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Sickle cell disease (SCD) patients require urgent red cell exchange (RCE) procedures for acute chest syndrome (ACS), demanding a coordinated effort of multiple clinical services. Execution of RCE is a multistep process from the time the procedure is requested to the time the procedure is initiated. A retrospective review of patients with SCD requiring urgent RCE for ACS and stroke from 2012 to 2017 was performed at a centralized hemapheresis service covering a multihospital healthcare system. A total of 30 urgent RCE procedures performed on 28 patients were evaluated. The time required for red blood cell (RBC) preparation was the longest step in the process (median 3.8 hours). Furthermore, RBC preparation time was longer for sickle cell patients with RBC alloimmunization compared with nonalloimmunized patients (8.6 vs 3.8 hours, P = .03). One mortality event occurred in Ab- group. This study identified potentially modifiable factors, which impact the time to implementation of RCE in one service area. It highlights the importance of a structured and coordinated approach for the efficient and timely delivery of this vital treatment modality.

BACKGROUND Currently, low titer A plasma is used on a routine basis in bleeding trauma patients of unknown AB type. Three AB non-bleeding apheresis patients are presented here who safely received a combination of AB and low titer A plasma during therapeutic plasma exchange (TPE). One control AB patient received AB plasma only. METHODS Data was obtained retrospectively on number of procedures, volume replaced, total plasma, and A plasma volumes including hemolysis laboratory data. Average A plasma volume and % of A plasma out of total plasma volume used were calculated. RESULTS Two female AB patients were treated for thrombotic thrombocytopenic purpura (TTP) with TPE and a third female AB patient was treated for microangiopathic hemolytic anemia. Patient 1 received a total of 12 procedures, 10/12 with AB+A plasma (average 916.3 ±84.6 mL). Patient 2 received a total of 12 procedures, 4/12 with AB+A plasma (average 1210.5 ±27.9 mL). Patient 3 received a total of six procedures, four out of six procedures with AB+A plasma (average 1009.8 ±80.3 mL). Patient 4, control, received AB plasma only. Percent of A plasma volume exchanged ranged between 23.8% and 47.8%. Haptoglobin, LDH, hemoglobin, and total bilirubin were monitored and trends were comparable with the control patient. The patients had a negative follow up direct antiglobulin test, adequate platelet recovery and a favorable clinical outcome with treatments. CONCLUSIONS TPE was effectively performed without evidence of increased hemolysis using up to 47.8% of low titer A plasma. This approach can reduce strains on limited supplies of AB plasma whereas providing a vital treatment alternative for AB patients undergoing TPE with plasma replacement.

Apheresis can remove pathogens and mediators that contribute to pathogenic inflammatory responses in diseases not generally considered to be "Hematologic." Erythrocytapheresis can remove intracellular pathogens such as Babesiosis. Plasmapheresis can remove mediators of the inflammatory response in conditions such as sepsis, chronic autoimmune urticaria and malignant pertussis. Leukapheresis can remove potentially harmful leukocytes in Crohn's Disease and malignant pertussis. While apheresis can remove all of these substances, the clinical efficacy and pathophysiologic changes that occur during apheresis in these conditions are largely unknown. Hence, the clinical utility of apheresis in these conditions is largely unknown and research in these areas has the potential to benefit many patients with a variety of diseases.

The American Society for Apheresis (ASFA) conducted a 1 day consensus conference on Thrombotic Thrombocytopenic Purpura (TTP) during its annual meeting in Atlanta, GA, on April 10, 2012. The authors of this article, a subcommittee of ASFA's Clinical Applications Committee, developed several questions with regard to definitions, classification, pathophysiology, diagnosis, management, and future research in TTP. These questions were provided to the seven invited speakers who are the experts in the field of TTP. Two moderators conducted the proceedings of the conference which was attended by more than 100 participants. After each presentation, there was an open discussion that included moderator-selected written questions submitted by the audience. A medical writer-generated transcript of the proceedings as well as each presentation was made available to the authors. Each summary was reviewed and approved by the respective speaker before submission of this article. The subcommittee also developed seven key questions for blinded, electronic polling conducted by the moderators to generate a consensus amongst the speakers. This article includes these presentation summaries as well as results of the electronic poll.

Background: Idiopathic thrombotic thrombocytopenic purpura (TTP) is a rare yet potentially fatal thrombotic microangiopathy. Severe deficiency of ADAMTS13 activity via autoantibody formation has been associated with idiopathic TTP; however, specific triggers are poorly defined. Previous studies have reported seasonal associations of TTP, with warm-weather seasons appearing to have the strongest predilection. We characterized the seasonal distribution of severe ADAMTS13 deficient idiopathic TTP at our multi-hospital institution. Methods: Apheresis records from 2005–2012 were queried for patients with a clinical diagnosis of TTP, accompanying pre-plasmapheresis ADAMTS13 activity

Immune thrombocytopenic purpura (ITP) is an autoimmune disease in which IgG-coated platelets are removed from circulation by the spleen, and platelet production is impaired due to increased thrombopoietin (TPO) clearance. Romiplostim, a novel TPO-mimetic agent, is approved for patients with ITP that are unresponsive to traditional treatments. However, there is little experience when using this drug before splenectomy. We describe herein the case of a young female with chronic ITP who was treated with romiplostim, underwent splenectomy shortly thereafter, and required plateletpheresis for postoperative rebound thrombocytosis with concomitant neurologic symptoms. J. Clin. Apheresis 28:321–324, 2013. © 2013 Wiley Periodicals, Inc.

Intravascular hemolysis and resulting plasma-free hemoglobin elevation has been associated with acute kidney injury via several mechanisms. Proximal tubular injury due to iron compound deposition and nitric oxide sequestration with subsequent impairment of the microcirculation and hypoperfusion are specific adverse effects of intravascular hemolysis that may contribute to acute kidney injury. Therefore, removal of plasma-free hemoglobin and restoration of normal nitric oxide vasodilatory mechanisms may play a beneficial role in treating acute kidney injury secondary to acute intravascular hemolysis. We report herein the case of a 13-year-old patient with severe intravascular hemolysis and acute kidney injury and describe the role of plasma exchange with plasma replacement in his successful recovery. J. Clin. Apheresis 27:274–277, © 2012 Wiley Periodicals, Inc.

Background: Fulminant sepsis-induced multisystem organ failure (MSOF) in pediatric patients carries substantial morbidity and mortality. Therapeutic plasma exchange (TPE) has been reported to be beneficial in sepsis-induced MSOF. We evaluated the outcomes of previously healthy children with fulminant sepsis-induced MSOF receiving TPE. Materials and Methods: Previously healthy pediatric ICU patients who underwent TPE for MSOF due to fulminant bacterial sepsis were retrospectively reviewed. Eleven patients (three females and eight males) with age ranging 8 months to 14 years were identified (eight meningococcemia and three other infections). All patients received daily TPE with fresh frozen plasma (FFP) as replacement fluid. Organ failure index (OFI—maximum score = 6) was assessed daily for 7 days. Results: A median of 4 TPE (1–14) were performed. Improvements in organ function and platelet count occurred in most patients with 2–4 TPE treatments. All 10 patients who were alive had reduced OFI to 2 by day 7 of initial TPE and were all fully recovered (survival rate = 10/11, 91%). The only death occurred in a patient who died the same day after his first TPE treatment, which was initiated 24 h after development of MSOF. The 10 survivors underwent early initiation of TPE (median 5.3 h) after the onset of MSOF. Conclusions: > TPE may contribute to a better outcome in previously healthy pediatric patients with fulminant sepsis-induced MSOF, especially if instituted early in the course of multiorgan failure. J. Clin. Apheresis, 2011. © 2011 Wiley-Liss, Inc.

Apheresis can remove pathogens and mediators that contribute to pathogenic inflammatory responses in diseases not generally considered to be "Hematologic." Erythrocytapheresis can remove intracellular pathogens such as Babesiosis. Plasmapheresis can remove mediators of the inflammatory response in conditions such as sepsis, chronic autoimmune urticaria and malignant pertussis. Leukapheresis can remove potentially harmful leukocytes in Crohn's Disease and malignant pertussis. While apheresis can remove all of these substances, the clinical efficacy and pathophysiologic changes that occur during apheresis in these conditions are largely unknown. Hence, the clinical utility of apheresis in these conditions is largely unknown and research in these areas has the potential to benefit many patients with a variety of diseases.

Idiopathic thrombotic thrombocytopenic purpura (TTP) is a rare yet potentially fatal thrombotic microangiopathy. Severe deficiency of ADAMTS13 activity via autoantibody formation has been associated with idiopathic TTP; however, specific triggers are poorly defined. Previous studies have reported seasonal associations of TTP, with warm-weather seasons appearing to have the strongest predilection. We characterized the seasonal distribution of severe ADAMTS13 deficient idiopathic TTP at our multi-hospital institution.Apheresis records from 2005-2012 were queried for patients with a clinical diagnosis of TTP, accompanying pre-plasmapheresis ADAMTS13 activity10%, and no other explanations for thrombotic microangiopathy. Date of admission and ADAMTS13 activity were collected. Both initial episodes and relapses in patients whose initial episodes occurred since 2005 were included in the analysis. Goodness-of-fit chi-square analysis was performed and statistical significance was defined as p0.05.Of 237 consecutive new patients with a suspected clinical diagnosis of TTP, 73 patients met inclusion criteria and had a total of 110 unique presentations for either initial TTP episodes or TTP relapses. Sixteen patients had 37 relapses of their idiopathic TTP (range 1-7). No statistically significant seasonal or monthly associations were identified with either initial or any TTP episodes.A seasonal association of severe ADAMTS13 deficient idiopathic TTP was not observed. Unknown variables may explain previous observations of seasonal associations of idiopathic TTP in other regions of the United States. Thus, individual institutions should characterize their own seasonal distributions of idiopathic TTP as part of the exploration of possible disease triggers in their respective areas.

Immune thrombocytopenic purpura (ITP) is an autoimmune disease in which IgG-coated platelets are removed from circulation by the spleen, and platelet production is impaired due to increased thrombopoietin (TPO) clearance. Romiplostim, a novel TPO-mimetic agent, is approved for patients with ITP that are unresponsive to traditional treatments. However, there is little experience when using this drug before splenectomy. We describe herein the case of a young female with chronic ITP who was treated with romiplostim, underwent splenectomy shortly thereafter, and required plateletpheresis for postoperative rebound thrombocytosis with concomitant neurologic symptoms.

Fulminant sepsis-induced multisystem organ failure (MSOF) in pediatric patients carries substantial morbidity and mortality. Therapeutic plasma exchange (TPE) has been reported to be beneficial in sepsis-induced MSOF. We evaluated the outcomes of previously healthy children with fulminant sepsis-induced MSOF receiving TPE.Previously healthy pediatric ICU patients who underwent TPE for MSOF due to fulminant bacterial sepsis were retrospectively reviewed. Eleven patients (three females and eight males) with age ranging 8 months to 14 years were identified (eight meningococcemia and three other infections). All patients received daily TPE with fresh frozen plasma (FFP) as replacement fluid. Organ failure index (OFI-maximum score = 6) was assessed daily for 7 days.A median of 4 TPE (1-14) were performed. Improvements in organ function and platelet count occurred in most patients with 2-4 TPE treatments. All 10 patients who were alive had reduced OFI to 2 by day 7 of initial TPE and were all fully recovered (survival rate = 10/11, 91%). The only death occurred in a patient who died the same day after his first TPE treatment, which was initiated 24 h after development of MSOF. The 10 survivors underwent early initiation of TPE (median 5.3 h) after the onset of MSOF.TPE may contribute to a better outcome in previously healthy pediatric patients with fulminant sepsis-induced MSOF, especially if instituted early in the course of multiorgan failure.

The rationale for immunosuppressive therapy of thrombotic thrombocytopenic purpura (TTP) was established by observations that TTP may be caused by autoantibodies to ADAMTS13. Patients with high-titer autoantibodies to ADAMTS13 may have a higher mortality, and survivors may require prolonged plasma exchange therapy in spite of adjunctive glucocorticoid treatment. More intensive immunosuppressive therapy with rituximab may provide benefit for many of these patients. The Transfusion Medicine/Hemostasis Clinical Trials Network is developing a randomized, clinical trial to test the hypothesis that addition of rituximab to standard treatment of TTP with plasma exchange and glucocorticoids will decrease initial treatment failure rates as well as subsequent relapses over the following 3 years. To provide the background data for this clinical trial, a systematic review of all published reports on rituximab treatment of immune-mediated disorders was performed. Twelve articles have reported 27 patients treated with rituximab for TTP, with benefit described in 25 (93%) of the patients. Additional reports have described rituximab treatment of 37 other immune-mediated disorders, with clinical response in most patients. These observations from small uncontrolled case series provide the background and rationale for a randomized clinical trial to establish the role of rituximab in the management of patients with TTP.