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
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.
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
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)
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)
Article
Oncology
Cecile Karsenty, Venee N. Tubman, Chyong-jy Joyce Liu, Titilope Fasipe, Karla E. K. Wyatt
Summary: Placing CPNB for analgesia in pediatric SCD patients with opioid-refractory upper-extremity VOC can reduce opioid consumption, improve pain scores, and decrease length of hospitalization.
PEDIATRIC BLOOD & CANCER
(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
Pediatrics
Jeanne Sigalla, Nathalie Duparc Alegria, Enora Le Roux, Artemis Toumazi, Anne-Francoise Thiollier, Laurent Holvoet, Malika Benkerrou, Sophie Dugue, Berengere Koehl
Summary: This study found that neuropathic pain (NP) is common during vaso-occlusive crises (VOC) in children with sickle cell disease (SCD), mostly occurring in the early stages of VOC. There were no significant differences in age, sex, and location of pain between patients with and without NP.
Article
Anesthesiology
Zachary Ramsay, Rachel Bartlett, Amza Ali, Justin Grant, Georgiana Gordon-Strachan, Monika Asnani
Summary: This study investigated the prevalence and characteristics of chronic pain and neuropathic pain in Jamaican patients with sickle cell disease, finding that most patients had experienced acute pain crises and many also had chronic and neuropathic pain. Opioids were commonly used, while nonpharmacological treatments like physiotherapy were reported to be effective.
CLINICAL JOURNAL OF PAIN
(2021)
Article
Hematology
Ondine Walter, Pierre Cougoul, Julien Maquet, Pablo Bartolucci, Maryse Lapeyre-Mestre, Margaux Lafaurie, Guillaume Moulis
Summary: This study aims to assess the risk of hospitalization for vaso-occlusive episodes (VOEs) associated with exposure to systemic corticosteroids in patients with sickle cell disease (SCD). The results indicate a significant association between systemic corticosteroid exposure and the occurrence of hospitalizations for VOEs.
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
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)