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Specialty: Hematology
Condition: Sickle Cell Anemia

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Total 508 results found since Jan 2013.

Plasma exchange-A useful adjunct therapy to red cell exchange in patients with sickle cell disease and multiorgan dysfunction
CONCLUSION: The findings suggest TPE may be considered as an adjunct treatment for patients with acute complications of SCD that progress to MODS, especially in cases where there is no significant improvement following RBCx.PMID:37317564 | DOI:10.1111/trf.17448
Source: Transfusion - June 15, 2023 Category: Hematology Authors: C B Webb S G Yates R Sarode J Kim Source Type: research

Hypercoagulability evaluation in congenital red blood cell disorders using thrombin generation assay
Congenital red blood cell (RBC) disorders are common diseases in the general population. The main outcomes are increased deformability due to erythrocyte instability, increased susceptibility to oxidative stress, and intravascular hemolysis. Hemolytic anemia is associated with thrombosis risk [1]. Main mechanisms are related to hypercoagulability, endothelial damage, altered blood flow, and decreased nitric oxide. Hypercoagulability is well documented in sickle cell disease (SCD) [2]. However, other RBC disorders are associated with pulmonary embolism, hypertension, non-ischemic stroke, and adverse vascular manifestations ...
Source: Thrombosis Research - March 23, 2023 Category: Hematology Authors: Guillaume Feugray, Maximilien Grall, C écile Dumesnil, Ygal Benhamou, Valery Brunel, Véronique Le Cam Duchez, Agnès Lahary, Paul Billoir Tags: Letter to the Editors-in-Chief Source Type: research

Sleep disordered breathing and its relation to stroke and pulmonary hypertension in children with sickle cell disease: a single-center cross-sectional study
AbstractSleep disordered breathing (SDB) is a common underdiagnosed sequela of sickle cell disease (SCD) that has been linked to the frequency of vaso-occlusive crises. To determine the frequency of SDB in children with SCD and its association to SCD-related complications, thirty children and adolescents with SCD at their steady state underwent clinical, laboratory, and radiological assessment using transcranial duplex (TCD) and echo assessment of tricuspid regurge velocity (TRV). All participants had an overnight polysomnography after completing the modified STOP-Bang questionnaire. The mean age of the studied cohort was ...
Source: Annals of Hematology - January 16, 2023 Category: Hematology Source Type: research

How do we operate a large monthly red blood cell exchange program
CONCLUSION: A combination of multidisciplinary coordination and process improvement can ensure the success of a high volume RBC exchange program. Frequent communication of upcoming appointments between the referring hematologists, the hemapheresis clinic, transfusion service, and interventional radiology is critical. Advance notice to the immunohematology reference lab of upcoming appointments is needed to allow enough time for allocating antigen-negative RBCs. Order sets can be leveraged to standardize and streamline RBC exchanges. Lastly, numerous mechanisms help patients compensate for the cognitive sequelae of stroke.P...
Source: Transfusion - January 11, 2023 Category: Hematology Authors: David Allison Luis Manon Vladimir Vidanovic Jason Crane Joan Sevcik Sara Janiszewski Kimberly Gabert Christine Clemente Dos Santos Joseph Discua Lewis L Hsu Victor Gordeuk Sally Campbell-Lee Source Type: research

Excess deaths among adults with sickle cell disease in 2020 compared to prior years
We examined all deaths of people with SCD at our center in 2020. Cause of death was determined, clinical variables, and healthcare utilization, and the presence of COVID infection, sepsis, and acute organ failure during the death event was obtained from the electronic medical record. Deaths in 2020 were compared to deaths in 2017 –2019. In 2020, deaths increase 244% (22 vs 9), but acute or previous COVID infections were identified in only 36% of 2020 deaths. People who died in 2020 were more likely to have developed acute organ failure during the death event (70.6% vs 21.1%,p = 0.003) compared to prior years. They we...
Source: Annals of Hematology - January 1, 2023 Category: Hematology Source Type: research

Organ function indications and potential improvements following curative therapy for sickle cell disease
Hematology Am Soc Hematol Educ Program. 2022 Dec 9;2022(1):277-282. doi: 10.1182/hematology.2022000372.ABSTRACTCurative therapies for sickle cell disease include allogeneic hematopoietic stem cell transplantation (HSCT) and gene-modified autologous stem cell transplantation. HSCT has been used for 30 years with success measured by engraftment, symptom control, graft-vs-host disease (GVHD) risk, organ toxicity, and immune reconstitution. While human leukocyte antigen-matched sibling donor (MSD) transplants have excellent outcomes, alternate donor transplants (unrelated/haploidentical) are just beginning to overcome GVHD and...
Source: Hematology ASH Education Program - December 9, 2022 Category: Hematology Authors: Monica L Hulbert Allison A King Shalini Shenoy Source Type: research