(range 6.340-390.000), median haemoglobin level was 11,8 g/dl (range 7,7-16,3), and median platelet count was 1466 x 109/L. (range 1054-4720 ). Sokal score was low in 6 patients (6,8%), intermediate in 23 (26,4%) and high in 55 (63,2%). Hasford score was low in 17 patients (22%), intermediate in 22 (28,6%) and high in 38(49,42%) out of 77 evaluable cases. Coagulation tests were available in 59/87 (67,8%), and aPTT was abnormal in only three patients (5%). Bleeding time test was available only in 5 patients and was prolonged in all of them (median value 41 sec1” ). Iron assessment was reported in 56 out of 87 patients and was consistent with iron deficiency in 7 patients (12,5%); assessment of the , JAK2V617F mutation was performed in 43 (49,4%) patients. At diagnosis, 9 out of 87 (10.3%) patients showed haemorragic or thrombotic complications, with only 3 of grade>2 according to the NCI-CTC.In order to reduce severe thrombocytosis, 73/87 patients (83%) received hydroxyurea before starting TKI and in two patients thrombocytapheresis was reported[s1] . As first line TKI 63/87 (72,4%) patients received imatinib, 16 (18,4%) dasatinib and 8 (9,2%) nilotinib.Seventy-seven out of 87 patients were evaluable after 12 months of treatment, and 67 of 77 (87%) obtained cCyR or major molecular response (MMR). Sixty-seven patients were evaluable at 24 months of treatment and 51 out of 67 (76,1%) were in cCyR and/or MMR. After a median follow-up of 66 months (range 3-179), 81/87 (93,1%) patients are still alive. Six patients (6,9%) died: two from myocardial infarction, three from concomitant neoplasia, and one from GvHD.Nine (10,3%) patients developed primary resistance to imatinib and they were shifted to the second line. Eight additional patients developed secondary resistance, mainly after imatinib
Conclusion
The incidence of extreme thrombocytosis in CML chronic phase is rare;this subset of patients shows the following peculiarities: predominance of female sex, high or intermediate risk score with a favourable outcome in terms of both cumulative response rate and survival, low incidence of thrombotic/haemorragic risk[s1] . Due to the inclusion of patients prior to the acquisition of JAK2 V617F in the differential diagnosis of thrombocytosis, only half of the patients were assessed for this mutation
Session topic: E-poster
Keyword(s): Chronic myeloid leukemia, Thrombocytosis
Type: Eposter Presentation
Background
Chronic myeloid leukemia (CML) is the result of t(9;22) balanced translocation and/or presence of BCR-ABL1 fusion gene. Usually CML presents with marked leukocytosis and only in rare cases CML can present at the diagnosis with an isolated and marked thrombocytosis, defined as platelet count >1000 x 109/L. There are no data on the optimal treatment of these subcategories of “theoretically high risk” patients in the era of tyrosine kinase inhibitors (TKIs).
Aims
To identify a specific subset of CML chronic phase patients with extreme thrombocytosis (platelet count >1000 x 109/L) at diagnosis and to assess clinical outcomes: overall survival, progression free survival and cumulative response in terms of CCyR and/or MMR at 12 and 24 months
Methods
Data were collected and evaluated through the analysis of 1591 CML CP patients registered in 16 Italian Haematological Departments from January 2002 to December 2015
Results
The incidence of extreme thrombocytosis at diagnosis was 5,4% (87/1591). Median age was 59 years (range 18-87), F/M 63/24. Median spleen size below the costal margin was 0 cm (range 0-15), and liver size below the costal margin was 0 cm (range 0-3). Median white blood cell count was 31.600 x 106/microl(range 6.340-390.000), median haemoglobin level was 11,8 g/dl (range 7,7-16,3), and median platelet count was 1466 x 109/L. (range 1054-4720 ). Sokal score was low in 6 patients (6,8%), intermediate in 23 (26,4%) and high in 55 (63,2%). Hasford score was low in 17 patients (22%), intermediate in 22 (28,6%) and high in 38(49,42%) out of 77 evaluable cases. Coagulation tests were available in 59/87 (67,8%), and aPTT was abnormal in only three patients (5%). Bleeding time test was available only in 5 patients and was prolonged in all of them (median value 41 sec1” ). Iron assessment was reported in 56 out of 87 patients and was consistent with iron deficiency in 7 patients (12,5%); assessment of the , JAK2V617F mutation was performed in 43 (49,4%) patients. At diagnosis, 9 out of 87 (10.3%) patients showed haemorragic or thrombotic complications, with only 3 of grade>2 according to the NCI-CTC.In order to reduce severe thrombocytosis, 73/87 patients (83%) received hydroxyurea before starting TKI and in two patients thrombocytapheresis was reported[s1] . As first line TKI 63/87 (72,4%) patients received imatinib, 16 (18,4%) dasatinib and 8 (9,2%) nilotinib.Seventy-seven out of 87 patients were evaluable after 12 months of treatment, and 67 of 77 (87%) obtained cCyR or major molecular response (MMR). Sixty-seven patients were evaluable at 24 months of treatment and 51 out of 67 (76,1%) were in cCyR and/or MMR. After a median follow-up of 66 months (range 3-179), 81/87 (93,1%) patients are still alive. Six patients (6,9%) died: two from myocardial infarction, three from concomitant neoplasia, and one from GvHD.Nine (10,3%) patients developed primary resistance to imatinib and they were shifted to the second line. Eight additional patients developed secondary resistance, mainly after imatinib
Conclusion
The incidence of extreme thrombocytosis in CML chronic phase is rare;this subset of patients shows the following peculiarities: predominance of female sex, high or intermediate risk score with a favourable outcome in terms of both cumulative response rate and survival, low incidence of thrombotic/haemorragic risk[s1] . Due to the inclusion of patients prior to the acquisition of JAK2 V617F in the differential diagnosis of thrombocytosis, only half of the patients were assessed for this mutation
Session topic: E-poster
Keyword(s): Chronic myeloid leukemia, Thrombocytosis
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