Article
Hematology
Carlton D. Dampier, Marilyn Jo Telen, Ted Wun, R. Clark Brown, Payal Desai, Fuad El Rassi, Beng Fuh, Julie Kanter, Yves Pastore, Jennifer Rothman, James G. Taylor, David Readett, Krupa M. Sivamurthy, Brinda Tammara, Li-Jung Tseng, Jay Nelson Lozier, Helen Thackray, John L. Magnani, Kathryn L. Hassell
Summary: This study evaluated the efficacy and safety of rivipansel in vaso-occlusive crisis (VOC) requiring hospitalization. The results showed that rivipansel did not significantly impact the primary end point, but early treatment with rivipansel after pain onset can accelerate the resolution of acute VOC.
Article
Hematology
Patrick C. Hines, Michael U. Callaghan, Ahmar U. Zaidi, Xiufeng Gao, Ke Liu, Jennell White, Michael Tarasev
Summary: The study found that adhesion of blood cells to P-selectin and VCAM1 is significantly increased on VOC days in patients with SCD. By establishing optimal cutoff values and a multimarker risk score, a better ability to identify VOC risk was demonstrated.
BRITISH JOURNAL OF HAEMATOLOGY
(2021)
Article
Immunology
Joan D. Beckman, Fuad Abdullah, Chunsheng Chen, Rachel Kirchner, Dormarie Rivera-Rodriguez, Zachary M. Kiser, Aithanh Nguyen, Ping Zhang, Julia Nguyen, Robert P. Hebbel, John D. Belcher, Gregory M. Vercellotti
Summary: This study found that in sickle cell disease, endothelial cell TLR4 expression, rather than hematopoietic cell TLR4, is crucial for initiating vaso-occlusive physiology.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Immunology
Chunliang Xu, Maria Gulinello, Paul S. Frenette
Summary: Sensory nerves play a protective role in SCD, relieving the severity of VOE, and manipulating nociception may provide a promising approach for treating SCD.
JOURNAL OF EXPERIMENTAL MEDICINE
(2021)
Review
Immunology
Vasilios A. Morikis, Alfredo A. Hernandez, John L. Magnani, Markus Sperandio, Scott I. Simon
Summary: This article discusses the dual role of neutrophils in SCD and the mechanism of vascular occlusion associated with sickle cell disease. Regulation of neutrophil impact on patients through selectin-mediated integrin adhesive bond formation is highlighted.
FRONTIERS IN IMMUNOLOGY
(2021)
Review
Medicine, Research & Experimental
Tim Jang, Maria Poplawska, Emanuela Cimpeanu, George Mo, Dibyendu Dutta, Seah H. Lim
Summary: VOC remains the most common reason for presentation in SCD patients, but current treatments to shorten VOC have had disappointing results. Exploration of the reasons for ineffective monotherapy and consideration of combination approaches with multiple agents may be necessary to target complex downstream pathways of VOC.
JOURNAL OF TRANSLATIONAL MEDICINE
(2021)
Review
Hematology
Connor Stewart, Tim Jang, George Mo, Nader Mohamed, Maria Poplawska, Ogechukwu Egini, Dibyendu Dutta, Seah H. Lim
Summary: The clinical use of hydroxyurea remains low among SCD patients in the US. Recent studies suggest a link between bacterial overgrowth, dysbiosis, and intestinal pathophysiological changes in SCD, affecting neutrophil aging and potentially contributing to vaso-occlusive crises. Understanding this relationship may lead to new antibiotic-based therapeutic approaches for modifying the clinical course of VOC in SCD.
Article
Immunology
Alexander Leonardo Silva-Junior, Nadja Pinto Garcia, Evilazio Cunha Cardoso, Stephanny Dias, Andrea Monteiro Tarrago, Nelson Abrahim Fraiji, Matheus Souza Gomes, Laurence Rodrigues Amaral, Andrea Teixeira-Carvalho, Olindo Assis Martins-Filho, Erich Vinicius De Paula, Allyson Guimaraes Costa, Adriana Malheiro
Summary: Sickle Cell Anemia (SCA) is characterized by genetic mutation in the beta-globin gene leading to increased hemolysis rate and involvement of immunological molecules. This study analyzed levels of various molecules in SCA patients in different clinical conditions, identifying a pro-inflammatory profile in steady-state patients and highlighting specific molecules in VOC patients. PDGF-BB and IL-1ra were important markers for transitioning from acute to chronic stage, showing significant decrease after crisis inflammation and statistical difference in different patient groups.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Hematology
Ravi Vats, Tomasz W. Kaminski, Eun-Mi Ju, Tomasz Brozska, Egemen Tutuncuoglu, Jesus Tejero, Enrico M. Novelli, Prithu Sundd, Tirthadipa Pradhan-Sundd
Summary: The chronic deficiency of P-selectin attenuates liver ischemia but fails to prevent hepatobiliary injury in SCD mice, possibly due to an increase in cellular senescence and reduced epithelial cell proliferation in the liver. This highlights the importance of investigating the long-term effects of chronic P-selectin inhibition therapy on liver pathophysiology in patients with SCD.
Article
Hematology
Maria Poplawska, Dibyendu Dutta, Manjunath Jayaram, Moro Salifu, Ngee S. Chong, Seah H. Lim
Summary: The study found that both SCD patients and Townes mice show abnormalities in the intestines, which worsen rapidly after VOC induction, and are associated with increased intestinal microbial density. The results suggest that SCD and its complications, rather than hospitalization or medications, are responsible for the intestinal pathophysiological changes.
BRITISH JOURNAL OF HAEMATOLOGY
(2022)
Article
Cell Biology
Lidiane S. Torres, Hanan Chweih, Fernanda C. Z. Fabris, Erica M. F. Gotardo, Flavia C. Leonardo, Sara T. Olalla Saad, Fernando F. Costa, Nicola Conran
Summary: This study investigates the role of transforming growth factor beta-1 (TGF-beta 1) in vascular inflammation and vaso-occlusion in a sickle cell disease (SCD) animal model. The results suggest that TGF-beta 1 may have a significant ameliorative effect on SCD pathophysiology by modulating inflammation and vaso-occlusion.
Article
Cardiac & Cardiovascular Systems
Guillaume Feugray, Fiston Kasonga, Maximilien Grall, Cecile Dumesnil, Ygal Benhamou, Valery Brunel, Veronique Le Cam Duchez, Agnes Lahary, Paul Billoir
Summary: This study found that patients with sickle cell disease have a hypercoagulable state, and TGA parameters such as ETP and peak may be used to predict the development of vaso-occlusive crisis within 1 year.
FRONTIERS IN CARDIOVASCULAR MEDICINE
(2022)
Article
Hematology
Alexis Leonard, Dana Furstenau, Allistair Abraham, Deepika S. Darbari, Robert S. Nickel, Emily Limerick, Courtney Fitzhugh, Matt Hsieh, John F. Tisdale
Summary: Hematopoietic stem cell transplantation (HSCT) is potentially curative for sickle cell disease (SCD) patients. However, there is limited data on the change in vaso-occlusive events (VOE) after HSCT. This study found a significant reduction in VOEs requiring medical care after allogeneic HSCT for SCD patients.
Article
Medicine, General & Internal
Guillaume Feugray, Fiston Kasonga, Maximilien Grall, Ygal Benhamou, Victor Bobee-Schneider, Gerard Buchonnet, Sylvie Daliphard, Veronique Le Cam Duchez, Agnes Lahary, Paul Billoir
Summary: In this study, we found that a combination of routine laboratory biomarkers, including reticulocyte count, immature reticulocyte count, and fluorescent reticulocyte fraction at steady state, could be used to predict the development of vaso-occlusive crisis (VOC) in patients with sickle cell disease (SCD).
FRONTIERS IN MEDICINE
(2022)
Article
Hematology
Aafke E. Gaartman, Ajab K. Sayedi, Jorn J. Gerritsma, Tim R. Back, Charlotte F. Tuijn, Man Wai Tang, Harriet Heijboer, Koen Heer, Bart J. Biemond, Erfan Nur
Summary: Patients with sickle cell disease (SCD) are at risk of developing fluid overload due to intravenous fluid therapy (IV-FT) during treatment of vaso-occlusive crises (VOC). Factors independently associated with fluid overload include positive history of fluid overload, lactate dehydrogenase level, and top-up transfusion during admission. Despite previous history of fluid overload, adjustments to IV-FT are often not made, highlighting the need for more awareness and personalized approach to this complication.
BRITISH JOURNAL OF HAEMATOLOGY
(2021)