The safety and performance of the Spectra Optia apheresis system platelet depletion protocol in patients with elevated platelet counts.

BACKGROUND: Thrombocytosis is a presenting and progressive clinical feature found in multiple disease states. It is characterized by high platelet (PLT) counts (>450 × 109 /L) and can lead to thrombohemorrhagic events. Thrombocytapheresis or platelet depletion (PLTD) can be performed in acutely symptomatic patients suffering from thrombocytosis and may reduce or prevent acute serious complications associated with thrombocythemia thereby enabling patients to receive potentially curative high-dose chemotherapy. METHODS: This report details the results from 2 clinical studies, one conducted in the European Union (EU) and one in the People's Republic of China, assessing the PLTD procedure on the Spectra Optia Apheresis System. The primary objective of both studies was to assess the safety and performance of the PLTD procedure in patients with elevated PLT counts. RESULTS: Data were collected from 56 participants completing 64 PLTD procedures. The mean percent change in PLT count and collection efficiency (CE1) was 55.1% and 68.5%, respectively. In the EU study, 6 participants experienced a total of 9 adverse events (AEs) and in the China study, 44 participants reported a total of 212 AEs. In both studies, the majority of AEs reported were Grade 2 or lower and no serious AEs, unanticipated adverse device effects, or AEs leading to death were reported. CONCLUSIONS: The data collected within these studies indicate that the PLTD procedure is well tolerated and effective at reducing circulating PLTs in patients suffering from thrombocytosis as evaluated by a percent decrease in PLT count, CE1, and AE incidence.

Thrombocytapheresis for acquired von Willebrand syndrome in a patient with essential thrombocythemia and recent multivisceral transplantation.

BACKGROUND: Essential thrombocythemia (ET) is associated with increased risk of bleeding secondary to acquired von Willebrand syndrome (AVWS). Bleeding in ET requires urgent platelet reduction by cytoreductive therapy such as hydroxyurea or thrombocytapheresis. We report on the efficacy and safety of thrombocytapheresis in managing AVWS in a patient with ET and multivisceral transplantation. CASE REPORT: The patient was a 51-year-old female who underwent multivisceral transplantation. Her postoperative course was complicated by bleeding from oral cavity, IV lines, gastrointestinal and upper respiratory tracts as well as vaginal bleeding, which coincided with ET flare with a platelet count of 1512 × 109 /L. Coagulation studies including von Willebrand factor (vWF) antigen and activity, vWF propeptide antigen, and vWF multimer analysis were consistent with AVWS. Hydroxyurea was initiated. However, due to major bleeding, rapidly increasing platelet count, and uncertainty of response to hydroxyurea being given through the enteral tube, thrombocytapheresis was initiated for rapid platelet reduction. The patient tolerated the procedure well. Platelet count was reduced from 1636 × 109 /L to 275 × 109 /L with rapid cessation of bleeding. The patient's condition stabilized over the next few days; however, bleeding recurred with increasing platelet count, which required a second thrombocytapheresis 8 days after the first one. The patient was maintained on hydroxyurea 500 mg twice/day. At 11-month follow-up, she had a normal platelet count and no recurrence of bleeding. DISCUSSION: Thrombocytapheresis is safe and efficient in managing postoperative bleeding due to ET/AVWS in solid organ transplant patients. The procedure can be an adjunct to bridging therapy before response to hydroxyurea is achieved.

Therapeutic thrombocytapheresis in myeloproliferative neoplasms: A single-institution experience.

Thrombocytosis is usually seen in myeloproliferative neoplasms (MPNs) and associated with thrombohemorrhagic complications. Therapeutic thrombocytapheresis using an automatic cell separator can help to achieve prompt platelet count reduction to decrease the rate of thrombotic events. In this study, we report a single center's experience in managing MPN patients with thrombocytapheresis prior to chemotherapy. Thrombocytapheresis procedures were performed in 185 MPN patients with thrombocytosis between January 2016 and June 2017. The median percentage reduction of platelets was 44.5% and the median percentage removal efficiency was 65.2% for 83 procedures where the waste bag was sampled. Procedures were generally well tolerated with few patients having mild adverse events (13 out of 185 patients).

Therapeutic thrombocytapheresis for extreme thrombocytosis after chemotherapy in essential thrombocytosis.

Essential thrombocytosis (ET) is a chronic myeloproliferative neoplasm characterized by the presence of thrombocytosis and it can be complicated by thrombotic and/or hemorrhagic events. Treatment options include low-dose aspirin and cytoreductive agents such as hydroxyurea. In cases of extreme thrombocytosis, therapeutic thrombocytapheresis can be a useful procedure. We present a case of a 61-year-old-man previously diagnosed with CALR-mutated ET, who develop acute myeloid leukemia. When recovering after induction chemotherapy, he developed an extreme thrombocytosis up to 2337 × 109 /L regardless hydroxyurea was started. Two therapeutic trombocytapheresis were performed and anagrelide was added to cytoreductive regimen. Platelet count stabilized around 570 × 109 /L. Both procedures were performed with the Spectra Optia Apheresis System version 11.3 (Terumo BCT) and we decided to use a higher collection preference and lower collection speed than manufacturer's recommendations. Platelet count decreased from 2380 × 109 /L to 1035 × 109 /L in the first procedure and from 1813 × 109 /L to 768 × 109 in the second procedure. Platelet collection efficiency was calculated to be 110.3% and 86.1% in the first and second thrombocytapheresis, respectively. Therapeutic thrombocytapheresis with Spectra Optia is a safe and efficient therapy to treat patients with primary thrombocytosis while effect of cytoreductive agents is attained. Platelet collection efficiency was calculated to be higher than previously reported. We suggest that changes in technical parameters such as a deeper aspiration point and/or lower collection speed may increase procedure's efficiency.

Safety and feasibility of therapeutic platelet depletion with Spectra Optia in a pregnant woman.

INTRODUCTION: Thrombocytapheresis is an alternative treatment beneficial in rare circumstances, when cytoreductive agents are contraindicated, drug therapy gave no response or the expected response would be too slow. Here we present a case of a pregnant woman who underwent 5 thrombocytaphereses using Spectra Optia device to reduce circulating platelets (PLT) count and prepare for Cesarian section. PATIENT CHARACTERISTICS AND PERFORMED TREATMENT: A 39-year-old woman with diagnosed chronic myeloid leukemia (CML) was treated with interferon because of too high PLT count. The treatment was well tolerated but the effect was not satisfactory (PLT count remained high). Because of high risk of bleeding during childbirth, the healthcare providers decided to perform thrombocytapheresis to reduce circulating PLT count below 1000×10E3/µl, and to prepare the patient for a planned Cesarean section. The results are presented as mean±SD. RESULTS: Five therapeutic aphereses procedures were performed, with a Spectra Optia device (TerumoBCT). A mean of 1.3±0.3 total blood volume was processed and we observed a mean PLT drop of 42.3±17.7%. Each apheresis procedure resulted in a PLT level ≤1000×10E3/µl. PLT CE1 was high 50.6±2.6% and reproducible. The white blood cell (WBC) loss was low (18.5%±11.0%). No adverse effects were observed. CONCLUSION: Therapeutic platelet depletion using the Spectra Optia™ Apheresis System can be effective and safe during pregnancy. Thrombocytapheresis procedures were reproducible and Spectra Optia system successfully adjusted settings to each procedure conditions. Thrombocytapheresis seems to be a viable and safe option even in pregnant women.

Guidelines on the Use of Therapeutic Apheresis in Clinical Practice-Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Seventh Special Issue.

The American Society for Apheresis (ASFA) Journal of Clinical Apheresis (JCA) Special Issue Writing Committee is charged with reviewing, updating, and categorizing indications for the evidence-based use of therapeutic apheresis in human disease. Since the 2007 JCA Special Issue (Fourth Edition), the Committee has incorporated systematic review and evidence-based approaches in the grading and categorization of apheresis indications. This Seventh Edition of the JCA Special Issue continues to maintain this methodology and rigor to make recommendations on the use of apheresis in a wide variety of diseases/conditions. The JCA Seventh Edition, like its predecessor, has consistently applied the category and grading system definitions in the fact sheets. The general layout and concept of a fact sheet that was used since the fourth edition has largely been maintained in this edition. Each fact sheet succinctly summarizes the evidence for the use of therapeutic apheresis in a specific disease entity. The Seventh Edition discusses 87 fact sheets (14 new fact sheets since the Sixth Edition) for therapeutic apheresis diseases and medical conditions, with 179 indications, which are separately graded and categorized within the listed fact sheets. Several diseases that are Category IV which have been described in detail in previous editions and do not have significant new evidence since the last publication are summarized in a separate table. The Seventh Edition of the JCA Special Issue serves as a key resource that guides the utilization of therapeutic apheresis in the treatment of human disease. J. Clin. Apheresis 31:149-162, 2016. © 2016 Wiley Periodicals, Inc.

Out with the bad and in with the good; red cell exchange, white cell reduction, and platelet reduction.

Automated techniques for red cell [red blood cell (RBC)] exchange or depletion of malignant cells from the peripheral blood have allowed patients with life-threatening conditions to survive long enough to receive definitive treatment. Examples of such conditions include acute chest syndrome in sickle cell disease (SCD) or acute respiratory insufficiency due to leukostasis in acute leukemia. Conversely, other patients with SCD undergo RBC exchanges on a chronic basis to maintain a reasonable quality of life and prevent another stroke. In this review, we will discuss the techniques as well as indications for RBC exchange, leukocytapheresis, and thrombocytapheresis. To illustrate the uses of these therapeutic apheresis procedures, the authors included a summary of the most common diagnoses that comprise their use.

Managing uncontrolled postsplenectomy reactive thrombocytosis in idiopathic thrombocytopenic purpura: role of thrombocytapheresis.

Reactive thrombocytosis occurs in response to infection, trauma, or surgery. Splenectomy alone accounts for 19% of all possible causes of extreme thrombocytosis. We performed thrombocytapheresis in a young lady with chronic idiopathic thrombocytopenic purpura (ITP) who developed postsplenectomy reactive thrombocytosis. Her post splenectomy platelet count was 227 × 10(6)/ml which elevated to 1623 × 10(6)/ml on the 7th postoperative day. A single thrombocytapheresis procedure reduced her platelet to 403 × 10(6)/ml. She was discharged on the 10th postoperative day and then maintained a count of 204-238 × 10(6)/ml with aspirin. Thrombocytapheresis reduces the platelet count rapidly in thrombocytosis and prevents patients from having thrombotic events. However, such procedures should be performed very meticulously to ensure patient safety.

The effect of thrombocytapheresis on hemogram and biochemistry parameters in patients with essential thrombocytemia.

BACKGROUND: Essential thrombocythemia is one of the chronic myeloproliferative neoplasms characterized by clonal proliferation of myeloid cells with variable morphological maturation and hematopoietic activity.It is characterized by excessive clonal platelet production with a tendency to thrombosis and bleeding. Thrombocytapheresis is the removal of platelets by apheresis techniques. Thrombocytapheresis is generally recommended in patients with essential thrombocythemia with acute, severe thrombotic or hemorrhagic events. METHODS: The study included 39 patients who were diagnosed with essential thrombocythemia, started cytoreductive and aspirin therapy, and underwent thrombocytapheresis due to the development of acute severe thrombotic or hemorrhagic events, diagnosed in the adult hematology clinic of Inönü University Turgut Ozal Medical Center. Hemogram and biochemistry values of the patients were scanned retrospectively. RESULTS: After thrombocytapheresis, a statistically significant difference was found between the first and last measurements of hemoglobin, mean platelet volume, White blood cell, neutrophil, platelet, platelet distribution width, creatine, lactate dehydrogenase, fibronogen and calcium levels of the patients. CONCLUSION: The use of thrombocytapheresis in patients with essential thrombocytosis causes a rapid decrease in platelet values as well as an effect on hemogram and biochemistry parameters. Other hemogram and biochemistry parameters such as platelet value should be monitored in patients.

The role of thrombocytapheresis in the management of extreme thrombocytosis: a 6 years' experience from a tertiary care center.

Background: According to the American Society for Apheresis (ASFA) guidelines, thrombocytapheresis is a Category II indication in symptomatic patients and is a Category III indication when used as secondary or prophylactic treatment. The role of thrombocytapheresis is to prevent untoward complications that might occur even before the cytoreductive agents can exert their action. Methods: A retrospective analysis of patients who underwent thrombocytapheresis between 2012 to 2018 was conducted. Demographic details, complete blood counts, diagnosis and indication for thrombocytapheresis were noted. Results: A total of 12 patients with thrombocytosis were included in the study. The cause of thrombocytosis was primary in 3 (25%) patients and secondary in 9 (75%) patients. The average percentage reduction in platelet count was 47.1% (range 12.3%-65.64%). There was a significant decrease in platelet count, platelet crit, and mean platelet volume after the procedure when compared to pre-procedure. Conclusion: Thrombocytapheresis selectively reduces platelet counts with no effect on other cellular and plasma components. The role of thrombocytapheresis in extreme thrombocytosis is to be considered for an immediate decrease in platelet count and to minimize the risks associated with thrombocytosis.

Thrombocytapheresis in Patient with Essential Thrombocythemia: A Case Report.

Essential thrombocythemia (ET) is one of the myeloproliferative neoplasms, characterized by persistent thrombocytosis, platelets >450,000/µL, and evident clonal abnormalities like JAK2 V617F, MPL, CALR mutation and not fulfilling WHO criteria for MDS, CML, PV, and IDA. Here we report a 24-year-old female who presented with headache and was found to have thrombocytosis with a platelet count of 2,141 × 103/µL, diagnosed as ET as per WHO criteria 2008; she required ICU admission and thrombocytapheresis with a favorable outcome.

Extreme Levels of Platelet Count in Essential Thrombocythemia: Management and Outcome, Report of Two Cases.

Myeloproliferative neoplasms including essential thrombocythemia (ET) is usually caused by somatic mutations in multiple genes, including the JAK2 (most frequently), CALR gene, and MPL. In rare cases, the disease is caused by other mutations such as THPO or TET2 gene; however, around 10-15% with ET might have triple-negative mutations. Here we present 2 cases of ET who were asymptomatic on diagnoses, but found to have extremely high platelet counts as never reported earlier. The management and treatment plan can be a challenging step. The objective is to draw attention to the early introduction of thrombocytapheresis in the management of such patients given its notable outcomes.

The role of thrombocytapheresis in the contemporary management of hyperthrombocytosis in myeloproliferative neoplasms: A case-based review.

Extreme thrombocytosis induces an acquired thrombotic-hemorrhagic diathesis, and left uncontrolled is a harbinger of potentially fatal vascular complications. Currently, cytoreduction with medical therapy remains the mainstay of hyperthrombocytosis management. However, it offers a less-than-ideal option in situations where a rapid reduction in platelets is urgently needed, as in the presence of vital end-organ ischemia or to ameliorate of life-threatening hemorrhage. The role of thrombocytapheresis, or plateletpheresis, in hyperthrombocytosis has become increasingly obsolete given the proactive titration of cytoreductive therapies and early identification and correction of reversible causes of reactive thrombocytosis. Despite its narrowed indications, plateletpheresis continues to offer a valuable temporizing measure in platelet count reduction before cytoreductive agents exert their maximal effect. In this context, it is important for the treating physician to be aware of the symptoms and risks associated with hyperthrombocytosis to inform best clinical practices. In this review, we discuss the role of plateletpheresis in the modern-day management of hyperthrombocytosis in patients with myeloproliferative neoplasms through a case based review of the literature. It becomes apparent throughout the discussion that the decision to perform plateletpheresis should be individualized based upon the clinical scenario, degree of thrombocytosis, available infrastructure and every patient's risk profile.

Procederes de aféresis en el Instituto de Hematología e Inmunología / Apheresis procedures at the Institute of Hematology and Immunology

Introducción: en los últimos decenios el interés por las aféresis se ha incrementado debido a que los avances tecnológicos han puesto a disposición de los profesionales de la salud equipamiento dotado de especificidad, seguridad, efectividad y rapidez para la realización de estos procederes en función de la transfusión y la terapéutica. Objetivo: presentar la experiencia acumulada en el Instituto de Hematología e Inmunología en la realización de los diferentes tipos de aféresis. Métodos: se realizó un estudio descriptivo retrospectivo que incluyó a todos los pacientes a los que se les realizó algún proceder de aféresis, en el período comprendido entre enero de 2008 y diciembre de 2013. Se analizaron las variables: datos generales del paciente, centro de procedencia, modalidad de aféresis empleada, método que se empleó y enfermedades que requirieron el proceder. Resultados: se realizaron 3 332 procederes de aféresis, 1 057 de producción con prevalencia de las tromboféresis que representó el 96,68 por ciento. De las 1 078 aféresis terapéuticas realizadas, el primer lugar correspondió a las plasmaféresis con el 26,5 por ciento, seguida de las exanguinotransfusiones (15,8 por ciento). Se realizaron, además, 1 197 aféresis de células mononucleares y plaquetas para su empleo en la Medicina Regenerativa. Conclusiones: los resultados de este primer trabajo muestran el desarrollo paulatino de las aféresis en la institución, tanto de producción como terapéutica, que por una parte promueven la producción de componentes por aféresis, lo que pone a disposición de médicos y pacientes necesitados de hemocomponentes con mayor calidad y seguridad para el tratamiento sustitutivo; y por otra, aporta información sobre las posibilidades reales de esta alternativa terapéutica para algunas enfermedades(AU)

Introduction: in recent decades, interest in apheresis has increased due to advances in technology that have made them available to health professionals with access to specific equipment, safety, effectiveness and speed to perform these procedures in terms of transfusion and therapeutics. Objective: to present the experience gained at the Institute of Hematology and Immunology in performing different types of apheresis. Methods: adescriptive retrospective study was performed which included all patients who underwent an apheresis procedure between January 2008 and December 2013. Variables were: patient´s general data, the establishment of provenance, method where analyzed apheresis was performed, method used, and diseases requiring the proceeding. Results: 3 332 apheresis proceedings were done : 1 057 for production with thrombopheresis prevalence which ranked 96,68 percent. Of the 1 078 therapeutic apheresis performed, the first place went to plasmapheresis with 26,5 percent, followed by exchange transfusions (15,8 percent); 1 197 apheresis of mononuclear cells and platelets to be used in regenerative medicine were also conducted. Conclusions: the results of this preliminary study show the gradual development in the use of apheresis procedures in our institution, with both production and therapy aims, which not only allows the diffusion of production of components by apheresis which provides physicians and patients in need of blood products with higher quality and security replacement therapy, but also provides knowledge about the actual possibilities for alternative therapy in some diseases(AU)

Apheresis: the Italian experience / Aférese: a experiência italiana

The Italian experience is a long one, beginning with granulocyte collection in the late Bruni R. months of 1972 and progressively expanding to new application and new techniques, many of which Italian in origin and diffusion. This is true for sequestration, multiple Carlier P. component collection, ascitapheresis, dy-platelet collection, but is also true for the new application of known techniques such as cascade, filtration for disorders such as acute Guillain Barr Syndrome, KT, Cyclosporin induced or secondary hypertriglericeridemia, lepromotous vasculitis leptospirosis, hyperacute kidney rejection, autoimmune pure redcell aplasia and many other disorders treated by plasma exchange for the first time in Italy and in general terms in Italy this intermediate level of complexity techniques have found a wide if not enthusiastic acceptance. Twenty-four years later the general appreciation and interest have not modified their impact onto transfusion medicine and Italy continues to be among the leaders also because of the presence in the country of a couple of industries of international excellence involved in apheresis and/or related fields. The presence of Italy in apheresis was marked by the first international Society (ESFH, European Society for Hemapheresis) set up in 1982, with its first meeting organized in Florence the following year and the one of 2001 that will be held in Italy. The Italian presence in apheresis is also marked by the large Italian participation in the international meetings, frequently as invited speakers and chairmen. Furthermore, many well recognized investigators got their training in Italy and Italy is proud of their achievements.

A experiência italiana em aféreses é antiga e teve início nos últimos meses de 1972 e progressivamente se expandiu com novas aplicações e novas técnicas, muitas delas de origem italiana. Isto é real para sequestração, coleção multipla de componentes, citaféreses e coleção de plaquetas, mas também é verdade que novas aplicações como na sindrome de Guillain-Barré, PTT, hipertrigliceridemia secundária a ciclosporina, vasculite lepromatosa, leptospirose, rejeição renal aguda.