Abdullah Kutlar
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View article: The Role of Hemoglobin F in Sickle Cell Disease Complications: A Descriptive Secondary Analysis
The Role of Hemoglobin F in Sickle Cell Disease Complications: A Descriptive Secondary Analysis Open
View article: The ASH Research Collaborative Sickle Cell Disease Research Network: Past, Present and Future
The ASH Research Collaborative Sickle Cell Disease Research Network: Past, Present and Future Open
Historically, Sickle Cell Disease (SCD) has been overshadowed by progress in other hematologic disorders, but recent advances are reshaping its clinical and research landscape. Despite SCD reducing life expectancy by more than 20 years eve…
View article: Mean Corpuscular Hemoglobin Modulates <scp>HbF</scp>/F‐Cell and Clinical Response to Gene Therapy and Hydroxyurea in Sickle Cell Disease
Mean Corpuscular Hemoglobin Modulates <span>HbF</span>/F‐Cell and Clinical Response to Gene Therapy and Hydroxyurea in Sickle Cell Disease Open
View article: Endothelial ENaC as a repressor of oxidative stress and a guardian of lung capillary barrier function in bacterial and viral pneumonia
Endothelial ENaC as a repressor of oxidative stress and a guardian of lung capillary barrier function in bacterial and viral pneumonia Open
The endothelium represents a crucial regulator of vascular homeostasis. Since endothelial cells mainly rely on glycolysis rather than on oxidative phosphorylation for their ATP generation, this allows capillaries to transport the maximum a…
View article: Clinical Images: Vasculitic rash as a presenting symptom of heparin‐induced thrombocytopenia in a woman with primary antiphospholipid syndrome
Clinical Images: Vasculitic rash as a presenting symptom of heparin‐induced thrombocytopenia in a woman with primary antiphospholipid syndrome Open
View article: Safety profile of crizanlizumab 5.0 mg/kg in sickle cell disease: pooled data from clinical trials
Safety profile of crizanlizumab 5.0 mg/kg in sickle cell disease: pooled data from clinical trials Open
Objectives This pooled analysis evaluated key safety endpoints in patients with sickle cell disease treated with crizanlizumab. Methods Two clinical data pools were created: Pool 1 (crizanlizumab group from three Phase 2 [SUSTAIN, SOLACE-a…
View article: Enhanced vasoconstriction in sickle cell disease is dependent on ETA receptor activation
Enhanced vasoconstriction in sickle cell disease is dependent on ETA receptor activation Open
Sickle cell disease (SCD) carries a significant risk for poor vascular health and vascular dysfunction. High levels of vascular reactive oxygen species (ROS) as well as elevated plasma endothelin-1 (ET-1), a potent vasoconstrictor with act…
View article: The clinical spectrum of <scp>HbSC</scp> sickle cell disease‐not a benign condition
The clinical spectrum of <span>HbSC</span> sickle cell disease‐not a benign condition Open
Summary Sickle cell disease (SCD) includes a group of heterogenous disorders that result in significant morbidities. HbSS is the most common type of SCD and HbSC is the second most common type of SCD. The prevalence of HbSC disease in the …
View article: PB-04 Induces HbF Expression in a Ph1b Trial in Sickle Cell Disease
PB-04 Induces HbF Expression in a Ph1b Trial in Sickle Cell Disease Open
Presentation Date: 6/9/2024 Presentation Start Time: 2:27:00 PM Background Fetal hemoglobin (HbF) is established as a potent modulator of severity in sickle cell disease (SCD); any increment has been shown to be beneficial. Hydroxyurea mai…
View article: Survival of transfused red blood cells from a donor with alpha‐thalassemia trait in a recipient with sickle cell disease
Survival of transfused red blood cells from a donor with alpha‐thalassemia trait in a recipient with sickle cell disease Open
Background Post‐transfusion survival of donor red blood cells (RBCs) is important for effective chronic transfusion therapy in conditions including sickle cell disease (SCD). Biotin labeling RBCs allows direct in vivo measurement of multip…
View article: Mortality in adults with sickle cell disease: Results from the sickle cell disease implementation consortium (SCDIC) registry
Mortality in adults with sickle cell disease: Results from the sickle cell disease implementation consortium (SCDIC) registry Open
The cause of death in people affected by sickle cell disease (SCD) is often challenging to define as prior studies have used retrospective or administrative data for analysis. We used a prospective longitudinal registry to assess mortality…
View article: Pharmacokinetics, pharmacodynamics, safety, and efficacy of crizanlizumab in patients with sickle cell disease: final results from the phase II SOLACE-adults study
Pharmacokinetics, pharmacodynamics, safety, and efficacy of crizanlizumab in patients with sickle cell disease: final results from the phase II SOLACE-adults study Open
Background: Crizanlizumab is a novel inhibitor of P-selectin, a key player in multicellular adhesion and inflammatory signaling, that leads to vaso-occlusion in sickle cell disease (SCD). Objectives: The SOLACE-adults study evaluated the p…
View article: A multilevel mHealth intervention boosts adherence to hydroxyurea in individuals with sickle cell disease
A multilevel mHealth intervention boosts adherence to hydroxyurea in individuals with sickle cell disease Open
Hydroxyurea reduces sickle cell disease (SCD) complications, but medication adherence is low. We tested 2 mobile health (mHealth) interventions targeting determinants of low adherence among patients (InCharge Health) and low prescribing am…
View article: MDS-273 Oral Iptacopan Monotherapy Increases Paroxysmal Nocturnal Hemoglobinuria (PNH) Red Blood Cell Clone Size Via Control of Intra- and Extravascular Hemolysis in Anti-C5-Treated PNH Patients With Anemia
MDS-273 Oral Iptacopan Monotherapy Increases Paroxysmal Nocturnal Hemoglobinuria (PNH) Red Blood Cell Clone Size Via Control of Intra- and Extravascular Hemolysis in Anti-C5-Treated PNH Patients With Anemia Open
View article: S182: ORAL IPTACOPAN MONOTHERAPY INCREASES PAROXYSMAL NOCTURNAL HEMOGLOBINURIA (PNH) RED BLOOD CELL CLONE SIZE VIA CONTROL OF INTRA- AND EXTRAVASCULAR HEMOLYSIS IN ANTI-C5-TREATED PNH PATIENTS WITH ANEMIA
S182: ORAL IPTACOPAN MONOTHERAPY INCREASES PAROXYSMAL NOCTURNAL HEMOGLOBINURIA (PNH) RED BLOOD CELL CLONE SIZE VIA CONTROL OF INTRA- AND EXTRAVASCULAR HEMOLYSIS IN ANTI-C5-TREATED PNH PATIENTS WITH ANEMIA Open
Background: Anemia caused by C3-mediated extravascular hemolysis (EVH) commonly occurs in patients (pts) with PNH receiving standard-of-care (SoC) anti-C5 therapies. Iptacopan (IPTA) is a first-in-class, oral, selective complement factor B…
View article: Successful Treatment of Refractory Thrombotic Thrombocytopenic Purpura (TTP) With Caplacizumab: A Case Report
Successful Treatment of Refractory Thrombotic Thrombocytopenic Purpura (TTP) With Caplacizumab: A Case Report Open
We report a female patient who presented with generalized weakness, episodes of altered mental status and slurred speech, and a history of systemic lupus erythematosus. Initial investigations showed profound thrombocytopenia and schistocyt…
View article: Burden of Aging: Health Outcomes Among Adolescents and Young Adults With Sickle Cell Disease
Burden of Aging: Health Outcomes Among Adolescents and Young Adults With Sickle Cell Disease Open
Although ≈95% of children with sickle cell disease (SCD) will reach adulthood in high-income settings,1–4 adolescents and young adults (AYA) face difficulties in establishing adult care and experience progression of disease severity as the…
View article: Evaluating the implementation of a multi-level mHealth study to improve hydroxyurea utilization in sickle cell disease
Evaluating the implementation of a multi-level mHealth study to improve hydroxyurea utilization in sickle cell disease Open
Background Sickle Cell Disease (SCD) is a progressive genetic disease that causes organ damage and reduces longevity. Hydroxyurea is an underutilized evidence-based medication that reduces complications and improves survival in SCD. In a m…
View article: A-two to the rescue
A-two to the rescue Open
In this issue of Blood, Zhu et al 1 report on a novel gene therapy approach, enhancing the expression of the δ-globin chain of human minor adult hemoglobin, HbA 2 (α2δ2), to treat β-hemoglobinopathies.
View article: Pharmacokinetics, pharmacodynamics, safety, and efficacy of crizanlizumab in patients with sickle cell disease
Pharmacokinetics, pharmacodynamics, safety, and efficacy of crizanlizumab in patients with sickle cell disease Open
Crizanlizumab is an anti–P-selectin monoclonal antibody indicated to reduce the frequency/prevent recurrence of vaso-occlusive crises (VOCs) in patients with sickle cell disease (SCD) aged ≥16 years. This analysis of an ongoing phase 2, no…
View article: Pregnancy outcomes with hydroxyurea use in women with sickle cell disease
Pregnancy outcomes with hydroxyurea use in women with sickle cell disease Open
Hydroxyurea reduces pain crises, acute chest syndrome, and blood transfusions in sickle cell disease (SCD), but potential detrimental effects on fertility and birth outcomes impede its use. Data on the effects of hydroxyurea taken for SCD …
View article: Implementation and Preliminary Effectiveness of mHealth Apps for Improving Sickle Cell Disease Care during COVID-19: A Mixed-Methods Evaluation
Implementation and Preliminary Effectiveness of mHealth Apps for Improving Sickle Cell Disease Care during COVID-19: A Mixed-Methods Evaluation Open
Background: Sickle cell disease (SCD) is a chronic blood disorder, which disproportionately impacts Black individuals. Hydroxyurea therapy prevents SCD-related complications; yet it is underutilized, which contributes to further health ine…
View article: Utilization of Patient-Controlled Analgesia Reduces Length of Stay of Sickle Cell Crisis Hospitalizations
Utilization of Patient-Controlled Analgesia Reduces Length of Stay of Sickle Cell Crisis Hospitalizations Open
Among patients with sickle cell crises who required inpatient hospitalizations, the use of patient-controlled analgesia demonstrated a statistically significant reduction of 1.2 days in their total length of stay. These findings support po…
View article: Novel Germline RUNX1 Mutation Associated with Familial Thrombocytopenia as well as B-Acute Lymphoblastic Leukemia: A Case Report and Review of the Literature
Novel Germline RUNX1 Mutation Associated with Familial Thrombocytopenia as well as B-Acute Lymphoblastic Leukemia: A Case Report and Review of the Literature Open
Germline RUNX1 mutations lead to a rare form of autosomal-dominant familial thrombocytopenia with a predisposition for myeloid malignancies and are classified as distinct entities by the WHO. We report a case of B lymphoblastic leuk…
View article: P-Selectin Blockade in the Treatment of Painful Vaso-Occlusive Crises in Sickle Cell Disease: A Spotlight on Crizanlizumab
P-Selectin Blockade in the Treatment of Painful Vaso-Occlusive Crises in Sickle Cell Disease: A Spotlight on Crizanlizumab Open
Microvascular vaso-occlusion driven pain crisis is the hallmark of sickle cell disease with profound morbidity and increased mortality. Selectins, most notably P-selectins have an integral role in this phenomenon. P-selection was first ide…
View article: Characteristics of sickle cell patients with frequent emergency department visits and hospitalizations
Characteristics of sickle cell patients with frequent emergency department visits and hospitalizations Open
Vaso-occlusive episodes (VOEs) are a hallmark of sickle cell disease (SCD), and account for >90% of health care encounters for this patient population. The Cooperative Study of Sickle Cell Disease, a large study enrolling >3000 patients, s…
View article: Streptococcus pneumoniae and Its Virulence Factors H2O2 and Pneumolysin Are Potent Mediators of the Acute Chest Syndrome in Sickle Cell Disease
Streptococcus pneumoniae and Its Virulence Factors H2O2 and Pneumolysin Are Potent Mediators of the Acute Chest Syndrome in Sickle Cell Disease Open
Sickle cell disease (SCD) is one of the most common autosomal recessive disorders in the world. Due to functional asplenia, a dysfunctional antibody response, antibiotic drug resistance and poor response to immunization, SCD patients have …
View article: Novel mechanism of hereditary pyropoikilocytosis phenotype due to co‐inheritance of β globin and α spectrin mutations
Novel mechanism of hereditary pyropoikilocytosis phenotype due to co‐inheritance of β globin and α spectrin mutations Open
To the Editor: Co-existence of red blood cell (RBC) membrane disorders and hemoglobinopathies are rare and may present a diagnostic challenge due to overlap of clinical findings. We describe a novel mechanism for the phenotype of hereditar…
View article: Sickle cell disease: progress towards combination drug therapy
Sickle cell disease: progress towards combination drug therapy Open
Summary Dr. John Herrick described the first clinical case of sickle cell anaemia (SCA) in the United States in 1910. Subsequently, four decades later, Ingram and colleagues characterized the A to T substitution in DNA producing the GAG to…
View article: Electronic Health Record–Embedded Individualized Pain Plans for Emergency Department Treatment of Vaso-occlusive Episodes in Adults With Sickle Cell Disease: Protocol for a Preimplementation and Postimplementation Study
Electronic Health Record–Embedded Individualized Pain Plans for Emergency Department Treatment of Vaso-occlusive Episodes in Adults With Sickle Cell Disease: Protocol for a Preimplementation and Postimplementation Study Open
Background Individuals living with sickle cell disease often require aggressive treatment of pain associated with vaso-occlusive episodes in the emergency department. Frequently, pain relief is poor. The 2014 National Heart, Lung, and Bloo…