PREGNANCY IN PAROXYSMAL NOCTURNAL HEMOGLOBINURIA: OUTCOMES DEPENDING ON THE THERAPEUTIC APPROACH
(Abstract release date: 05/19/16)
EHA Library. Vinogradova M. 06/09/16; 134994; PB2094
Dr. Maria Vinogradova
Contributions
Contributions
Abstract
Abstract: PB2094
Type: Publication Only
Background
The management of paroxysmal nocturnal hemoglobinuria (PNH) during pregnancy recently has been challenging because of a high risk of severe maternal and fetal complications. Now pregnancy planning and eculizumab treatment changed the prognosis and made it possible to minimize complications associated with pregnancy in PNH.
Aims
To analyze the pregnancy outcomes in PNH depending on the therapeutic approach- using eculizumab or symptomatic treatment only. Establishment of effective and safe algorithms for the management of pregnancy, delivery and postpartum period in PNH patients is crucial to their quality of life.
Methods
From 1999 to 2015, we have analyzed 17 pregnancies in eight women with PNH, treated in our centers. Only three patients had planned the pregnancy, most of cases- 14 (82,4%) were unplanned. The median age at the start of pregnancy - 25 years (21-34). All of them were diagnosed with PNH following treatment for aplastic anemia (AA) with antithymocyte globulin, cyclosporine A and splenectomy in two cases before pregnancy. Most patients were in partial remission of AA at the time of pregnancy- 8 (47,1%), complete remission was achieved in 6 (35,3%). Three patients (17,6%) from 2013 exposed to eculizumab: two- started the treatment before conceiving, one- received eculizumab from third trimester. Other women received only symptomatic therapy (82,3%), such as anticoagulation with low molecular weight heparin in 29,4%, erythrocytes or platelets transfusion- 35,3%, immunosupressive therapy- 17,6%.
Results
The median of PNH granulocyte clone at the start of pregnancy was 74,7% (17,8-94,1). Progression of aplasia observed during 23,5% pregnancies, but it was not severe and special treatment delayed until the completion of pregnancy. No thrombotic events during pregnancy and postpartum have been observed. There were pregnancy complications: abortion threat 76,5%, fetal growth retardation syndrome 3/9, preeclampsia 2/9. Pregnancies resulted in the birth of healthy infants in 9 (52,9%) cases - three girls and six boys. There were no adverse effects in the newborns from PNH patients both on eculizumab and without it. Newborn health status differed from the norm only in the group of patients without the targeted therapy due to the presence of complications, mainly related to prematurity. Successful outcomes were in 3/3 pregnancies on eculizumab treatment and in 6 (42,9%) cases without the drug. Caesarean sections were performed in all of births, early surgical delivery (30-34 weeks)– in 4/9 cases (preeclampsia-2, placenta previa- 1, breakthrough hemolysis-1). Adverse pregnancy outcomes occurred only in patients not receiving eculizumab and amounted to 8/14 (57,1%). 28,6% cases of pregnancy in the midst of illness required the abortion for medical reasons. Spontaneous miscarriage was registered in 3 (21,4%) patients, fetal death on 27th gestation week- in one case (7,1%). Transfusion requirements increased in two pregnancies (14,3%) with symptomatic therapy, but did not increase on eculizumab. PNH granulocyte clone size decreased in 2/3 cases of eculizumab treatment during pregnancy.
Conclusion
Pregnancy in PNH with symptomatic treatment with a high probability ends adversely. The risk of complications during pregnancy and postpartum in PNH may be minimized by pregnancy planning and applying the management algorithm with eculizumab treatment. Despite the small number of observations, we can conclude that pregnancy outcomes in PNH patients with eculizumab are better than with symptomatic therapy. Our experience confirms that eculizumab can be safely used in PNH during pregnancy. This therapeutic approach may result in improved quality of life in PNH.
Session topic: E-poster
Keyword(s): Paroxysmal nocturnal hemoglobinuria (PNH), Pregnancy
Type: Publication Only
Background
The management of paroxysmal nocturnal hemoglobinuria (PNH) during pregnancy recently has been challenging because of a high risk of severe maternal and fetal complications. Now pregnancy planning and eculizumab treatment changed the prognosis and made it possible to minimize complications associated with pregnancy in PNH.
Aims
To analyze the pregnancy outcomes in PNH depending on the therapeutic approach- using eculizumab or symptomatic treatment only. Establishment of effective and safe algorithms for the management of pregnancy, delivery and postpartum period in PNH patients is crucial to their quality of life.
Methods
From 1999 to 2015, we have analyzed 17 pregnancies in eight women with PNH, treated in our centers. Only three patients had planned the pregnancy, most of cases- 14 (82,4%) were unplanned. The median age at the start of pregnancy - 25 years (21-34). All of them were diagnosed with PNH following treatment for aplastic anemia (AA) with antithymocyte globulin, cyclosporine A and splenectomy in two cases before pregnancy. Most patients were in partial remission of AA at the time of pregnancy- 8 (47,1%), complete remission was achieved in 6 (35,3%). Three patients (17,6%) from 2013 exposed to eculizumab: two- started the treatment before conceiving, one- received eculizumab from third trimester. Other women received only symptomatic therapy (82,3%), such as anticoagulation with low molecular weight heparin in 29,4%, erythrocytes or platelets transfusion- 35,3%, immunosupressive therapy- 17,6%.
Results
The median of PNH granulocyte clone at the start of pregnancy was 74,7% (17,8-94,1). Progression of aplasia observed during 23,5% pregnancies, but it was not severe and special treatment delayed until the completion of pregnancy. No thrombotic events during pregnancy and postpartum have been observed. There were pregnancy complications: abortion threat 76,5%, fetal growth retardation syndrome 3/9, preeclampsia 2/9. Pregnancies resulted in the birth of healthy infants in 9 (52,9%) cases - three girls and six boys. There were no adverse effects in the newborns from PNH patients both on eculizumab and without it. Newborn health status differed from the norm only in the group of patients without the targeted therapy due to the presence of complications, mainly related to prematurity. Successful outcomes were in 3/3 pregnancies on eculizumab treatment and in 6 (42,9%) cases without the drug. Caesarean sections were performed in all of births, early surgical delivery (30-34 weeks)– in 4/9 cases (preeclampsia-2, placenta previa- 1, breakthrough hemolysis-1). Adverse pregnancy outcomes occurred only in patients not receiving eculizumab and amounted to 8/14 (57,1%). 28,6% cases of pregnancy in the midst of illness required the abortion for medical reasons. Spontaneous miscarriage was registered in 3 (21,4%) patients, fetal death on 27th gestation week- in one case (7,1%). Transfusion requirements increased in two pregnancies (14,3%) with symptomatic therapy, but did not increase on eculizumab. PNH granulocyte clone size decreased in 2/3 cases of eculizumab treatment during pregnancy.
Conclusion
Pregnancy in PNH with symptomatic treatment with a high probability ends adversely. The risk of complications during pregnancy and postpartum in PNH may be minimized by pregnancy planning and applying the management algorithm with eculizumab treatment. Despite the small number of observations, we can conclude that pregnancy outcomes in PNH patients with eculizumab are better than with symptomatic therapy. Our experience confirms that eculizumab can be safely used in PNH during pregnancy. This therapeutic approach may result in improved quality of life in PNH.
Session topic: E-poster
Keyword(s): Paroxysmal nocturnal hemoglobinuria (PNH), Pregnancy
Abstract: PB2094
Type: Publication Only
Background
The management of paroxysmal nocturnal hemoglobinuria (PNH) during pregnancy recently has been challenging because of a high risk of severe maternal and fetal complications. Now pregnancy planning and eculizumab treatment changed the prognosis and made it possible to minimize complications associated with pregnancy in PNH.
Aims
To analyze the pregnancy outcomes in PNH depending on the therapeutic approach- using eculizumab or symptomatic treatment only. Establishment of effective and safe algorithms for the management of pregnancy, delivery and postpartum period in PNH patients is crucial to their quality of life.
Methods
From 1999 to 2015, we have analyzed 17 pregnancies in eight women with PNH, treated in our centers. Only three patients had planned the pregnancy, most of cases- 14 (82,4%) were unplanned. The median age at the start of pregnancy - 25 years (21-34). All of them were diagnosed with PNH following treatment for aplastic anemia (AA) with antithymocyte globulin, cyclosporine A and splenectomy in two cases before pregnancy. Most patients were in partial remission of AA at the time of pregnancy- 8 (47,1%), complete remission was achieved in 6 (35,3%). Three patients (17,6%) from 2013 exposed to eculizumab: two- started the treatment before conceiving, one- received eculizumab from third trimester. Other women received only symptomatic therapy (82,3%), such as anticoagulation with low molecular weight heparin in 29,4%, erythrocytes or platelets transfusion- 35,3%, immunosupressive therapy- 17,6%.
Results
The median of PNH granulocyte clone at the start of pregnancy was 74,7% (17,8-94,1). Progression of aplasia observed during 23,5% pregnancies, but it was not severe and special treatment delayed until the completion of pregnancy. No thrombotic events during pregnancy and postpartum have been observed. There were pregnancy complications: abortion threat 76,5%, fetal growth retardation syndrome 3/9, preeclampsia 2/9. Pregnancies resulted in the birth of healthy infants in 9 (52,9%) cases - three girls and six boys. There were no adverse effects in the newborns from PNH patients both on eculizumab and without it. Newborn health status differed from the norm only in the group of patients without the targeted therapy due to the presence of complications, mainly related to prematurity. Successful outcomes were in 3/3 pregnancies on eculizumab treatment and in 6 (42,9%) cases without the drug. Caesarean sections were performed in all of births, early surgical delivery (30-34 weeks)– in 4/9 cases (preeclampsia-2, placenta previa- 1, breakthrough hemolysis-1). Adverse pregnancy outcomes occurred only in patients not receiving eculizumab and amounted to 8/14 (57,1%). 28,6% cases of pregnancy in the midst of illness required the abortion for medical reasons. Spontaneous miscarriage was registered in 3 (21,4%) patients, fetal death on 27th gestation week- in one case (7,1%). Transfusion requirements increased in two pregnancies (14,3%) with symptomatic therapy, but did not increase on eculizumab. PNH granulocyte clone size decreased in 2/3 cases of eculizumab treatment during pregnancy.
Conclusion
Pregnancy in PNH with symptomatic treatment with a high probability ends adversely. The risk of complications during pregnancy and postpartum in PNH may be minimized by pregnancy planning and applying the management algorithm with eculizumab treatment. Despite the small number of observations, we can conclude that pregnancy outcomes in PNH patients with eculizumab are better than with symptomatic therapy. Our experience confirms that eculizumab can be safely used in PNH during pregnancy. This therapeutic approach may result in improved quality of life in PNH.
Session topic: E-poster
Keyword(s): Paroxysmal nocturnal hemoglobinuria (PNH), Pregnancy
Type: Publication Only
Background
The management of paroxysmal nocturnal hemoglobinuria (PNH) during pregnancy recently has been challenging because of a high risk of severe maternal and fetal complications. Now pregnancy planning and eculizumab treatment changed the prognosis and made it possible to minimize complications associated with pregnancy in PNH.
Aims
To analyze the pregnancy outcomes in PNH depending on the therapeutic approach- using eculizumab or symptomatic treatment only. Establishment of effective and safe algorithms for the management of pregnancy, delivery and postpartum period in PNH patients is crucial to their quality of life.
Methods
From 1999 to 2015, we have analyzed 17 pregnancies in eight women with PNH, treated in our centers. Only three patients had planned the pregnancy, most of cases- 14 (82,4%) were unplanned. The median age at the start of pregnancy - 25 years (21-34). All of them were diagnosed with PNH following treatment for aplastic anemia (AA) with antithymocyte globulin, cyclosporine A and splenectomy in two cases before pregnancy. Most patients were in partial remission of AA at the time of pregnancy- 8 (47,1%), complete remission was achieved in 6 (35,3%). Three patients (17,6%) from 2013 exposed to eculizumab: two- started the treatment before conceiving, one- received eculizumab from third trimester. Other women received only symptomatic therapy (82,3%), such as anticoagulation with low molecular weight heparin in 29,4%, erythrocytes or platelets transfusion- 35,3%, immunosupressive therapy- 17,6%.
Results
The median of PNH granulocyte clone at the start of pregnancy was 74,7% (17,8-94,1). Progression of aplasia observed during 23,5% pregnancies, but it was not severe and special treatment delayed until the completion of pregnancy. No thrombotic events during pregnancy and postpartum have been observed. There were pregnancy complications: abortion threat 76,5%, fetal growth retardation syndrome 3/9, preeclampsia 2/9. Pregnancies resulted in the birth of healthy infants in 9 (52,9%) cases - three girls and six boys. There were no adverse effects in the newborns from PNH patients both on eculizumab and without it. Newborn health status differed from the norm only in the group of patients without the targeted therapy due to the presence of complications, mainly related to prematurity. Successful outcomes were in 3/3 pregnancies on eculizumab treatment and in 6 (42,9%) cases without the drug. Caesarean sections were performed in all of births, early surgical delivery (30-34 weeks)– in 4/9 cases (preeclampsia-2, placenta previa- 1, breakthrough hemolysis-1). Adverse pregnancy outcomes occurred only in patients not receiving eculizumab and amounted to 8/14 (57,1%). 28,6% cases of pregnancy in the midst of illness required the abortion for medical reasons. Spontaneous miscarriage was registered in 3 (21,4%) patients, fetal death on 27th gestation week- in one case (7,1%). Transfusion requirements increased in two pregnancies (14,3%) with symptomatic therapy, but did not increase on eculizumab. PNH granulocyte clone size decreased in 2/3 cases of eculizumab treatment during pregnancy.
Conclusion
Pregnancy in PNH with symptomatic treatment with a high probability ends adversely. The risk of complications during pregnancy and postpartum in PNH may be minimized by pregnancy planning and applying the management algorithm with eculizumab treatment. Despite the small number of observations, we can conclude that pregnancy outcomes in PNH patients with eculizumab are better than with symptomatic therapy. Our experience confirms that eculizumab can be safely used in PNH during pregnancy. This therapeutic approach may result in improved quality of life in PNH.
Session topic: E-poster
Keyword(s): Paroxysmal nocturnal hemoglobinuria (PNH), Pregnancy
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