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Specialty: Hematology
Condition: Sickle Cell Anemia

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Total 508 results found since Jan 2013.

Variation and impact of polygenic hematological traits in monogenic sickle cell disease
Haematologica. 2022 Oct 13. doi: 10.3324/haematol.2022.281180. Online ahead of print.ABSTRACTSeveral complications observed in sickle cell disease (SCD) are influenced by variation in hematological traits (HT), such as fetal hemoglobin (HbF) level and neutrophil count. Previous large-scale genome-wide association studies carried out in largely healthy individuals have identified 1000s of variants associated with HT, which have then been used to develop multiancestry polygenic trait scores (PTS). Here, we tested if these PTS associate with HT in SCD patients and can improve statistical models associated with SCD-related com...
Source: Haematologica - October 13, 2022 Category: Hematology Authors: Thomas Pincez Ken Sin Lo Anne-Laure Pham Hung d'Alexandry D'Orengiani Melanie E Garrett Carlo Brugnara Allison E Ashley-Koch Marilyn J Telen Frederic Galacteros Philippe Joly Pablo Bartolucci Guillaume Lettre 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

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 ā€“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%,pā€‰=ā€‰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