Aortitis is an under-recognized manifestation of antiphospholipid syndrome: A case report and literature review.

Aortitis is a classic manifestation of large vessel vasculitis. Antiphospholipid syndrome (APS), sometimes known as Hughes syndrome, is an acquired autoimmune disorder that manifests clinically as recurrent venous or arterial thrombosis. Patients with APS may also suffer from various underlying diseases, most frequently systemic lupus erythematosus (SLE). Catastrophic antiphospholipid syndrome (CAPS) is a rare but serious complication of APS characterized by failure of several organs due to diffuse microcirculatory thrombi. Its main manifestations involve the kidneys, lungs, heart and central nervous system, and require early diagnosis and rapid therapeutic management. While APS can affect virtually any blood vessel, aortitis is not a known symptom of APS. We report the case of a 36-year-old patient with APS and SLE who presented with CAPS during pregnancy, with no concomitant SLE flare. The first manifestation of CAPS was aortitis, preceding renal, cardiac and haematological manifestations. The outcome was favourable with combined treatment including corticosteroids, anticoagulants, plasma exchange and rituximab. We then carried out a literature search for papers describing the presence of aortitis in APS and/or SLE. In the cases of aortic involvement identified in the literature, including another case of CAPS, the occurrence of aortitis in SLE, often associated with the presence of antiphospholipid antibodies/APS, suggests that aortitis should be considered as an under-recognized manifestation and potential non-criterion feature of APS.

Efficacy and safety of plasma exchange using a double viral inactivated and prion reduced solvent/detergent fresh frozen plasma for the treatment of thrombotic microangiopathy: The first French experience in a single center.

BACKGROUND: Octaplas LG® is the first plasma with marketing authorisation, available in France only since February 2016. This is a double viral inactivated and prion reduced solvent/detergent fresh frozen plasma. Clinical data on Octaplas LG® use in thrombotic microangiopathy (TMA) remains very limited. In May 2017, we were the first hospital in France to benefit of this new plasma product now dispensed by hospital pharmacies. We present a prospective review of all therapeutic plasma exchange (TPE) procedures for TMA patients in our hospital to evaluate the new delivery circuit, the efficacy and the adverse events (AE) related to this plasma. STUDY DESIGN AND METHODS: We prospectively reviewed 166 TPE procedures where Octaplas LG® was used as replacement fluid in 15 consecutive TMA patients required TPE in our hospital from May 2017 until December 2018. RESULTS: The total replacement plasma volume administered was 763 L (3818 units) with a median on 32 L (range 6-157) per episode. Remission was achieved in all cases after a median of 7 TPE per patient's episode. No exacerbation nor relapse were noted. One patient presented a grade 1 citrate reaction, and another patient an allergic reaction. We deplored pulmonary embolism in 2 patients. CONCLUSION: In our experience OctaplasLG® was well-tolerated and was effective at inducing a full clinical remission. Although two PE were noted, the relationship to OctaplasLG® in unclear. The new dispensing circuit through the hospital pharmacy has proven to be safe and efficient.

Automated red blood cell depletion: Results from a validation trial of the Fenwal Amicus new red blood cell depletion program.

PURPOSE: A new software release of the Fenwal Amicus device is now available for red blood cell (RBC) apheresis, offering RBC exchange, RBC depletion (RBCd)/RCB exchange, and RBCd procedures. The main goal of this study was to validate the new RBCd program through the accuracy of the patient's postprocedure hematocrit (HCT) (actual end HCT) to the HCT reported by the device at the end of the procedure (target end HCT). Secondary objectives were to assess the device-related significant adverse events, the patient's cellular losses, and the degree of device induced hemolysis. METHODS: The study was an open-label, single-center, prospective trial in adult patients eligible for RBCd. Patients were enrolled between October 2015 and June 2016. In all procedures, saline was used as replacement fluid. Blood samples were collected from the patient before and after the procedure. Device settings, adverse events, and procedure results were recorded. RESULTS: Thirty-nine patients were enrolled (34 treated for iron storage disease and 5 for polycythemia vera); only 36 were evaluable for the primary objective. The mean actual end HCT was 34.6 ± 2.79% and the mean target end HCT was 35.8 ± 2.25%. The ratio (actual end to target end HCT) was 0.97 ± 0.05 with a 95% CI demonstrating the accuracy of the target HCT. One patient experienced a vasovagal event not related to the device. Cellular losses were not clinically relevant. CONCLUSION: The RBCd Amicus program met all RBCd efficiency and safety requirements with high accuracy of the target HCT.

Comparative evaluation of the depletion-red cell exchange program with the Spectra Optia and the isovolemic hemodilution-red cell exchange method with the COBE Spectra in sickle cell disease patients.

BACKGROUND: This study aims to compare in patients with sickle cell disease (SCD), the technical performance and packed red blood cell unit consumption between the automated depletion/Red Blood Cell exchange (RBCx) program (Spectra Optia Apheresis System) with the isovolemic hemodilution (IHD)/RBCx procedure (COBE Spectra Apheresis System) in a routine clinical setting. METHODS: We retrospectively reviewed the data of 23 patients treated between October 2010 and August 2013 who underwent repeated RBCx on both apheresis systems for preventive indications. Each patient was their own control and had undergone two procedures on each system, totaling 46 sessions per group. On Spectra Optia, we performed the automated depletion/RBCx program. For COBE Spectra, we used a modified IHD/RBCx protocol. All patients had an initial 250 mL depletion offset by a 5% albumin prior to the exchange procedure, for the respective device, with leucodepleted Rh/Kell compatible and cross-matched RBC packs. RESULTS: All procedures were well tolerated except three mild febrile nonhemolytic reactions. Postprocedure hemoglobin S (HbS), fraction of cells remaining (FCR), procedure duration and processed blood and anticoagulant volumes were comparable in the two groups. However, the RBCx volume was significantly higher for the Spectra Optia group (+71 mL, P = 0.01), with no significant difference in the number of RBC units used. CONCLUSIONS: Technical performance and packed RBC unit consumption were not compromised when switching from the COBE Spectra IHD/RBCx protocol to the depletion/RBCx protocol on the Spectra Optia. Tolerability was equal for both protocols. J. Clin. Apheresis 31:429-433, 2016. © 2015 Wiley Periodicals, Inc.