More efficient exchange of sickle red blood cells can be achieved by exchanging the densest red blood cells: An ex vivo proof of concept study.

BACKGROUND: In sickle cell disease (SCD), red blood cells (RBCs) containing hemoglobin S can be denser than RBCs containing wild-type hemoglobin, especially when dehydrated. We hypothesize that targeting denser RBCs during red blood cell (RBC) exchange for SCD could result in more efficient removal of dehydrated, sickled RBCs and preservation of non-sickled RBCs. STUDY DESIGN AND METHODS: Waste products from RBC exchanges for SCD were used as "simulated patients". One RBC volume was exchanged using ABO-compatible blood. The apheresis instrument was programmed to exchange the entire RBC layer by indicating the hematocrit (control), or the bottom half by indicating the hematocrit was half the hematocrit (experimental), with or without subsequent transfusion. Hemoglobin S levels, and complete blood counts were measured. RESULTS: Hemoglobin S levels were lower after the modified versus control RBC exchange (post-RBC exchange mean 4.96% and 11.27%); total hemoglobin S amounts were also lower (mean 19.27 and 58.29 mL of RBCs). Mean RBC density decreased after the modified RBC exchange by 8.86%. Hematocrit decreased in the modified RBC exchange by 36.37%, with partial correction by direct transfusion following a truncated RBC exchange. CONCLUSIONS: Targeting denser RBCs in RBC exchange enhanced hemoglobin S removal and decreased RBC density. Further development of this ex vivo model could potentially allow for: 1) improved reduction in hemoglobin S levels (allowing for longer periods between RBC exchange or maintained lower levels), or 2) achievement of previous goal hemoglobin S levels with fewer donor units (reducing alloimmunization risk and improving blood utilization).

The thrombotic microangiopathy Registry of North America: A United States multi-institutional TMA network.

The thrombotic microangiopathy (TMA) Registry Network of North America (TRNA) is a collaborative network organized for the purpose of developing a multi-institutional registry and network to conduct clinical studies in a rare patient population. The TRNA was founded in 2013 by four academic medical centers (Columbia University Medical Center, Duke University Medical Center, University of Alabama at Birmingham, and University of Pennsylvania) to develop a national and demographically diverse dataset of patients with TMA. A clinical database was developed by network members using REDCap (Research Electronic Data Capture), a web-based database developed for clinical research. To facilitate rapid Institutional Review Board (IRB) approval at multiple sites, the TRNA utilized IRBshare, a streamlined IRB process to allow patient recruitment and enrollment into the TMA registry. This article reviews the process used to establish the TRNA network and discusses the significance of the first multi-institutional clinical apheresis network developed in the United States. J. Clin. Apheresis 31:448-453, 2016. © 2015 Wiley Periodicals, Inc.

Anaphylactoid-like reactions in a patient with HyperLp(a)lipidemia undergoing LDL apheresis with dextran sulfate adsorption and herbal therapy with the spice turmeric.

Elevated Lipoprotein (a) (Lp(a)) levels are associated with atherosclerosis and are independent risk factors for coronary artery disease and stroke [Ariyo et al., N Engl J Med 2003;349:2108­2115; Price et al., Atherosclerosis 2001;157:241­249]. Low-density lipoprotein (LDL)-apheresis is the most effective therapy for reducing Lp(a) levels [Parker, Chem Phys Lipids 1994;67­68:331­338; Stefanutti et al., Transfus Apher Sci 2010;42:21­26]. Dextran sulfate-cellulose adsorption (Liposorber®) removes both LDL and Lp(a) particles with minimal effect on high-density lipoprotein levels. During the procedure, high levels of bradykinin are generated as the kallikrein-kinin system is activated by contact with the negatively charged dextran-sulfate cellulose [Krieter et al., Artif Organs 2005;29:47­52]. Bradykinin is a potent vasodilator and a substrate of the angiotension converting enzyme (ACE). ACE inhibitors are contraindicated for apheresis procedures because these drugs prevent bradykinin degradation, which causes anaphylatoid reactions characterized by hypotension, bradycardia, dyspnea, and flushing [Owen and Brecher, Transfusion 1994;34:891­894]. Turmeric is a yellow spice that is used as an herbal remedy to treat a myriad of conditions ranging from abdominal pain to pulmonary infections. Scientific investigations of the ethnomedicinal properties of curcumin, the major derivative of turmeric, suggest that this compound has anti-inflammatory, antioxidant, and antineoplastic properties [Lobo et al., J Pharm Pharmacol 2009;61:13­21]. We report a case of a patient undergoing Liposorber® therapy for treatment of hyperLp(a)lipidemia who had three episodes of anaphylactoid-like reactions after starting therapy with the spice turmeric.