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“…Notably, two of our patients developed multi-organ system failure during their hospitalization, both with HbSC disease and older than 10 years. A clinically severe course of TAC has been described previously in HbSC patients [11] and our cases confirm that parvovirus infection in older children with HbSC may have increased morbidity. Children with HbSC appear to be more likely to develop ASSC and acute chest syndrome during TAC than children with HbSS [6].…”
Section: Discussionsupporting
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“…Notably, two of our patients developed multi-organ system failure during their hospitalization, both with HbSC disease and older than 10 years. A clinically severe course of TAC has been described previously in HbSC patients [11] and our cases confirm that parvovirus infection in older children with HbSC may have increased morbidity. Children with HbSC appear to be more likely to develop ASSC and acute chest syndrome during TAC than children with HbSS [6].…”
Section: Discussionsupporting
“…The diagnosis of TTP in this setting could have been overlooked because of the overlapping syndromes of splenic sequestration and vaso-occlusive crisis. 10,11 The patient's clinical picture and rapid response to plasma exchange strongly supported the diagnosis of TTP.…”
Section: Discussionmentioning
“…Lower ADAMTS 13/VWF:Ag ratio were observed in asymptomatic patients compared with healthy controls, which was more pronounced during acute vaso-occlusive crisis. 10 In TTP, the presence of ULVWF is the direct result of severe ADAMTS13 deficiency; elevation of circulating ULVWF in sickle cell disease could be due to presence of continuous endothelial activity. 9 Many patients with sickle cell disease who present with multiorgan failure do not have TTP.…”
Section: Discussionmentioning
“…This patient responded well to RBC exchange transfusion therapy, but some adult HbSC patients do require splenectomy. 8 No indications for surgical splenectomy have been officially outlined for adults with HbSC, though persistent abdominal pain and/or fever not responsive to transfusion in the setting of ASSC, a life-threatening case of ASSC, and recurrent sequestration crises have been proposed. 6 ASSC is a rare event in adults with HbSC disease, but clinicians must be aware of the risk for this complication in this population of patients.…”
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