HIGH INCIDENCE OF NEUROLOGICAL COMPLICATIONS IN SICKLE CELL DISEASE UNDERGOING ALLOGENEIC HEMATOPOIETIC STEM CELL TRANSPLANTATION: EARLY DIAGNOSIS CAN CHANGE THE PROGNOSIS
(Abstract release date: 05/21/15)
EHA Library. Helman R. 06/12/15; 102882; PB2045
Disclosure(s): Hospital Israelita Albert Einstein
Ricardo Helman
Contributions
Contributions
Abstract
Abstract: PB2045
Type: Publication Only
Background
Hematopoietic stem cell transplantation (HSCT) was offered primarily as a therapeutic option for severe sickle cell disease in the context of myeloablative matched sibling donor transplants over the last two decades and helped to establish the benefits of transplantation for this disorder. In recent years, the transplant community has set out to explore ways to make stem-cell transplantation more available to patients with the disease, define indications and better timing, and offset toxicities with novel approaches to conditioning and better supportive care. In this context, neurological complication such as stroke and blood flow alterations in cerebral artery constitute the main indications of HSCT but neurological complications are the main causes of TRM in D+100 and is important to find ways to prevent this problem.
Aims
Describe early neurological complications in sickle cell patients undergoing related HSCT on single center and its following after the early diagnosis.
Methods
Results
The median of the neutrophil engraftment was D+ 25 and the platelets engraftment was in D+60. Two patient died, one by intestinal and liver GVHD on D+120, and another with sagittal sinus thrombosis and hemorrhagic stroke on D+3. Other two patients showed PRES syndrome related to cyclosporine use. The patients showed generalized seizures with tomographic neurological alteration. After the imunossupressor change to tacrolimus and the change of Phenytoin to Lamotrigine, the patient had total resolution of neurological complications without development of neurological sequelae. Patients who used Lamotrigine since the beginning of the conditioning have not shown neurological alterations.
Summary
The PRES Syndrome and Stroke are two of the main causes of mortality related to the use of calcineurin inhibitors. Patients with sickle disease have shown endothelial and cerebral microcirculation changes, which made them highly susceptible to neurological complication. The control of blood pressure, maintenance of 50.000 platelets level and the use of Lamotrigine as prophylaxis of seizures seems to decrease the risk of neurological complications. Prospective studies with lamotrigine as primary prevention of PRES Syndrome must be performed. Patients with severe neurological alteration as vessels stenosis more than 90% and Moya-Moya Syndrome must be better evaluated before the conditioning because of high TRM risk.
Keyword(s): Bone marrow transplant, Complications, Sickle cell anemia
Session topic: Publication Only
Type: Publication Only
Background
Hematopoietic stem cell transplantation (HSCT) was offered primarily as a therapeutic option for severe sickle cell disease in the context of myeloablative matched sibling donor transplants over the last two decades and helped to establish the benefits of transplantation for this disorder. In recent years, the transplant community has set out to explore ways to make stem-cell transplantation more available to patients with the disease, define indications and better timing, and offset toxicities with novel approaches to conditioning and better supportive care. In this context, neurological complication such as stroke and blood flow alterations in cerebral artery constitute the main indications of HSCT but neurological complications are the main causes of TRM in D+100 and is important to find ways to prevent this problem.
Aims
Describe early neurological complications in sickle cell patients undergoing related HSCT on single center and its following after the early diagnosis.
Methods
Seven patient undergone related HSCT for sickle disease from 2011 to 2013. All patients filled inclusion criteria in the study and signed agreement term. Seven HSCT were developed in the period, being four males. The average age was 13 years old (7-24). The HSCT indications were previous stroke, cerebral flow alteration on Doppler, acute chest syndrome and alloimunization. All patient were on blood transfusion therapy. The conditioning regimen was BuCy + ATG and the GVHD prophylaxis was MTX and CSA. Related donors were chosen with 10/10 HLA match and graft source marrow
Results
The median of the neutrophil engraftment was D+ 25 and the platelets engraftment was in D+60. Two patient died, one by intestinal and liver GVHD on D+120, and another with sagittal sinus thrombosis and hemorrhagic stroke on D+3. Other two patients showed PRES syndrome related to cyclosporine use. The patients showed generalized seizures with tomographic neurological alteration. After the imunossupressor change to tacrolimus and the change of Phenytoin to Lamotrigine, the patient had total resolution of neurological complications without development of neurological sequelae. Patients who used Lamotrigine since the beginning of the conditioning have not shown neurological alterations.
Summary
The PRES Syndrome and Stroke are two of the main causes of mortality related to the use of calcineurin inhibitors. Patients with sickle disease have shown endothelial and cerebral microcirculation changes, which made them highly susceptible to neurological complication. The control of blood pressure, maintenance of 50.000 platelets level and the use of Lamotrigine as prophylaxis of seizures seems to decrease the risk of neurological complications. Prospective studies with lamotrigine as primary prevention of PRES Syndrome must be performed. Patients with severe neurological alteration as vessels stenosis more than 90% and Moya-Moya Syndrome must be better evaluated before the conditioning because of high TRM risk.
Keyword(s): Bone marrow transplant, Complications, Sickle cell anemia
Session topic: Publication Only
Abstract: PB2045
Type: Publication Only
Background
Hematopoietic stem cell transplantation (HSCT) was offered primarily as a therapeutic option for severe sickle cell disease in the context of myeloablative matched sibling donor transplants over the last two decades and helped to establish the benefits of transplantation for this disorder. In recent years, the transplant community has set out to explore ways to make stem-cell transplantation more available to patients with the disease, define indications and better timing, and offset toxicities with novel approaches to conditioning and better supportive care. In this context, neurological complication such as stroke and blood flow alterations in cerebral artery constitute the main indications of HSCT but neurological complications are the main causes of TRM in D+100 and is important to find ways to prevent this problem.
Aims
Describe early neurological complications in sickle cell patients undergoing related HSCT on single center and its following after the early diagnosis.
Methods
Results
The median of the neutrophil engraftment was D+ 25 and the platelets engraftment was in D+60. Two patient died, one by intestinal and liver GVHD on D+120, and another with sagittal sinus thrombosis and hemorrhagic stroke on D+3. Other two patients showed PRES syndrome related to cyclosporine use. The patients showed generalized seizures with tomographic neurological alteration. After the imunossupressor change to tacrolimus and the change of Phenytoin to Lamotrigine, the patient had total resolution of neurological complications without development of neurological sequelae. Patients who used Lamotrigine since the beginning of the conditioning have not shown neurological alterations.
Summary
The PRES Syndrome and Stroke are two of the main causes of mortality related to the use of calcineurin inhibitors. Patients with sickle disease have shown endothelial and cerebral microcirculation changes, which made them highly susceptible to neurological complication. The control of blood pressure, maintenance of 50.000 platelets level and the use of Lamotrigine as prophylaxis of seizures seems to decrease the risk of neurological complications. Prospective studies with lamotrigine as primary prevention of PRES Syndrome must be performed. Patients with severe neurological alteration as vessels stenosis more than 90% and Moya-Moya Syndrome must be better evaluated before the conditioning because of high TRM risk.
Keyword(s): Bone marrow transplant, Complications, Sickle cell anemia
Session topic: Publication Only
Type: Publication Only
Background
Hematopoietic stem cell transplantation (HSCT) was offered primarily as a therapeutic option for severe sickle cell disease in the context of myeloablative matched sibling donor transplants over the last two decades and helped to establish the benefits of transplantation for this disorder. In recent years, the transplant community has set out to explore ways to make stem-cell transplantation more available to patients with the disease, define indications and better timing, and offset toxicities with novel approaches to conditioning and better supportive care. In this context, neurological complication such as stroke and blood flow alterations in cerebral artery constitute the main indications of HSCT but neurological complications are the main causes of TRM in D+100 and is important to find ways to prevent this problem.
Aims
Describe early neurological complications in sickle cell patients undergoing related HSCT on single center and its following after the early diagnosis.
Methods
Seven patient undergone related HSCT for sickle disease from 2011 to 2013. All patients filled inclusion criteria in the study and signed agreement term. Seven HSCT were developed in the period, being four males. The average age was 13 years old (7-24). The HSCT indications were previous stroke, cerebral flow alteration on Doppler, acute chest syndrome and alloimunization. All patient were on blood transfusion therapy. The conditioning regimen was BuCy + ATG and the GVHD prophylaxis was MTX and CSA. Related donors were chosen with 10/10 HLA match and graft source marrow
Results
The median of the neutrophil engraftment was D+ 25 and the platelets engraftment was in D+60. Two patient died, one by intestinal and liver GVHD on D+120, and another with sagittal sinus thrombosis and hemorrhagic stroke on D+3. Other two patients showed PRES syndrome related to cyclosporine use. The patients showed generalized seizures with tomographic neurological alteration. After the imunossupressor change to tacrolimus and the change of Phenytoin to Lamotrigine, the patient had total resolution of neurological complications without development of neurological sequelae. Patients who used Lamotrigine since the beginning of the conditioning have not shown neurological alterations.
Summary
The PRES Syndrome and Stroke are two of the main causes of mortality related to the use of calcineurin inhibitors. Patients with sickle disease have shown endothelial and cerebral microcirculation changes, which made them highly susceptible to neurological complication. The control of blood pressure, maintenance of 50.000 platelets level and the use of Lamotrigine as prophylaxis of seizures seems to decrease the risk of neurological complications. Prospective studies with lamotrigine as primary prevention of PRES Syndrome must be performed. Patients with severe neurological alteration as vessels stenosis more than 90% and Moya-Moya Syndrome must be better evaluated before the conditioning because of high TRM risk.
Keyword(s): Bone marrow transplant, Complications, Sickle cell anemia
Session topic: Publication Only
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