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Sickle cell disease (SCD) is a devastating hemolytic disease, marked by recurring bouts of painful vaso-occlusion, leading to tissue damage from ischemia/reperfusion pathophysiology. Central to this process are oxidative stress, endothelial cell activation, inflammation, and vascular dysfunction. The endothelium exhibits a pro-inflammatory, pro-coagulant, and enhanced permeability phenotype.

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Emergencies in Hematology: Why, When and How I Treat?

J Clin Med

December 2024

Hematology Unit with BMT, A.O.U. Policlinico "G.Rodolico-San Marco", 95123 Catania, Italy.

Hematological emergencies are critical medical conditions that require immediate attention due to their rapid progression and life-threatening nature. As various examples, hypercalcemia, often associated with cancers such as multiple myeloma, can lead to severe neurological and cardiac dysfunction. Hyperleukocytosis, common in acute myeloid leukemias, increases the risk of leukostasis and multiorgan failure.

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Sickle cell disease (SCD) is a monogenic disease, resulting from a single-point mutation, that presents a complex pathophysiology and high clinical heterogeneity. Inflammation stands as a prominent characteristic of SCD. Over the past few decades, the role of different cells and molecules in the regulation of the inflammatory process has been elucidated.

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The pathophysiology of sickle cell disease (SCD) is characterized by hemolytic anemia and vaso-occlusion, although its impact on the adaptive immune responses remains incompletely understood. To comprehensibly profile the humoral immune responses, we immunized SCD mice with T cell-independent (TI) and T cell-dependent (TD) antigens. Our study showed that SCD mice have significantly enhanced type 2 TI (TI-2) immune responses in a manner dependent on the level of type I IFN (IFN-I), while maintaining similar or decreased TD immune responses depending on the route of antigen administration.

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Beyond IV push: alternative methods for management of acute pain in SCD.

Hematology Am Soc Hematol Educ Program

December 2024

Division of Hematology, Oncology, and Bone Marrow Transplant, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI.

Article Synopsis
  • Acute pain in sickle cell disease (SCD) is caused by complications like vaso-occlusion, leading to intense and sudden pain; traditional treatments include opioids and NSAIDs.
  • Increased understanding of pain pathways in SCD suggests a need for a multimodal approach that includes nonopioid medications and nonpharmacologic methods to better manage pain without the side effects of traditional drugs.
  • The article reviews recent research on alternative treatments and care processes to enhance pain outcomes in SCD while identifying areas that require further investigation to improve treatment effectiveness.
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