A protective anti-infalmmatory phenotype in high risk individuals who do not develop coronary artery disease.

Background: Atherosclerosis and CAD involve lipid deposit, endothelial dysfunction and inflammation. Hypercholesterolemia may cause endothelial dysfunction and inflammation, but few studies have investigated possible anti-inflammatory and endothelial protective mechanisms in high-risk individuals without CAD. Objective: To study endothelial and inflammatory mechanisms in individuals with different lipoprotein profiles relative to verified coronary artery disease (CAD). Methods: Statin naive individuals referred to coronary angiography were consecutively included in the study if serum LDL was 4.5 mmol/L. The patients were categorized according to angiographically verified CAD: 1.high LDL with CAD( n=16); 2. high LDL without CAD( n=16); 3. low LDL with CAD( n=15), 4. low LDL without CAD (n=18). Results: Patients with hypercholesterolemia and verified normal coronary arteries had preserved endothelial function, as assessed by the microcirculatory response to acetylcholine, reduced levels of inflammatory markers and in particular high levels of the anti inflammatory cytokine interleukin-10 (IL-10). On the other hand, patients with CAD, regardless of cholesterol levels, were characterized by endothelial dysfunction as well as enhanced levels of markers of inflammation (i.e. tumor necrosis factor  and high-sensitivity C-reactive protein) and platelet activation (i.e. soluble CD40 ligand and soluble P-selectin). Conclusion: We show that individuals with hypercholesterolemia and verified normal coronary arteries are characterized by raised plasma levels of the anti inflammatory cytokine IL-10, accompanied by preserved endothelial function and a lower degree of platelet activation and inflammation, possibly reflecting a protective anti-inflammatory phenotype in these high-risk individuals

Two consecutive episodes of severe delayed hemolytic transfusion reaction in a sickle cell disease patient

CITATION: Mpinganzima, C. et al. 2020. Two consecutive episodes of severe delayed hemolytic transfusion reaction in a sickle cell disease patient. Case Reports in Hematology, 2020:2765012, doi:10.1155/2020/2765012.The original publication is available at https://www.hindawi.com/journals/crihemPatients with sickle cell disease (SCD) suffer from anemia and painful vaso-occlusive crisis (VOC) and sometimes need blood transfusions. Delayed hemolytic transfusion reaction (DHTR) is a rare life-threatening complication observed in SCD and mimics VOC. We describe a female SCD patient undergoing three surgical procedures during which DHTR developed following the first two. Prior to a planned tonsillectomy, she received transfusion and three days after surgery developed severe hemolysis as well as pain and respiratory symptoms. On suspicion of VOC, she received additional transfusions and became hemodynamically unstable, and her hemolytic anemia worsened. Gradually, she recovered and could be discharged after two weeks; DHTR was not suspected. Sixteen months later, an arthroplasty was performed due to avascular necrosis, and again she was transfused preoperatively. Similar to the initial surgery, she developed symptoms and signs of VOC after three days, but this time, DHTR was suspected and further transfusions were withheld. Although immunosuppressive medication did not alleviate the condition, she improved on combined treatment with darbepoietin, rituximab, and eculizumab. Six months later, a second arthroplasty was performed uneventfully after prophylaxis with rituximab and without transfusion. DHTR should be considered in the presence of severe, unexplained hemolysis following a recent transfusion, and additional transfusions in this setting should be given only on vital indication.https://www.hindawi.com/journals/crihem/2020/2765012/Publisher's versio

Two Consecutive Episodes of Severe Delayed Hemolytic Transfusion Reaction in a Sickle Cell Disease Patient

Patients with sickle cell disease (SCD) suffer from anemia and painful vaso-occlusive crisis (VOC) and sometimes need blood transfusions. Delayed hemolytic transfusion reaction (DHTR) is a rare life-threatening complication observed in SCD and mimics VOC. We describe a female SCD patient undergoing three surgical procedures during which DHTR developed following the first two. Prior to a planned tonsillectomy, she received transfusion and three days after surgery developed severe hemolysis as well as pain and respiratory symptoms. On suspicion of VOC, she received additional transfusions and became hemodynamically unstable, and her hemolytic anemia worsened. Gradually, she recovered and could be discharged after two weeks; DHTR was not suspected. Sixteen months later, an arthroplasty was performed due to avascular necrosis, and again she was transfused preoperatively. Similar to the initial surgery, she developed symptoms and signs of VOC after three days, but this time, DHTR was suspected and further transfusions were withheld. Although immunosuppressive medication did not alleviate the condition, she improved on combined treatment with darbepoietin, rituximab, and eculizumab. Six months later, a second arthroplasty was performed uneventfully after prophylaxis with rituximab and without transfusion. DHTR should be considered in the presence of severe, unexplained hemolysis following a recent transfusion, and additional transfusions in this setting should be given only on vital indication