Contributions
Abstract: PB1835
Type: Publication Only
Background
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired clonal stem cell disorder characterized by intravascular hemolysis, cytopenia and thrombophilia. Thromboembolism, infection and premature birth are main reasons for significantly increased maternal and fetal morbidity and mortality during pregnancy and the following post-partum period in PNH patients. Therefore, PNH has been considered a relative contraindication for pregnancy. The terminal complement cascade inhibitor Eculizumab prevents fatal complications and nearly normalizes overall survival in PNH. Consequently, it has become the standard treatment in patients with symptomatic PNH. However, there are limited published data regarding the use of Eculizumab during pregnancy, the postpartum period or, more less, during lactation.
Aims
To evaluate the management of pregnancy in PNH in our institution.
Methods
We report three cases of pregnancy in PNH
Results
A 28 year old PNH patient became pregnant while on Eculizumab and the therapy was continued throughout the whole pregnancy. At diagnosis of her pregnancy (6th week of gestation) anticoagulation therapy with low molecular weight heparin was initiated and continued although no clinical sign of thrombosis was present. She was immediately introduced in an interdisciplinary team for high risk pregnancies consisting of specialists for gynecology, internal medicine, anesthesia and hematology. During the third trimester she developed a breakthrough hemolysis in terms of symptomatic anemia requiring repeated blood transfusions. Hemolysis was successfully controlled by dose escalation of Eculizumab first from 900 mg to 1200 mg biweekly and consequently shortening the administration interval from a bi-weekly to a weekly scheme until the birth of the baby. She successfully delivered a healthy baby at term by natural birth without complications. One month after delivery the patient has returned to her usual therapeutic Eculizumab regimen (900 mg biweekly). She breastfeed her baby for six months without complications. The anticoagulation treatment was continued, as recommended, for the first three months. The baby girl is developing well according to her age and she is now one years old. Currently, we are managing other two young pregnant PNH patients. Our second patients is a 27 years old girl who became pregnant while she was not on Eculizumab but only in follow up due to indolent disease without hemolysis. She was started with anticoagulation prophylaxis with low molecular weight heparin as soon as the pregnancy was noted, and she started the standard therapeutic Eculizumab regimen (900 mg biweekly) from the beginning of second trimester. She is now at the end of the third trimester, fetal growth is regular, no signs of hemolysis on the mother and no thromboembolic complications were noted. The estimated date of delivery is scheduled for the end of March, 2018. The third pregnancy is on a 27 years old PNH patient currently at the 15th week of gestation; she was on Eculizumab standard regimen treatment from 2009 due to breakthrough hemolysis and we are managing her pregnancy according to the policies followed for the first described case.
Conclusion
Our single center experience reports a favorable outcome of a PNH patient who became pregnant while under Eculizumab, supporting the scarce published experience that this drug can be given safely and can even be escalated during pregnancy in PNH patients, with a good disease control. We are currently managing other two pregnant PNH patients, at the moment without complications.
Session topic: 12. Bone marrow failure syndromes incl. PNH - Clinical
Keyword(s): PNH, Pregnancy
Abstract: PB1835
Type: Publication Only
Background
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired clonal stem cell disorder characterized by intravascular hemolysis, cytopenia and thrombophilia. Thromboembolism, infection and premature birth are main reasons for significantly increased maternal and fetal morbidity and mortality during pregnancy and the following post-partum period in PNH patients. Therefore, PNH has been considered a relative contraindication for pregnancy. The terminal complement cascade inhibitor Eculizumab prevents fatal complications and nearly normalizes overall survival in PNH. Consequently, it has become the standard treatment in patients with symptomatic PNH. However, there are limited published data regarding the use of Eculizumab during pregnancy, the postpartum period or, more less, during lactation.
Aims
To evaluate the management of pregnancy in PNH in our institution.
Methods
We report three cases of pregnancy in PNH
Results
A 28 year old PNH patient became pregnant while on Eculizumab and the therapy was continued throughout the whole pregnancy. At diagnosis of her pregnancy (6th week of gestation) anticoagulation therapy with low molecular weight heparin was initiated and continued although no clinical sign of thrombosis was present. She was immediately introduced in an interdisciplinary team for high risk pregnancies consisting of specialists for gynecology, internal medicine, anesthesia and hematology. During the third trimester she developed a breakthrough hemolysis in terms of symptomatic anemia requiring repeated blood transfusions. Hemolysis was successfully controlled by dose escalation of Eculizumab first from 900 mg to 1200 mg biweekly and consequently shortening the administration interval from a bi-weekly to a weekly scheme until the birth of the baby. She successfully delivered a healthy baby at term by natural birth without complications. One month after delivery the patient has returned to her usual therapeutic Eculizumab regimen (900 mg biweekly). She breastfeed her baby for six months without complications. The anticoagulation treatment was continued, as recommended, for the first three months. The baby girl is developing well according to her age and she is now one years old. Currently, we are managing other two young pregnant PNH patients. Our second patients is a 27 years old girl who became pregnant while she was not on Eculizumab but only in follow up due to indolent disease without hemolysis. She was started with anticoagulation prophylaxis with low molecular weight heparin as soon as the pregnancy was noted, and she started the standard therapeutic Eculizumab regimen (900 mg biweekly) from the beginning of second trimester. She is now at the end of the third trimester, fetal growth is regular, no signs of hemolysis on the mother and no thromboembolic complications were noted. The estimated date of delivery is scheduled for the end of March, 2018. The third pregnancy is on a 27 years old PNH patient currently at the 15th week of gestation; she was on Eculizumab standard regimen treatment from 2009 due to breakthrough hemolysis and we are managing her pregnancy according to the policies followed for the first described case.
Conclusion
Our single center experience reports a favorable outcome of a PNH patient who became pregnant while under Eculizumab, supporting the scarce published experience that this drug can be given safely and can even be escalated during pregnancy in PNH patients, with a good disease control. We are currently managing other two pregnant PNH patients, at the moment without complications.
Session topic: 12. Bone marrow failure syndromes incl. PNH - Clinical
Keyword(s): PNH, Pregnancy