Current Issue : October - December Volume : 2020 Issue Number : 4 Articles : 6 Articles
Hemophagocytic lymphohistiocytosis (HLH), which was first described in\n1939 by paediatricians Scott and Robb-Smith, is a life-threatening disease.\nHLH is characterized as cytokine release syndrome which is caused by excessive\nbut non-malignant activation of macrophages and/or histiocytes in bone\nmarrow and other reticuloendothelial systems. EBV-HLH is the most common\ntype of infection-associated HLH, has a high mortality rate without\nprompt and effective treatment. A previous study showed that the one-year\nmortality rate of EBV-HLH patients is 75%. Here we report a case of\nEBV-associated hemophagocytic syndrome in adult, and the lessons from the\ntreatment process. Through this case, we think that for EBV-related HLH,\nEBV-DNA should also be monitored in addition to hemophagocytosis-\nrelated indicators during treatment. In addition, DEP regimen may not be\nsuitable for patients who have received at least partial response, because impaired\nimmunological functioning may lead to EBV and hemophagocytic\nre-activity....
Sickle cell disease is a hemoglobinopathy that results in paroxysmal vascular\nocclusion and tissue infarction that can manifest in a plurality of tissues. Vasoocclusive\ncrises in sickle cell disease commonly involve bone marrow of the\nlong bones and vertebrae. Involvement of bones with less marrow space, including\nthe bones of the orbit, is reported rarely in the literature and can\nclosely mimic orbital cellulitis, both clinically and radiologically. The present\ncase is a 3 years old boy, a known case of sickle cell disease, who presented\nwith what was thought to be orbital cellulitis and was treated accordingly.\nSubtle radiologic features of sickle cell orbitopathy were initially overlooked,\nresulting in an incorrect diagnosis and a treatment delay for the patient. Correctly\ntreated most cases resolve with no adverse effects. This case highlights\nthe importance of maintaining a high index of suspicion in patients with\nknown sickle cell disease, even when the presentation is not classic....
Rituximab hypersensitivity reactions are rare but are one of the main causes of rituximab elimination from antilymphoma\nimmunochemotherapy treatments. While the clinical picture may be indistinguishable from other infusion-related reactions,\nhypersensitivity reactions (HSR) do not disappear and instead become more intense with subsequent administrations. Objective.\nTo describe the use of the 12-step protocol for desensitization to intravenous rituximab in clinical practice and the complementary\nstudy of a possible IgE-mediated HSR in the context of B-cell lymphoma treatment. Methods. A 12-step rituximab desensitization\nprotocol was performed prospectively within clinical practice in 10 patients with a history of severe infusion reactions or in\npatients who had a repeated reaction at subsequent doses despite taking more intense preventive measures. Skin prick tests were\nperformed at the time of reaction and at a later time to eliminate false negatives due to possible drug interference. Results. Overall,\nwith the desensitization protocol, 70% of patients were able to complete the scheduled immunochemotherapy. Two patients had to\ndiscontinue the therapy due to clinical persistence and the third due to lymphoma progression. Intradermal tests with 0.1%\nrituximab were positive in only 20% of cases, demonstrating a mechanism of hypersensitivity. Conclusions. The 12-step desensitization\nprotocol is very effective and assumable within healthcare practice. There is a need to determine the mechanism\nunderlying the infusion reaction in a large proportion of cases due to the risk of future drug exposure....
The high prevalence of haemoglobin variants and glucose 6-phosphate dehydrogenase disorder (G6PDd) in sub-\nSaharan Africa means that substantial proportions of donor blood units carry these red cell abnormalities. Aim. This study\ninvestigated the impact that inherited haemoglobin variants and/or G6PD status have on whole blood banked at......................
Right ventricular (RV) function is an important predictor of prognosis in patients with heart\nfailure. However, the relationship of the RV free wall longitudinal strain (RV FWS) and the degree of\nhepatic dysfunction during the acute worsening of heart failure (AWHF) is unknown. We sought to\ndetermine associations of RV FWS with laboratory liver function tests and parameters of RV function\nincluding tricuspid annular plane systolic excursion (TAPSE), RV fractional area change (RV FAC),\nmaximal tricuspid jet velocity..................
Percutaneous coronary intervention (PCI) is widely used in patients with\nchronic total occlusion (CTO), but its benefit in improving long-term outcomes is controversial.\nWe aimed to develop a prediction score for grading â??survival advantageâ? conferred by successful\nresults of CTO-PCI and a scoring system for prediction of the influence of CTO-PCI results on major\nadverse cardiac and cerebrovascular events (MACCEs). Methods: Follow-up data of 2625 patients\nwho underwent CTO-PCI at 65 Japanese centers were analyzed. An integer scoring system was\ndeveloped by including statistical effect modifiers on the association between successful CTO-PCI\nand one-year mortality. Results: Follow-up at 12 months was completed in 2034 patients. During\nfollow-up, 76 deaths (3.7%) occurred. Patients with successful CTO-PCI had a better one-year survival\nthan patients with failed CTO-PCI..............
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