The current results demonstrate that the remote blood-release system is safe and useful for improving the efficiency of blood issue for patients in remote operating theatres.
13 previously untreated patients with poor prognosis non‐Hodgkin's lymphoma (NHL) underwent high‐dose therapy followed by autologous bone marrow transplantation (ABMT). All patients experienced a great cytoreductive effect and 9 of them reached a complete remission (mean duration 32 months). The best results were observed in patients with more limited disease and in those without symptoms. 7 patients still remain in complete unmantained remission 15–46 months from the transplant. The probability of survival is 74% at 46 months. No therapy‐related deaths were recorded. In differentiating our preliminary approach, we propose high dose therapy followed by ABMT as induction phase in patients with stage II and as consolidation after first line therapy in patients with stages III‐IV. Further studies are warranted to determine which type of lymphoma may benefit more and which conditioning regimens may improve the remission rate.
Background
Clarkson disease (monoclonal gammopathy-associated idiopathic systemic capillary leak syndrome, ISCLS) is a rare idiopathic condition marked by transient, relapsing-remitting episodes of systemic microvascular hyper-permeability, which liberates plasma fluid and macromolecules into the peripheral tissues. This pathology manifests clinically as the abrupt onset of hypotensive shock, hemoconcentration, and hypoalbuminemia.
Methods
We analysed endothelial glycocalyx (eGCX)-related markers in plasma from patients with ISCLS during acute disease flares and convalescence by ELISA and comprehensive proteomic profiling. We evaluated eGCX-related components and gene expression in cultured endothelial cells using RNA-sequencing, real-time PCR, and fluorescence staining.
Results
Serum levels of eGCX-related core components including hyaluronic acid (HA) and the core proteoglycan soluble syndecan-1 (sCD138) were elevated at baseline and during acute ISCLS flares. Serial measurements demonstrated that sCD138 levels peaked during the recovery (post-leak) phase of the illness. Proteomic analysis of matched acute and convalescent ISCLS plasma revealed increased abundance of eGCX-related proteins, including glypicans, thrombospondin-1 (TSP-1), and eGCX-degrading enzymes in acute compared to remission plasma. Abundance of endothelial cell damage markers did not differ in acute and baseline plasma. Expression of several eGCX-related genes and surface carbohydrate content in endothelial cells from patients with ISCLS did not differ significantly from that observed in healthy control cells.
Conclusions
eGCX dysfunction, but not endothelial injury, may contribute to clinical symptoms of acute ISCLS.
Serum levels of of eGCX components including sCD138 may be measured during acute episodes of ISCLS to monitor clinical status and therapeutic responses.
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