Human Neutrophil Antigen frequency data for Malays, Chinese and Indians
(Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - October 4, 2019 Category: Hematology Authors: Che Ghazali Norul Hajar, Zulkifli Zefarina, Nor Suhaila Md. Riffin, Tuan Hulwani Tuan Mohammad, Sabreena Safuan, Bryan Raveen Nelson, Mohd Tajuddin Abdullah, Geoffrey Keith Chambers, Hisham Atan Edinur Source Type: research

Therapeutic Plasma Exchange in Neurological Disorders: Experience From A Tertiary Neuroscience Centre
Therapeutic plasma exchange (TPE) involves the extracorporeal separation of plasma from the cellular components of blood with replacement fluid, such as human albumin or fresh frozen plasma. A number of studies across the world revealed that more than one third of TPE procedures were performed for neurological disorders.Myasthenia gravis (MG), Guillain-Barr é syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP) were the most frequently cited indications for TPE, followed by multiple sclerosis (MS). (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - October 4, 2019 Category: Hematology Authors: Joyutpal Das, Vanisha D. Chauhan, Daniel Mills, Nicholas J. Johal, Maevis Tan, Rachael Matthews, Ryan Keh, James B Lilleker, David Gosal, Nazar Sharaf Source Type: research

Exchange Transfusion in Neonatal Hyperbilirubinemia: A Single Centre Experience from Northern India
To determine the indication, efficacy and adverse events related to exchange transfusion (ET) with reconstituted blood (RB) in neonatal hyperbilirubinemia (NNH). (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - October 4, 2019 Category: Hematology Authors: Brinda Kakkar, Soma Agrawal, Mohit Chowdhry, P.J. Muthukumaravel, Raj Nath Makroo, Uday K. Thakur Tags: Original article Source Type: research

Thymoma and pure red cell aplasia with hypoplasia of megakaryocytopoiesis: a rare and life-treating condition
Thymic tumors are rare diseases with an incidence of 0.15 cases per 100,000 person-years. They can be associated with a variety of other syndromes, such as Myasthenia Gravis or autoimmune disorders. Among them, pure red cell aplasia is a hemato-pathological condition characterized by anemia, reticulocytopenia and erythroid cell hypoplasia of bone marrow. Here, we reported a case of a 62-year-old female with a long history of neurologic symptoms due to Myasthenia Gravis. She was diagnosed with thymoma, with mediastinal mass and pleural thickening. (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - October 4, 2019 Category: Hematology Authors: Giorgio Lo Iacono, Federica Gigli, Lorenzo Gherzi, Daniele Avenoso, Stefano Fiori, Giulia Sedda, Corrado Tarella, Lorenzo Spaggiari Source Type: research

Transfusion management in multiple myeloma patients receiving daratumumab: experience of a single tertiary care centre
(Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - October 4, 2019 Category: Hematology Authors: Pilar Solves, Susana Tur, Mario Arnao, Carmen Freiria, Lara Dominguez, M Jes ús Pons, Inés Gómez, GF Sanz, N Carpio Source Type: research

Direct Oral Anticoagulant (DOAC) Reversal: Andexxa Versus 4 Factor Prothrombin Concentrates
(Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - October 1, 2019 Category: Hematology Authors: Minh-Ha Tran, Brian Knight, Anh Hoang Duong Source Type: research

HLA-DRB1*01:01, but not HLA-DRB1:1503 or HLA-DRB1*11, is associated with decreased inhibitor risk in Iranian hemophilia A patients
Hemophilia A is a genetic disorder through which patients suffer from recurrent bleeding. This can be caused by a defect in human plasma coagulation Factor VIII. High incidence of FVIII inhibitors in some severe hemophilia A patients after FVIII therapy is a considerable complication. Determination of good predictive factors can improve the safety of this treatment. HLA-II have been shown as a predictive element for inhibitor development. The goal of this study is to determine the association between HLA-DRB1*15:03, HLA-DRB1*11 and HLA-DRB1*01:01 alleles and FVIII inhibitors in severe hemophilia A patients in Iran. (Source...
Source: Transfusion and Apheresis Science - September 16, 2019 Category: Hematology Authors: S. Hosseini, S. Arabi, F. Yari, A. Pourfatollah, N. Rezaie, S. Moazezi, A. Aghaie Source Type: research

Hospital admission for blood collection for the preparation of autologous serum eye drops
(Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - September 13, 2019 Category: Hematology Authors: Pauline Khoo, Mondy Phillip, Peter McCluskey, Franzco Stephanie Watson Source Type: research

Study for the diagnostic screening of paroxsymal nocturnal hemoglobinuria in Turkey: Prospective multicentric evaluation of suspected patients
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disease presenting with variable and various clinical findings. PNH might be overlooked and diagnosis may be delayed due to low awareness about PNH. This is the first multicenter study in Turkey, investigating the efficiency of diagnostic screening of PNH by multiparameter flow cytometry (FCM) according to consensus guidelines. (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - September 6, 2019 Category: Hematology Authors: Osman Ilhan, Zehra Narli Ozdemir, Gulsum Ozet, Mesude Falay, Mustafa Yenerel, Tulin Tuglular, Mehmet Turgut, Birol Guvenc, Ali Unal, Orhan Ayy ıldız, Neslihan Andic, Abdullah Hacıhanefioğlu, Fahri Sahin, Mehmet Sencan, Ridvan Ali, Guner Hayri Ozsan, R Source Type: research

The Direct Labor Cost for Providing Life-Saving Blood to Air Medical Transport for Pre-Hospital Transfusion
Studies have shown that pre-hospital transfusions of packed red blood cells (pRBCs) are associated with increased survival rates within the first 24  hours, a decrease in the odds of stroke, and a reduction in the number of pRBCs needed within the first 24 hours following a Level 1 trauma activation [1]. Recently, we have shown [2] the logistical and blood product variability utilized in the pre-hospital medical transport, but the financial implication for the diagnostic laboratory to maintain such a program remains unknown. (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - September 6, 2019 Category: Hematology Authors: Penny C. Szklarski, Garrett S. Booth Source Type: research

Clinical impact of massive transfusion protocol implementation in non-traumatic patients
Massive transfusion protocol (MTP) has been used to provide plasma and packed red blood cells (pRBCs) rapidly. MTP also has been adapted for non-traumatic patients. The effects of hospital-wide MTP implementation on clinical outcomes were reviewed. (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - September 5, 2019 Category: Hematology Authors: Kyoung Won Yoon, Duck Cho, Dae-Sang Lee, Eunmi Gil, Keesang Yoo, Kyoung Jin Choi, Chi-Min Park Source Type: research

Hereditary protein C deficiency caused by novel compound heterozygous mutants in a Chinese pedigree: A case report
We report a pedigree who suffered from the hereditary PC deficiency with compound heterozygous mutants; genetic analysis revealed compound heterozygous mutations of 262  G > T(Asp88Tyr) and 400 + 5G > A that were identified in the proband; moreover, Asp88Tyr and 400 + 5G > A were also detected in the father and the mother, respectively. (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - September 5, 2019 Category: Hematology Authors: Yi Li, Ruimin Cai, Wenyang Wang, Qiang Feng, Xue Gao, Ping Liu, Meirong Liu, Chunling Qi Source Type: research

Correlation among automated scores of agglutination, antigen density by flow cytometry and genetics of D variants
Laboratory testing to identify the molecular basis of serologic weak D phenotypes is recommended to determine whether a pregnant woman or potential transfusion recipient should be managed as RhD-positive or RhD-negative. The variation in D antigen expression on RBCs, different potencies of anti-D typing reagents, lack of standardized test methods, and the subjectivity of interpreting agglutination reactions complicate the detection of D variants. We evaluated the correlation of agglutination scores by an automated immunoassay analyzer with D antigen densities determined by flow cytometry, and D variant types identified by ...
Source: Transfusion and Apheresis Science - September 5, 2019 Category: Hematology Authors: Carine Prisco Arnoni, Jana ína Guilhem Muniz, Tatiane Aparecida de Paula Vendrame, Rosangela de Medeiros, Afonso José Pereira Cortez, Flavia Roche Moreira Latini, Lilian Castilho Source Type: research

A university – led initiative to promote voluntary non-remunerated blood donation in a developing country
To describe the process, challenges and impact of developing a voluntary non-remunerated blood donor programme in a replacement- based blood donation system. (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - September 5, 2019 Category: Hematology Authors: Kenneth S. Charles, Melissa Friday, Deepak Lall, Kasturi Harrichan, Melina De Four, Kendra Guy, Andre Earle, Rawlins D, Andrew D. Chantry Source Type: research

Factors that contribute to blood donation behavior among primary healthcare users: a structural approach
This study aimed to evaluate the relative contribution of psychosocial variables to donation behavior in a sample of primary healthcare users in a Brazilian municipality. Donor recruitment and retention continue to pose significant challenges to blood collection services worldwide and is especially important among low and middle income countries (LMICs), challenged with higher rates of stigma and low public awareness about donation. A cross-sectional study with randomized stratified sampling of 1,055 primary healthcare users was conducted across 12 healthcare facilities in Ribeir ão Preto, state of São Paulo....
Source: Transfusion and Apheresis Science - September 5, 2019 Category: Hematology Authors: Miriane L. Zucoloto, Thelma T. Gon çalez, Philippe T. Gilchrist, Brian Custer, Willi McFarland, Edson Z. Martinez Source Type: research

Therapeutic apheresis during pregnancy: a single center experience
. Therapeutic apheresis (TA) represents a treatment option for pre-existing conditions or diseases occurring during gestation. Although pregnancy is not a contraindication per se, due to the lack of evidence-based guidelines and presumed risk of maternal/fetal adverse events there is a general resistance to its application. (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - September 5, 2019 Category: Hematology Authors: Anna Colpo, Piero Marson, Francesca Pavanello, Tiziana Tison, Maria Teresa Gervasi, Alessandra Zambon, Amelia Ruffatti, Giustina De Silvestro, Ariela Hoxha Source Type: research

Iron overload and malignancies in patients with haemoglobinopathies: a single center experience
Thalassemias and sickle cell disease are a group of inherited blood disorders caused by alterations of the synthesis or of the structure of hemoglobin chains. It results in variable outcomes ranging from severe anemia to clinically asymptomatic individuals. Hemolysis and transfusions therapies lead to iron overload and, thus, to an high production of reactive oxygen species (ROS). Recently, it was found an increasing frequency of tumors in patients with hemoglobinopathies and it was underlined the probable role of iron overload in the carcinogenesis. (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - September 5, 2019 Category: Hematology Authors: Luciana Rigoli, Annamaria Petrungaro, Chiara Di Bella, Rosario Caruso Source Type: research

Evaluation of health and quality of life in apheresis patients – data from the waa register
: The World Apheresis Association (WAA) register contains data from more than 89 000 apheresis procedures in more than 12000 patients. The aim of this study was to evaluate functional health and quality of life (QoL) in patients during apheresis treatment. (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - September 5, 2019 Category: Hematology Authors: J Goto, E Newman, V Witt, D Deeren, M Blaha, M Lanska, Z Gasova, Z Bhuiyan-Ludvikova, R Hrdlickova, W Ramlow, H Prophet, G Liumbruno, A Griskevicius, J Audzijoniene, H Vrielink, A Aandahl, S Ortega Sanchez, V Strineholm, G Berlin, M M örtzell-Henriksson, Source Type: research

Repeat Exchange Transfusion for Treatment of Severe Babesiosis
We report a case of severe babesiosis presenting with 43% parasitemia in a 73-year-old splenectomized woman on etanercept for rheumatoid arthritis. She initially was treated aggressively with clindamycin and quinine and exchange transfusion. Despite a post-exchange drop in parasitemia to 7.6%, it rebounded to 11.4% on hospital day 5 accompanied by new onset high fevers and hypoxia. She improved after a second exchange transfusion and ultimately resolved her infection after 12 weeks of antibabesial antibiotics. (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - September 5, 2019 Category: Hematology Authors: Christopher Radcliffe, Peter J. Krause, Matthew Grant Source Type: research

Severe drug-induced hypertriglyceridemia treated with plasmapheresis in children with acute lymphoblastic leukemia
Asparaginase (ASP) and steroids are a main part of treatment for ALL, in both front-line and relapse setting. It is known, that ASP can cause several toxicities such as hypersensitivity, pancreatitis, as well as severe lipid and coagulation disturbances. Administered steroids can result in diabetes, obesity, hyponatremia and also mild hyperlipemia, which can intensify side effects of asparaginase. When triglyceride elevation is greater than 1000  mg/dl, the risk of pancreatitis is significantly increased. (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - September 5, 2019 Category: Hematology Authors: Joanna Zawitkowska, Monika Lejman, Agnieszka Zaucha-Pra żmo, Natasza Sekuła, Teresa Greczkowska-Chmiel, Katarzyna Drabko Source Type: research

An update of the transfusion-related acute lung injury (TRALI) definition
(Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - September 5, 2019 Category: Hematology Authors: Alexander P.J. Vlaar, Steve Kleinman Source Type: research

Determinants of blood transfusion for elective surgical procedures at Tikur Anbessa Specialized Hospital, Addis Ababa, Ethiopia, 2017
Till date 80% of the world's population has access to only 20% of the world's safe blood that is properly collected and tested. Paradoxically, despite a severely inadequate supply, blood is often transfused unnecessarily and it will expose patients to complications. Despite lots of publication and investigation on the efficiency of blood usage, there is no clear demarcation which patients really need blood transfusion. (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - September 5, 2019 Category: Hematology Authors: Mekuriaw Mesfin Birhan, Gebrehiwot Asfaw Source Type: research

A case report on anti-mia antibody in a multi-transfused patient from india
The MNSs blood group system has 46 antigens identified and owes its complexity to the recombination between closely linked homologous genes. GP.Mur (previously Mi.III, Miltenberger series) is one such phenotype formed due to a hybrid gene of GYPB. The corresponding antibody is called as anti-Mia antibody. [1] (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - September 5, 2019 Category: Hematology Authors: Soma Agrawal, Mohit Chowdhry Source Type: research

Women and inherited bleeding disorders – A review with a focus on key challenges for 2019
The area of women and inherited bleeding disorders has undergone quick expansion in recent years. More patients are being identified and expertise to diagnose and manage these patients is now essential for practising physicians. Programs to help educate and empower patients and caregivers are now in place. Common inherited bleeding disorders affecting women include von Willebrand disease (VWD), inherited platelet disorders, and rare inherited bleeding disorders such as factor VII (FVII) deficiency and factor XI (FXI) deficiency. (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - August 9, 2019 Category: Hematology Authors: R. Winikoff, M.F. Scully, K.S. Robinson Source Type: research

Patient testimonial: A caregiver whose son with inhibitors has been receiving emicizumab
Canadian Hemophilia Society Medical/Scientific Symposium, Rendez-vous May 24, 2019 - 9:10am to 9:20am (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - August 9, 2019 Category: Hematology Authors: David Page Source Type: research

The Evolution of Recombinant Factor Replacement for Hemophilia
Hemophilia A and Hemophilia B are the most common of the severe bleeding disorders and are caused by a deficiency in blood clotting factor VIII or factor IX respectively. Factor replacement therapy has been the cornerstone of treatment to treat life threatening bleeds and prevent joint disease. The treatment of hemophilia has evolved tremendously over the past five decades from fresh frozen plasma as the only available therapy to more specific plasma-derived and recombinant-derived factor replacement. (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - August 9, 2019 Category: Hematology Authors: Amanda D. Sankar, Angela C. Weyand, Steven W. Pipe Source Type: research

Women and inherited bleeding disorders- A review with a focus on key challenges for 2019
After a landmark study reporting on the challenges faced by women with bleeding disorders was published in 1998 [1], this important clinical challenge began to receive more attention in the wider medical community. Obstetricians and gynecologists were long focused on both gynecologic and obstetric causes of bleeding as were all those involved in blood banking in hospitals with maternity units. Specifically, pediatric and adult hematologists became more aware of the challenges of women living with inherited bleeding disorders and interest grew in the hereditary bleeding disorders community. (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - August 9, 2019 Category: Hematology Authors: R. Winikoff, M.F. Scully, S. Robinson Source Type: research

Predictive factors for stem cell mobilization failure in multiple myeloma patients: A single center experience
High-dose chemotherapy followed by autologous stem cell transplantation (ASCT) has been considered the standard of treatment care for patients with multiple myeloma (MM). Insufficient mobilization and harvest of peripheral stem cells can be a major obstacle for performing ASCT. This is resulting in a lacking opportunity of cure in patients with MM. The aim of this study was to evaluate the factors which influence mobilization failure in patients with MM. (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - August 8, 2019 Category: Hematology Authors: Hakan Goker, Rafiye Ciftciler, Haluk Demiroglu, Mehmet Turgut, Nilgun Say ınalp, IC Haznedaroglu, Mufide Okay, Fatma Tekin, Yahya Buyukasık Source Type: research

Introduction to the third part of a three-part theme edition on Hereditary Bleeding Disorders
This is an exciting time for patients, families and health-care providers dedicated to the field of Hereditary Bleeding Disorders, and it has been a real honour to work as a Guest Editor for a theme edition of the Journal of Transfusion and Apheresis Science, which ultimately spanned three parts. Topics were chosen with advice from key leaders in this field, who were invited to either mentor junior colleagues or to write papers themselves. The response to these invitations has been very generous. (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - August 8, 2019 Category: Hematology Authors: Frances Scully Source Type: research

Inherited disorders of the fibrinolytic pathway
Deficiencies or excessive activation of the  fibrinolytic system can result in severe, lifelong bleeding disorders. The most severe clinical phenotype is caused by α2−Antiplasmin (α2−AP) deficiency which results in excess fibrinolysis due to the inability to inhibit plasmin. Another bleeding disorder due to a defect in the fibrinolyti c pathway results from Plasminogen activator inhibitor-1 (PAI-1) deficiency causing enhanced fibrinolysis due to the decreased inhibition of plasminogen activators resulting in increased conversion of plasminogen to plasmin. (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - August 8, 2019 Category: Hematology Authors: Shilpa Jain, Suchitra S. Acharya Source Type: research

Patient Testimonial: My Experience on a Gene Therapy Trial
I am a participant in the Children ’s Hospital of Philadelphia’s (CHOP) Gene Therapy Trial for Hemophilia B (SPK 9001). (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - August 8, 2019 Category: Hematology Authors: John Konduros Source Type: research

A personalized approach to the management of VWD
The goal of treating Von Willebrand Disease (VWD) is to replace deficient or dysfunctional Von Willebrand Factor (VWF) protein. However, the choice of treatment has to be considered carefully in view of patient factors and the unique properties of replacement products. Tailoring a treatment plan to an individual patient ’s bleeding challenge is an intricate process. This review describes personalization of treatment selection for desmopressin (DDAVP), VWF replacement concentrates, including the newly available recombinant VWF (rVWF) and prophylaxis as a treatment approach in VWD. (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - August 7, 2019 Category: Hematology Authors: Chai W. Phua, Erik Berntorp Source Type: research

An Open Call Out On Continual Quality/Safety Improvement Strategies in Transfusion Science and Medicine
In the previous issue of Trasci [58.4], I submitted a report on some UK strategies for enhancing the healthcare of donors and recipients. Believing in the values of collaboration and cooperation throughout my academic life to achieve educational objectives, I have obtained the agreement of two leaders in the transfusion field to share some of their currently planned strategies, with Trasci readers. (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - August 7, 2019 Category: Hematology Authors: Jerard Seghatchian Source Type: research

Plasma-derived Industry and Plasma-derived Medicinal Products in the Italian National Blood Transfusion Service
The priority objectives of the Italian Transfusion System are self-sufficiency for blood components and for plasma-derived products. Currently the collection activity guarantees self-sufficiency of blood components, including plasma for clinical use, but not for plasma-derived drugs, ie albumin and immunoglobulins in particular. The National Plasma Program of 2016-2020, issued a Ministerial Decree on December, 2, 2016, aims to achieve this goal, both by increasing the collection of plasma and therefore its transfer to plasma derivation companies, and by increasing the appropriateness of the requests. (Source: Transfusion a...
Source: Transfusion and Apheresis Science - August 6, 2019 Category: Hematology Authors: Giustina De Silvestro, Piero Marson, Antonio Breda, Vincenzo De Angelis Source Type: research

Emergency management of patients with bleeding disorders: practical points for the emergency physician
Emergency department (ED) physicians are often the first point of contact for patients who present with bleeding symptoms. Work up and management of bleeding in the emergency room can be a daunting task as it requires: (1) accurate diagnosis of the bleeding cause, of which there is long list of common and rare etiologies (2); appropriate investigations and interpretation of the results; and (3) timely management of bleeding symptoms to prevent limb- or life-threatening complications. Crucial to the management of a bleeding patient is a thorough yet focused history exploring bleeding symptoms, medications/drugs (anticoagula...
Source: Transfusion and Apheresis Science - August 6, 2019 Category: Hematology Authors: Adrienne Lee Source Type: research

Comprehensive Care for Hemophilia and Other Inherited Bleeding Disorders
The World Federation of Hemophilia (WFH) states in its Guidelines for the Management of Hemophilia, Second Edition1, that people with hemophilia are best managed in a comprehensive care setting. That team is typically comprised of a core group including a hematologist, nurse coordinator, physiotherapist, social worker, specialized lab technologist and data manager, and as needed, by other specialists. Hemophilia is an X-linked congenital bleeding disorder caused by a deficiency of coagulation factor VIII (FVIII) in hemophilia A or factor IX (FIX) in hemophilia B. (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - August 6, 2019 Category: Hematology Authors: David Page Source Type: research

An Overview of Inherited Factor VII Deficiency
Factor VII (FVII) deficiency is the most common of the Rare Inherited Coagulation Disorders. The inheritance is autosomal recessive but there is variable penetrance. Overall there is poor correlation between the FVII level and the bleeding phenotype. Heterozygotes may have significant bleeding and severe homozygotes, or compound heterozygotes can be asymptomatic. Typically, homozygotes have FVII levels10-20%. (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - August 6, 2019 Category: Hematology Authors: Sue Robinson Source Type: research

Hemophilia A with inhibitor: Immune tolerance induction (ITI) in the mirror of time
Inhibitor (neutralizing antibodies) development remains the most significant complication in patients with severe congenital hemophilia A receiving exogenous factor VIII (FVIII). Although our understanding of the pathophysiology of inhibitor development has advanced, the knowledge gained has not yet translated into a robust decline in incidence, with the overall risk remaining at ˜30%. Immune Tolerance Induction (ITI) is the only current method to successfully eradicate an inhibitor and achieve long-term tolerance. (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - August 6, 2019 Category: Hematology Authors: Charles Nakar, Amy Shapiro Source Type: research

Gene therapy for hemophilia: Progress to date and challenges moving forward
Over the past decades hemophilia has been transformed from a debilitating disease to a manageable condition. However, the current treatment options are expensive, complex, and inaccessible to a large portion of the global population. Moreover, the development of antibodies to replacement factors, termed inhibitors, is a common complication that not only renders conventional prophylaxis regimens ineffective but also increase the annual bleeding rate in affected patients. Fortunately, much progress has been made toward developing a curative gene therapy treatment for hemophilia and these efforts have led to a series of human...
Source: Transfusion and Apheresis Science - August 6, 2019 Category: Hematology Authors: Kandace L. Gollomp, Bhavya S. Doshi, Valder R. Arruda Source Type: research

Transfusion medicine: A research agenda for the coming years
The next great advance(s) will likely come from the recognition that our current transfusion products and practices are much less effective and much more toxic to patients than previously realized. We believe that growing recognition of the toxicity and therapeutic limitations of blood transfusion will catalyze the next major advance(s) in transfusion medicine. An overwhelming body of data demonstrates that current transfusion practices are associated with major morbidity, including nosocomial infections, thrombosis, inflammation, organ failure and even mortality. (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - August 6, 2019 Category: Hematology Authors: Neil Blumberg, Jill M. Cholette, Christine Cahill, Anthony P Pietropaoli, Shira Winters, Richard Phipps, Suzie A. Noronha, Joanna M. Heal, Scott A. Kirkley, Thomas J. Fountaine, Kelly Henrichs, Debra Masel, Majed A. Refaai Source Type: research

Implementation of a closed platelet-rich-plasma preparation method using the local blood bank infrastructure at the Principality of Asturias (Spain): back to basic methodology and a demographics perspective after 1 year
Platelet-rich plasma (PRP) is used with increasing demand as autologous adjuvant therapy in many areas of regenerative medicine, thanks to the platelet rich content of growth factors and bio-active molecules. However, to date there is a lack of consensus on PRP preparation methods, on processing and application forms, on clinical application guidelines and on knowledge-based composition at the cellular and molecular level, making difficult the assessment of clinical results from different groups in different clinical areas. (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - August 6, 2019 Category: Hematology Authors: Ana Mar ía Ojea-Pérez, Andrea Acebes-Huerta, Tamara Arias-Fernández, Laura Gutiérrez, María Carmen Muñoz-Turrillas Source Type: research

Editorial Board EDB
(Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - August 1, 2019 Category: Hematology Source Type: research

The experience of allogeneic hematopoietic stem cell transplantation in a patient with X-linked adrenoleukodystrophy
In this study, we would like to present our experience of alloHSCT for X-ALD from a HLA matched related sibling by the use of reduced intensity conditioning regimen composed of fludarabine, busulfan and ATG which allows us to reduce procedure-related toxicity and prevent mortality while achieving a curative effect. (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - July 22, 2019 Category: Hematology Authors: Rafiye Ciftciler, Hakan Goker, Yahya Buyukas ık, Meral Topcu, Nesive Gevher, Haluk Demiroglu Source Type: research

Pre-operative Therapeutic Plasma Exchange and Intravenous Immune Globulin for the treatment of Heparin Induced Thrombocytopenia in a lung transplant recipient
Lung transplantation surgery often relies on the use of intraoperative extracorporeal membrane oxygenation (ECMO) and necessitates the need for high dose anticoagulation. Heparin induced thrombocytopenia complicates intraoperative anticoagulation management during lung transplant surgery requiring ECMO. Though other anticoagulants such as argatroban and bivalrudin are utilized for the treatment of Heparin Induced Thrombocytopenia (HIT), the lack of reversal agents makes it difficult to use these agents intraoperatively in cases with high bleeding risk. (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - July 22, 2019 Category: Hematology Authors: Molly W. Mandernach, Sravanthi Nandavaram, Bilal Salame, Tiago Machuca, Andres Pelaez Source Type: research

Ghosal hematodiaphyseal dysplasia with autoimmune anemia in two adult siblings
Ghosal hematodiaphyseal dysplasia (GHDD) is an autosomal recessive inherited disorder associated with biallelic mutations in the TBXAS1 gene located on the chromosome 7q33-34, which encodes thromboxane-A-synthase. GHDD is characterized by defective hematopoiesis due to bone marrow fibrosis and metadiaphyseal dysplasia of long bones. The accurate diagnosis of this rare syndrome is critical since it reduces the need of blood transfusions by corticosteroid therapy, leading to a significant improvement in anemia and bone changes. (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - July 22, 2019 Category: Hematology Authors: Rafiye Ciftciler, Yahya Buyukas ık Source Type: research

A case study: Therapeutic plasma exchange in voltage-gated potassium channel autoimmune encephalitis
Neurological syndromes associated with voltage-gated potassium channels (VGKC) affect the nerve and muscle physiology. Presence of antibodies to VGKC are associated with three main neurologic syndromes namely neuromyotonia (NMT), limbic encephalitis (LE) and Morvan's syndrome(MVS) LE is a variably treatable neurologic syndrome associated with high levels of antibodies to the voltage-gated potassium channel (VGKC) complex. These antibodies are directed against protein antigens that bind to the VGKC complex. (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - July 22, 2019 Category: Hematology Authors: Mohit Chowdhry, Shiva Prasad Gajulapalli, Soma Agrawal Source Type: research

Extracorporeal photopheresis for the treatment of graft rejection in 33 adult kidney transplant recipients
Background - Extracorporeal photopheresis (ECP) has shown encouraging results in the prevention of allograft rejection in heart transplantation. However, the role of ECP in kidney transplant (KT) rejection needs to be determined.Methods - This multicentre retrospective study included 33 KT recipients who were treated with ECP for allograft rejection (23 acute antibody-mediated rejections (AMRs), 2 chronic AMRs and 8 acute cellular rejections (ACRs)). The ECP indications were KT rejection in patients who were resistant to standard therapies (n  = 18) or in patients for whom standard therapies were contraindica...
Source: Transfusion and Apheresis Science - July 22, 2019 Category: Hematology Authors: Mathilde Tamain, Johnny Sayegh, Arnaud Lionet, Philippe Grimbert, Carole Philipponnet, Marc Hazzan, Jean-Fran çois Augusto, Mathias Büchler, Etienne Merlin, George Kosmadakis, Aurélien Tiple, Bruno Pereira, Cyril Garrouste, Anne-Elisabeth Heng Source Type: research

Acquired hemophilia A in chronic lymphocytic leukemia: A case report
We report herein is such a rare case of acquired hemophilia in a patient with CLL. (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - July 10, 2019 Category: Hematology Authors: Volkan Karaku ş, Egemen Kaya, Yelda Dere, Erdal Kurtoğlu Source Type: research

Cascade Plasmapheresis as a Desensitization Strategy for Patients undergoing ABO Incompatible Living Donor Liver Transplantation (ABOi LDLT): A Single Center Experience
(Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - July 10, 2019 Category: Hematology Authors: Meenu Bajpai, Brinda Kakkar, Shruti Gupta, Archana Rastogi, Viniyendra Pamecha Source Type: research

Phenotypical and functional analysis of donor lymphocyte infusion products after long-term cryopreservation
Donor lymphocyte infusions, collected from peripheral blood by apheresis, are regularly used to re-establishing disease control in patients with impending or full relapse after allogeneic cell transplantation. The cryopreservation and thawing processes of the cellular products, required for clinical needs, result in a decreased cellular recovery. The aim of this study was to perform an integral analysis of phenotypic and functional characteristics in different cell populations, within cryopreserved products used for therapeutic purposes. (Source: Transfusion and Apheresis Science)
Source: Transfusion and Apheresis Science - July 10, 2019 Category: Hematology Authors: Ann-Kathrin Sch äfer, Miguel Waterhouse, Marie Follo, Jesus Duque-Afonso, Justus Duyster, Hartmut Bertz, Jürgen Finke Source Type: research