PREGNANCY-ASSOCIATED ATYPICAL HEMOLYTIC UREMIC SYNDROME
(Abstract release date: 05/19/16)
EHA Library. Vinogradova M. 06/09/16; 135007; PB2107
Dr. Maria Vinogradova
Contributions
Contributions
Abstract
Abstract: PB2107
Type: Publication Only
Background
Pregnancy-associated atypical hemolytic uremic syndrome (P-aHUS) is a rare catastrophic disorder defined by the occurrence of fibrin and/or platelet thrombi in the microvasculature, resulting in microangiopathic hemolytic anemia (MAHA) and thrombocytopenia without Shiga toxin and ADAMTS13 deficiency. An estimated incidence of P-aHUS is approximately 1 in 25,000 pregnancies during antepartum and puerperium. It is associated with a significant perinatal or maternal morbidity and mortality.
Aims
The evaluation of outcomes in P-aHUS. The establishment of adequate strategies for the timely diagnosis and treatment is very important to improve patient survival and pregnancy outcomes.
Methods
This is a retrospective analysis of aHUS during pregnancy treated in our center and other clinics via telemedicine between November 2010 and February 2016. Nine women mean age 30,4 years (range: 21-36) were diagnosed with aHUS. Most of them (8/9) presented postpartum, 1/9 developed aHUS in II trimester. All had MAHA, thrombocytopenia and severe organ dysfunction. 7/9 had at least one pregnancy before the onset of P-aHUS.
Results
Mean nadir hemoglobin was 60,3 g/L (range 46 – 69); mean nadir platelet count- 53,000/mm3 (range 16,000–100,000); mean peak serum creatinine- 454 mkmol/l (range 67–998), mean peak LDH- 2,953 IU/L (range 996–11,360). 6/9 required hemodialysis and mechanical ventilation. All underwent plasmaexchange or plasma infusion and all of them was appointed unfractionated or low molecular weight heparins. Signs of mild preeclampsia were observed in 8/9 patients with an average of 7 days (3-14) to debut P-aHUS, 4 of them were diagnosed with fetal death on 27-34 gestation weeks. Live births resulted in 5/9 of pregnancies on 28-38 weeks (median 32). Since the onset of the disease state of the patient rapidly deteriorated: acute renal failure was detected in 8/9 patients (6 cases performed hemodialysis), adult respiratory distress syndrome, requiring mechanical ventilation, was diagnosed in 6/9, 6/9 have pankreatit, neurological symptoms were accompanied by observations of 5/9, 2/9 observed in dilated cardiomyopathy and ischemic colitis in one case. The most common organ dysfunction was kidney failure (8/9). 8/9 patients received therapy with fresh frozen plasma. Treatment with eculizumab was started on 4 patients on 5-17 days from the time of P-aHUS debut (median, 7), but the full course was not held anyone: performed from 1 to 3 injections of 900 mg (median 1). All patients received prophylactic anticoagulation. Outcomes as a whole are extremely unfavorable: 5 of 9 patients died (3/5 causes of death-septic complications, thrombosis- 1, hemorrhage- 1), 2/9 - reached end-stage renal failure by 1 month, 2/9 gained the renal function impairment (CKD3-4) by last follow-up.
Conclusion
P-aHUS is a severe life-threatening disorder associated with a significant perinatal and maternal morbidity and mortality. We evaluated the outcomes of P-aHUS. Currently, they are extremely pessimistic now. Diagnostic and therapeutic approaches for this disease requires further study in order to optimize results. Clinical suspicion, early identification and effective approaches including target therapy can improve the pregnancy outcomes and prognosis in general.
Session topic: E-poster
Keyword(s): Pregnancy, Thrombotic microangiopathy
Type: Publication Only
Background
Pregnancy-associated atypical hemolytic uremic syndrome (P-aHUS) is a rare catastrophic disorder defined by the occurrence of fibrin and/or platelet thrombi in the microvasculature, resulting in microangiopathic hemolytic anemia (MAHA) and thrombocytopenia without Shiga toxin and ADAMTS13 deficiency. An estimated incidence of P-aHUS is approximately 1 in 25,000 pregnancies during antepartum and puerperium. It is associated with a significant perinatal or maternal morbidity and mortality.
Aims
The evaluation of outcomes in P-aHUS. The establishment of adequate strategies for the timely diagnosis and treatment is very important to improve patient survival and pregnancy outcomes.
Methods
This is a retrospective analysis of aHUS during pregnancy treated in our center and other clinics via telemedicine between November 2010 and February 2016. Nine women mean age 30,4 years (range: 21-36) were diagnosed with aHUS. Most of them (8/9) presented postpartum, 1/9 developed aHUS in II trimester. All had MAHA, thrombocytopenia and severe organ dysfunction. 7/9 had at least one pregnancy before the onset of P-aHUS.
Results
Mean nadir hemoglobin was 60,3 g/L (range 46 – 69); mean nadir platelet count- 53,000/mm3 (range 16,000–100,000); mean peak serum creatinine- 454 mkmol/l (range 67–998), mean peak LDH- 2,953 IU/L (range 996–11,360). 6/9 required hemodialysis and mechanical ventilation. All underwent plasmaexchange or plasma infusion and all of them was appointed unfractionated or low molecular weight heparins. Signs of mild preeclampsia were observed in 8/9 patients with an average of 7 days (3-14) to debut P-aHUS, 4 of them were diagnosed with fetal death on 27-34 gestation weeks. Live births resulted in 5/9 of pregnancies on 28-38 weeks (median 32). Since the onset of the disease state of the patient rapidly deteriorated: acute renal failure was detected in 8/9 patients (6 cases performed hemodialysis), adult respiratory distress syndrome, requiring mechanical ventilation, was diagnosed in 6/9, 6/9 have pankreatit, neurological symptoms were accompanied by observations of 5/9, 2/9 observed in dilated cardiomyopathy and ischemic colitis in one case. The most common organ dysfunction was kidney failure (8/9). 8/9 patients received therapy with fresh frozen plasma. Treatment with eculizumab was started on 4 patients on 5-17 days from the time of P-aHUS debut (median, 7), but the full course was not held anyone: performed from 1 to 3 injections of 900 mg (median 1). All patients received prophylactic anticoagulation. Outcomes as a whole are extremely unfavorable: 5 of 9 patients died (3/5 causes of death-septic complications, thrombosis- 1, hemorrhage- 1), 2/9 - reached end-stage renal failure by 1 month, 2/9 gained the renal function impairment (CKD3-4) by last follow-up.
Conclusion
P-aHUS is a severe life-threatening disorder associated with a significant perinatal and maternal morbidity and mortality. We evaluated the outcomes of P-aHUS. Currently, they are extremely pessimistic now. Diagnostic and therapeutic approaches for this disease requires further study in order to optimize results. Clinical suspicion, early identification and effective approaches including target therapy can improve the pregnancy outcomes and prognosis in general.
Session topic: E-poster
Keyword(s): Pregnancy, Thrombotic microangiopathy
Abstract: PB2107
Type: Publication Only
Background
Pregnancy-associated atypical hemolytic uremic syndrome (P-aHUS) is a rare catastrophic disorder defined by the occurrence of fibrin and/or platelet thrombi in the microvasculature, resulting in microangiopathic hemolytic anemia (MAHA) and thrombocytopenia without Shiga toxin and ADAMTS13 deficiency. An estimated incidence of P-aHUS is approximately 1 in 25,000 pregnancies during antepartum and puerperium. It is associated with a significant perinatal or maternal morbidity and mortality.
Aims
The evaluation of outcomes in P-aHUS. The establishment of adequate strategies for the timely diagnosis and treatment is very important to improve patient survival and pregnancy outcomes.
Methods
This is a retrospective analysis of aHUS during pregnancy treated in our center and other clinics via telemedicine between November 2010 and February 2016. Nine women mean age 30,4 years (range: 21-36) were diagnosed with aHUS. Most of them (8/9) presented postpartum, 1/9 developed aHUS in II trimester. All had MAHA, thrombocytopenia and severe organ dysfunction. 7/9 had at least one pregnancy before the onset of P-aHUS.
Results
Mean nadir hemoglobin was 60,3 g/L (range 46 – 69); mean nadir platelet count- 53,000/mm3 (range 16,000–100,000); mean peak serum creatinine- 454 mkmol/l (range 67–998), mean peak LDH- 2,953 IU/L (range 996–11,360). 6/9 required hemodialysis and mechanical ventilation. All underwent plasmaexchange or plasma infusion and all of them was appointed unfractionated or low molecular weight heparins. Signs of mild preeclampsia were observed in 8/9 patients with an average of 7 days (3-14) to debut P-aHUS, 4 of them were diagnosed with fetal death on 27-34 gestation weeks. Live births resulted in 5/9 of pregnancies on 28-38 weeks (median 32). Since the onset of the disease state of the patient rapidly deteriorated: acute renal failure was detected in 8/9 patients (6 cases performed hemodialysis), adult respiratory distress syndrome, requiring mechanical ventilation, was diagnosed in 6/9, 6/9 have pankreatit, neurological symptoms were accompanied by observations of 5/9, 2/9 observed in dilated cardiomyopathy and ischemic colitis in one case. The most common organ dysfunction was kidney failure (8/9). 8/9 patients received therapy with fresh frozen plasma. Treatment with eculizumab was started on 4 patients on 5-17 days from the time of P-aHUS debut (median, 7), but the full course was not held anyone: performed from 1 to 3 injections of 900 mg (median 1). All patients received prophylactic anticoagulation. Outcomes as a whole are extremely unfavorable: 5 of 9 patients died (3/5 causes of death-septic complications, thrombosis- 1, hemorrhage- 1), 2/9 - reached end-stage renal failure by 1 month, 2/9 gained the renal function impairment (CKD3-4) by last follow-up.
Conclusion
P-aHUS is a severe life-threatening disorder associated with a significant perinatal and maternal morbidity and mortality. We evaluated the outcomes of P-aHUS. Currently, they are extremely pessimistic now. Diagnostic and therapeutic approaches for this disease requires further study in order to optimize results. Clinical suspicion, early identification and effective approaches including target therapy can improve the pregnancy outcomes and prognosis in general.
Session topic: E-poster
Keyword(s): Pregnancy, Thrombotic microangiopathy
Type: Publication Only
Background
Pregnancy-associated atypical hemolytic uremic syndrome (P-aHUS) is a rare catastrophic disorder defined by the occurrence of fibrin and/or platelet thrombi in the microvasculature, resulting in microangiopathic hemolytic anemia (MAHA) and thrombocytopenia without Shiga toxin and ADAMTS13 deficiency. An estimated incidence of P-aHUS is approximately 1 in 25,000 pregnancies during antepartum and puerperium. It is associated with a significant perinatal or maternal morbidity and mortality.
Aims
The evaluation of outcomes in P-aHUS. The establishment of adequate strategies for the timely diagnosis and treatment is very important to improve patient survival and pregnancy outcomes.
Methods
This is a retrospective analysis of aHUS during pregnancy treated in our center and other clinics via telemedicine between November 2010 and February 2016. Nine women mean age 30,4 years (range: 21-36) were diagnosed with aHUS. Most of them (8/9) presented postpartum, 1/9 developed aHUS in II trimester. All had MAHA, thrombocytopenia and severe organ dysfunction. 7/9 had at least one pregnancy before the onset of P-aHUS.
Results
Mean nadir hemoglobin was 60,3 g/L (range 46 – 69); mean nadir platelet count- 53,000/mm3 (range 16,000–100,000); mean peak serum creatinine- 454 mkmol/l (range 67–998), mean peak LDH- 2,953 IU/L (range 996–11,360). 6/9 required hemodialysis and mechanical ventilation. All underwent plasmaexchange or plasma infusion and all of them was appointed unfractionated or low molecular weight heparins. Signs of mild preeclampsia were observed in 8/9 patients with an average of 7 days (3-14) to debut P-aHUS, 4 of them were diagnosed with fetal death on 27-34 gestation weeks. Live births resulted in 5/9 of pregnancies on 28-38 weeks (median 32). Since the onset of the disease state of the patient rapidly deteriorated: acute renal failure was detected in 8/9 patients (6 cases performed hemodialysis), adult respiratory distress syndrome, requiring mechanical ventilation, was diagnosed in 6/9, 6/9 have pankreatit, neurological symptoms were accompanied by observations of 5/9, 2/9 observed in dilated cardiomyopathy and ischemic colitis in one case. The most common organ dysfunction was kidney failure (8/9). 8/9 patients received therapy with fresh frozen plasma. Treatment with eculizumab was started on 4 patients on 5-17 days from the time of P-aHUS debut (median, 7), but the full course was not held anyone: performed from 1 to 3 injections of 900 mg (median 1). All patients received prophylactic anticoagulation. Outcomes as a whole are extremely unfavorable: 5 of 9 patients died (3/5 causes of death-septic complications, thrombosis- 1, hemorrhage- 1), 2/9 - reached end-stage renal failure by 1 month, 2/9 gained the renal function impairment (CKD3-4) by last follow-up.
Conclusion
P-aHUS is a severe life-threatening disorder associated with a significant perinatal and maternal morbidity and mortality. We evaluated the outcomes of P-aHUS. Currently, they are extremely pessimistic now. Diagnostic and therapeutic approaches for this disease requires further study in order to optimize results. Clinical suspicion, early identification and effective approaches including target therapy can improve the pregnancy outcomes and prognosis in general.
Session topic: E-poster
Keyword(s): Pregnancy, Thrombotic microangiopathy
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