CLINICAL-BIOLOGICAL PROFILE AND RESPONSE TO IMATINIB IN MYELOID AND LYMPHOID NEOPLASM ASSSOCIATED WITH EOSINOPHILIA AND IDIOPATHIC HYPEREOSINOPHILIC SYMDROME
(Abstract release date: 05/19/16)
EHA Library. Hernandez Mohedo F. 06/09/16; 134917; PB2017
Dr. Francisca Hernandez Mohedo
Contributions
Contributions
Abstract
Abstract: PB2017
Type: Publication Only
Background
Primary eosinophilic diseases are a broad and heterogeneous subgroup of hematological disorders with a very low incidence (SEER database age-adjusted incidence rate 0.036 per 100.000). They have been generally defined as peripheral blood eosinophilia (≥ 1.5 x 109/L), tissue infiltration and end-organ damage. According to the OMS-2008 classification, primary eosinophilic diseases are subdivide in a mayor category of myeloid and lymphoid neoplasms with eosinophilia and abnormalities of PDGFRA/B or FGFR1(MNP-Eo); chronic eosinophilic leukemia not otherwise specified (CEL-NOS), idiopathic hypereosinophilic syndrome (HES) and lymphocytic T variant hypereosinophilia (HES-L)
Aims
The MPN-Eo associated with abnormalities of PDGFRA/B frequently respond to low doses of Imatinib, while is controversial the role of Imatinib in idiopathic HES. In this study we show our series of HES, with the aim of describing the clinical-biological profile and the rate of responses to Imatinib in both subtypes of HES, with and without PDGFRA/B abnormalities
Methods
We performed a retrospective and descriptive analysis in GAMFIN cooperative group (Andaluz philadelphia-negative chronic myeloproliferative neoplasm group) of diagnosed patients with hypereosinophilic disease (OMS-2008 and HDWG Workshop 2010 classification) and differential diagnostic algorithm of Tefferi et al. (Mayo Clin. Proc. 2010; 85:158-164). From 2005 to 2015 clinical and biological profile is analyzed with statistical software IBM SPSS.19
Results
We have analyzed a total of 20 cases of MPN-Eo, with an age-adjusted incidence rate of 0.029/100.000 habitants/year (2014 Andalucía Population Statistic), subdivided according to the OMS-2008 classification of eosinophilic diseases in:7 cases ofMPNs-Eo associated with abnormalities of PDGFRA(6)/PDGFRB(1), 1 case of CEL-NOS(t(8;13)(p12;q12)+21 and FISH FGFR1 positive), 1 HES-L case and 11 cases of idiopathic HES. Our series shows a similar distribution by sex (11M/9F) and a lower age at diagnosis in MPN with PDGFRA/B(41,14 vs 54,6; p=0.044). The most frequent clinical presentation was: constitutional syndrome (60%),lung disease (40%), heart disease (35%:71,4% vs18,2%, 0.049; in PDGFRA/B and HES), skin involvement (35%), bowel damage (25%), rhinitis/asthma (20%), adenopathy (10%) and myalgias(5%); 35% of patients with splenomegaly(71,4% vs 9,1%, p=0.013; in NMP-PDGFRA/B and Idiopathic HES) (p=0,013). Bone marrow karyotype was normal in all but 2 patients: 1 PDGFR-B (5q33) case and another CEL-NOS(8p). 25% cases were initially treated with Imatinib, and another 16 patients (55%) received it in second line. With a median follow-up of 94,33 months in the 6 cases of PDGFRA, : 100% reach early hematological complete response (HCR: 6,20+/-4,60 weeks) and molecular response in 4 evaluable cases by FISH(MCR5+/-4,12 moths), 1 case PDGFRB, after 1 moth of follow-up, reach partial response (PR) and 1 case CEL-NOS(FGFR1+), is treated with low doses of PEG-IFN, but 6 months later progressed to lymphoblastic lymphoma. In the 11 cases of idiopathic HES, with a median follow-up of 28+/-29,16 months, 9 cases received Imatinib and six of them reached CHR (66%), while three patients discontinued the therapy (1: no response, and 2 intolerance). Finally, only three cases of Idiopathic HES treated with hydroxycarbamide reached CHR
Conclusion
The incidence rate of HES, gender and age distribution, and clinical-biological profile, in our community of Andalucía, are similar to that previously described in other series. In the majority of patients with MPN-Eo we must tested a therapy with Imatinib, regardless PDGFRA or PDGFRB abnormalities, because a significant subgroups of patients respond, while responses in patients with PDGFRA or PDGFRB abnormalities are more frequents, deepest and fastest. The prognosis and survival depend on the tissue damage, especially cardiac injury, presents at diagnosis in 71,4% of patients with PDGFRA/B abnormalities, frequently irreversible. This suggests a clear recommendation to treat these types of MPN-Eo early, mainly with Imatinib at diagnosis, in order to avoid cardiac damage
Session topic: E-poster
Keyword(s): Hypereosinophilic syndrome, Imatinib, Myeloproliferative disorder
Type: Publication Only
Background
Primary eosinophilic diseases are a broad and heterogeneous subgroup of hematological disorders with a very low incidence (SEER database age-adjusted incidence rate 0.036 per 100.000). They have been generally defined as peripheral blood eosinophilia (≥ 1.5 x 109/L), tissue infiltration and end-organ damage. According to the OMS-2008 classification, primary eosinophilic diseases are subdivide in a mayor category of myeloid and lymphoid neoplasms with eosinophilia and abnormalities of PDGFRA/B or FGFR1(MNP-Eo); chronic eosinophilic leukemia not otherwise specified (CEL-NOS), idiopathic hypereosinophilic syndrome (HES) and lymphocytic T variant hypereosinophilia (HES-L)
Aims
The MPN-Eo associated with abnormalities of PDGFRA/B frequently respond to low doses of Imatinib, while is controversial the role of Imatinib in idiopathic HES. In this study we show our series of HES, with the aim of describing the clinical-biological profile and the rate of responses to Imatinib in both subtypes of HES, with and without PDGFRA/B abnormalities
Methods
We performed a retrospective and descriptive analysis in GAMFIN cooperative group (Andaluz philadelphia-negative chronic myeloproliferative neoplasm group) of diagnosed patients with hypereosinophilic disease (OMS-2008 and HDWG Workshop 2010 classification) and differential diagnostic algorithm of Tefferi et al. (Mayo Clin. Proc. 2010; 85:158-164). From 2005 to 2015 clinical and biological profile is analyzed with statistical software IBM SPSS.19
Results
We have analyzed a total of 20 cases of MPN-Eo, with an age-adjusted incidence rate of 0.029/100.000 habitants/year (2014 Andalucía Population Statistic), subdivided according to the OMS-2008 classification of eosinophilic diseases in:7 cases ofMPNs-Eo associated with abnormalities of PDGFRA(6)/PDGFRB(1), 1 case of CEL-NOS(t(8;13)(p12;q12)+21 and FISH FGFR1 positive), 1 HES-L case and 11 cases of idiopathic HES. Our series shows a similar distribution by sex (11M/9F) and a lower age at diagnosis in MPN with PDGFRA/B(41,14 vs 54,6; p=0.044). The most frequent clinical presentation was: constitutional syndrome (60%),lung disease (40%), heart disease (35%:71,4% vs18,2%, 0.049; in PDGFRA/B and HES), skin involvement (35%), bowel damage (25%), rhinitis/asthma (20%), adenopathy (10%) and myalgias(5%); 35% of patients with splenomegaly(71,4% vs 9,1%, p=0.013; in NMP-PDGFRA/B and Idiopathic HES) (p=0,013). Bone marrow karyotype was normal in all but 2 patients: 1 PDGFR-B (5q33) case and another CEL-NOS(8p). 25% cases were initially treated with Imatinib, and another 16 patients (55%) received it in second line. With a median follow-up of 94,33 months in the 6 cases of PDGFRA, : 100% reach early hematological complete response (HCR: 6,20+/-4,60 weeks) and molecular response in 4 evaluable cases by FISH(MCR5+/-4,12 moths), 1 case PDGFRB, after 1 moth of follow-up, reach partial response (PR) and 1 case CEL-NOS(FGFR1+), is treated with low doses of PEG-IFN, but 6 months later progressed to lymphoblastic lymphoma. In the 11 cases of idiopathic HES, with a median follow-up of 28+/-29,16 months, 9 cases received Imatinib and six of them reached CHR (66%), while three patients discontinued the therapy (1: no response, and 2 intolerance). Finally, only three cases of Idiopathic HES treated with hydroxycarbamide reached CHR
Conclusion
The incidence rate of HES, gender and age distribution, and clinical-biological profile, in our community of Andalucía, are similar to that previously described in other series. In the majority of patients with MPN-Eo we must tested a therapy with Imatinib, regardless PDGFRA or PDGFRB abnormalities, because a significant subgroups of patients respond, while responses in patients with PDGFRA or PDGFRB abnormalities are more frequents, deepest and fastest. The prognosis and survival depend on the tissue damage, especially cardiac injury, presents at diagnosis in 71,4% of patients with PDGFRA/B abnormalities, frequently irreversible. This suggests a clear recommendation to treat these types of MPN-Eo early, mainly with Imatinib at diagnosis, in order to avoid cardiac damage
Session topic: E-poster
Keyword(s): Hypereosinophilic syndrome, Imatinib, Myeloproliferative disorder
Abstract: PB2017
Type: Publication Only
Background
Primary eosinophilic diseases are a broad and heterogeneous subgroup of hematological disorders with a very low incidence (SEER database age-adjusted incidence rate 0.036 per 100.000). They have been generally defined as peripheral blood eosinophilia (≥ 1.5 x 109/L), tissue infiltration and end-organ damage. According to the OMS-2008 classification, primary eosinophilic diseases are subdivide in a mayor category of myeloid and lymphoid neoplasms with eosinophilia and abnormalities of PDGFRA/B or FGFR1(MNP-Eo); chronic eosinophilic leukemia not otherwise specified (CEL-NOS), idiopathic hypereosinophilic syndrome (HES) and lymphocytic T variant hypereosinophilia (HES-L)
Aims
The MPN-Eo associated with abnormalities of PDGFRA/B frequently respond to low doses of Imatinib, while is controversial the role of Imatinib in idiopathic HES. In this study we show our series of HES, with the aim of describing the clinical-biological profile and the rate of responses to Imatinib in both subtypes of HES, with and without PDGFRA/B abnormalities
Methods
We performed a retrospective and descriptive analysis in GAMFIN cooperative group (Andaluz philadelphia-negative chronic myeloproliferative neoplasm group) of diagnosed patients with hypereosinophilic disease (OMS-2008 and HDWG Workshop 2010 classification) and differential diagnostic algorithm of Tefferi et al. (Mayo Clin. Proc. 2010; 85:158-164). From 2005 to 2015 clinical and biological profile is analyzed with statistical software IBM SPSS.19
Results
We have analyzed a total of 20 cases of MPN-Eo, with an age-adjusted incidence rate of 0.029/100.000 habitants/year (2014 Andalucía Population Statistic), subdivided according to the OMS-2008 classification of eosinophilic diseases in:7 cases ofMPNs-Eo associated with abnormalities of PDGFRA(6)/PDGFRB(1), 1 case of CEL-NOS(t(8;13)(p12;q12)+21 and FISH FGFR1 positive), 1 HES-L case and 11 cases of idiopathic HES. Our series shows a similar distribution by sex (11M/9F) and a lower age at diagnosis in MPN with PDGFRA/B(41,14 vs 54,6; p=0.044). The most frequent clinical presentation was: constitutional syndrome (60%),lung disease (40%), heart disease (35%:71,4% vs18,2%, 0.049; in PDGFRA/B and HES), skin involvement (35%), bowel damage (25%), rhinitis/asthma (20%), adenopathy (10%) and myalgias(5%); 35% of patients with splenomegaly(71,4% vs 9,1%, p=0.013; in NMP-PDGFRA/B and Idiopathic HES) (p=0,013). Bone marrow karyotype was normal in all but 2 patients: 1 PDGFR-B (5q33) case and another CEL-NOS(8p). 25% cases were initially treated with Imatinib, and another 16 patients (55%) received it in second line. With a median follow-up of 94,33 months in the 6 cases of PDGFRA, : 100% reach early hematological complete response (HCR: 6,20+/-4,60 weeks) and molecular response in 4 evaluable cases by FISH(MCR5+/-4,12 moths), 1 case PDGFRB, after 1 moth of follow-up, reach partial response (PR) and 1 case CEL-NOS(FGFR1+), is treated with low doses of PEG-IFN, but 6 months later progressed to lymphoblastic lymphoma. In the 11 cases of idiopathic HES, with a median follow-up of 28+/-29,16 months, 9 cases received Imatinib and six of them reached CHR (66%), while three patients discontinued the therapy (1: no response, and 2 intolerance). Finally, only three cases of Idiopathic HES treated with hydroxycarbamide reached CHR
Conclusion
The incidence rate of HES, gender and age distribution, and clinical-biological profile, in our community of Andalucía, are similar to that previously described in other series. In the majority of patients with MPN-Eo we must tested a therapy with Imatinib, regardless PDGFRA or PDGFRB abnormalities, because a significant subgroups of patients respond, while responses in patients with PDGFRA or PDGFRB abnormalities are more frequents, deepest and fastest. The prognosis and survival depend on the tissue damage, especially cardiac injury, presents at diagnosis in 71,4% of patients with PDGFRA/B abnormalities, frequently irreversible. This suggests a clear recommendation to treat these types of MPN-Eo early, mainly with Imatinib at diagnosis, in order to avoid cardiac damage
Session topic: E-poster
Keyword(s): Hypereosinophilic syndrome, Imatinib, Myeloproliferative disorder
Type: Publication Only
Background
Primary eosinophilic diseases are a broad and heterogeneous subgroup of hematological disorders with a very low incidence (SEER database age-adjusted incidence rate 0.036 per 100.000). They have been generally defined as peripheral blood eosinophilia (≥ 1.5 x 109/L), tissue infiltration and end-organ damage. According to the OMS-2008 classification, primary eosinophilic diseases are subdivide in a mayor category of myeloid and lymphoid neoplasms with eosinophilia and abnormalities of PDGFRA/B or FGFR1(MNP-Eo); chronic eosinophilic leukemia not otherwise specified (CEL-NOS), idiopathic hypereosinophilic syndrome (HES) and lymphocytic T variant hypereosinophilia (HES-L)
Aims
The MPN-Eo associated with abnormalities of PDGFRA/B frequently respond to low doses of Imatinib, while is controversial the role of Imatinib in idiopathic HES. In this study we show our series of HES, with the aim of describing the clinical-biological profile and the rate of responses to Imatinib in both subtypes of HES, with and without PDGFRA/B abnormalities
Methods
We performed a retrospective and descriptive analysis in GAMFIN cooperative group (Andaluz philadelphia-negative chronic myeloproliferative neoplasm group) of diagnosed patients with hypereosinophilic disease (OMS-2008 and HDWG Workshop 2010 classification) and differential diagnostic algorithm of Tefferi et al. (Mayo Clin. Proc. 2010; 85:158-164). From 2005 to 2015 clinical and biological profile is analyzed with statistical software IBM SPSS.19
Results
We have analyzed a total of 20 cases of MPN-Eo, with an age-adjusted incidence rate of 0.029/100.000 habitants/year (2014 Andalucía Population Statistic), subdivided according to the OMS-2008 classification of eosinophilic diseases in:7 cases ofMPNs-Eo associated with abnormalities of PDGFRA(6)/PDGFRB(1), 1 case of CEL-NOS(t(8;13)(p12;q12)+21 and FISH FGFR1 positive), 1 HES-L case and 11 cases of idiopathic HES. Our series shows a similar distribution by sex (11M/9F) and a lower age at diagnosis in MPN with PDGFRA/B(41,14 vs 54,6; p=0.044). The most frequent clinical presentation was: constitutional syndrome (60%),lung disease (40%), heart disease (35%:71,4% vs18,2%, 0.049; in PDGFRA/B and HES), skin involvement (35%), bowel damage (25%), rhinitis/asthma (20%), adenopathy (10%) and myalgias(5%); 35% of patients with splenomegaly(71,4% vs 9,1%, p=0.013; in NMP-PDGFRA/B and Idiopathic HES) (p=0,013). Bone marrow karyotype was normal in all but 2 patients: 1 PDGFR-B (5q33) case and another CEL-NOS(8p). 25% cases were initially treated with Imatinib, and another 16 patients (55%) received it in second line. With a median follow-up of 94,33 months in the 6 cases of PDGFRA, : 100% reach early hematological complete response (HCR: 6,20+/-4,60 weeks) and molecular response in 4 evaluable cases by FISH(MCR5+/-4,12 moths), 1 case PDGFRB, after 1 moth of follow-up, reach partial response (PR) and 1 case CEL-NOS(FGFR1+), is treated with low doses of PEG-IFN, but 6 months later progressed to lymphoblastic lymphoma. In the 11 cases of idiopathic HES, with a median follow-up of 28+/-29,16 months, 9 cases received Imatinib and six of them reached CHR (66%), while three patients discontinued the therapy (1: no response, and 2 intolerance). Finally, only three cases of Idiopathic HES treated with hydroxycarbamide reached CHR
Conclusion
The incidence rate of HES, gender and age distribution, and clinical-biological profile, in our community of Andalucía, are similar to that previously described in other series. In the majority of patients with MPN-Eo we must tested a therapy with Imatinib, regardless PDGFRA or PDGFRB abnormalities, because a significant subgroups of patients respond, while responses in patients with PDGFRA or PDGFRB abnormalities are more frequents, deepest and fastest. The prognosis and survival depend on the tissue damage, especially cardiac injury, presents at diagnosis in 71,4% of patients with PDGFRA/B abnormalities, frequently irreversible. This suggests a clear recommendation to treat these types of MPN-Eo early, mainly with Imatinib at diagnosis, in order to avoid cardiac damage
Session topic: E-poster
Keyword(s): Hypereosinophilic syndrome, Imatinib, Myeloproliferative disorder
{{ help_message }}
{{filter}}