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Enhanced Long-Term Brain Magnetic Resonance Imaging Evaluation of Children with Sickle Cell Disease after Hematopoietic Cell Transplantation
Sickle cell disease (SCD) is the most common cause of stroke in children. Strokes and “clinically silent” infarcts are caused by progressive sickle vasculopathy in large and small vessels [1-4], leading to SCD brain vasculopathy. Both stroke types can affect neurological and cognitive function starting early in childhood [1,2,5-9]. Stroke or stroke risk is a frequent indication f or pediatric SCD transplantation [10-12]. Based on several studies using brain magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA), hematopoietic stem cell transplantation (HCT) is believed to halt progression of vasculopathy [10,12-16].
Source: Biology of Blood and Marrow Transplantation - January 8, 2017 Category: Hematology Authors: Nancy S. Green, Monica Bhatia, Erica Y. Griffith, Mahvish Qureshi, Courtney Briamonte, Mirko Savone, Stephen Sands, Margaret T. Lee, Angela Lignelli, Adam M. Brickman Source Type: research

Long-Term Outcome and Evaluation of Organ Function in Pediatric Patients Undergoing Haploidentical and Matched Related Hematopoietic Cell Transplantation for Sickle Cell Disease
We report the long-term outcomes of 22 pediatric patients who underwent related donor HSCT for SCD at St. Jude Children's Research Hospital, either a myeloablative sibling MRD HSCT (n = 14) or reduced-intensity parental haploidentical donor HSCT (n = 8). The median patient age was 11.0 ± 3.9 years in the MRD graft recipients and 9.0 ± 5.0 years in the haploidentical donor graft recipients. The median follow-up was 9.0 ± 2.3 years, with an overall survival (OS) of 93% and a recurrence/graft failure rate of 0%, for the MRD cohort and 7.4 ± 2.4 years, with an OS of 75%, disease-free survival of 38%, and disease recurren...
Source: Biology of Blood and Marrow Transplantation - February 18, 2013 Category: Hematology Authors: Mari H. Dallas, Brandon Triplett, David R. Shook, Christine Hartford, Ashok Srinivasan, Joseph Laver, Russell Ware, Wing Leung Tags: Clinical Research Source Type: research

Sustaining and Improving Current Practices Surrounding Timely Hypertension Management in Pediatric Hematopoietic Stem Cell Patients
Topic Significance& Study Purpose/Background/Rationale: Hypertension (HTN) is a known complication during pediatric hematopoietic stem cell transplant (HSCT). The risks for developing hypertension depend on a variety of factors including the use of drugs (calcineurin inhibitors, steroids, and chemotherapy), total body irradiation (TBI), renal injury, transplant associated thrombotic microangiopathy (TMA) and sinusoidal obstructive syndrome (SOS). Uncontrolled HTN may lead to significant life-threatening complications including impaired renal and heart function, stroke, posterior reversible encephalopathy (PRES) and death.
Source: Biology of Blood and Marrow Transplantation - February 22, 2017 Category: Hematology Authors: Michelle Lewyckyj, Adam S. Nelson, Christopher E. Dandoy, Ashley Teusink, Sonata Jodele, Laura Flesch Source Type: research

Outcomes of Unrelated Donor Stem Cell Transplant with Post-Transplant Cyclophosphamide for Graft-versus-Host Disease Prophylaxis in Patients with Severe Sickle Cell Disease
Sickle cell disease (SCD) is a hemoglobinopathy affecting 1 in every 500 African American newborns and an estimated 100,000 persons in the United States [1]. Vascular occlusion caused by the sickled RBCs leads to decreased perfusion in target organs and manifests clinically as vaso-occlusive pain crisis, stroke (cerebral infarction), pulmonary restrictive disease, and pulmonary hypertension [2]. Despite advances in supportive care, including the use of hydroxyurea (HU), RBC transfusion, and iron chelation therapy, patients with SCD continue to experience considerable morbidity and mortality in their lifetime.
Source: Biology of Blood and Marrow Transplantation - October 20, 2017 Category: Hematology Authors: Hemalatha G. Rangarajan, Rolla Abu-Arja, Vinita Pai, Gregory M.T. Guilcher, Sandeep Soni Source Type: research

Outcomes of Unrelated Donor Stem Cell Transplantion with Post-Transplant Cyclophosphamide for Graft-versus-Host Disease Prophylaxis in Patients with Severe Sickle Cell Disease
Sickle cell disease (SCD) is a hemoglobinopathy affecting 1 in every 500 African American newborns and an estimated 100,000 persons in the United States [1]. Vascular occlusion caused by the sickled RBCs leads to decreased perfusion in target organs and manifests clinically as vaso-occlusive pain crisis, stroke (cerebral infarction), pulmonary restrictive disease, and pulmonary hypertension [2]. Despite advances in supportive care, including the use of hydroxyurea (HU), RBC transfusion, and iron chelation therapy, patients with SCD continue to experience considerable morbidity and mortality in their lifetime.
Source: Biology of Blood and Marrow Transplantation - October 20, 2017 Category: Hematology Authors: Hemalatha G. Rangarajan, Rolla Abu-Arja, Vinita Pai, Gregory M.T. Guilcher, Sandeep Soni Source Type: research

Low Rates of Cerebral Infarction after Hematopoietic Stem Cell Transplantation in Patients with Sickle Cell Disease at High Risk for Stroke
(CNS)
Source: Biology of Blood and Marrow Transplantation - September 13, 2021 Category: Hematology Authors: Jessica L. Carpenter, Robert S. Nickel, Jennifer Webb, Zarir Khademian, Barbara Speller-Brown, Suvankar Majumdar, Deepika S. Darbari, Andrew Campbell, Anqing Zhang, Allistair Abraham Source Type: research