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Novel mutations in KLF1 encoding the In(Lu) phenotype reflect a diversity of clinical presentations.
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Novel mutations in KLF1 encoding the In(Lu) phenotype reflect a diversity of clinical presentations.

Transfusion. 2017 Oct 19;:

Authors: Keller J, Vege S, Horn T, Keller MA, Leger RM, Aeschlimann J, Lomas-Francis C, Westhoff CM

Abstract
BACKGROUND: Mutation in the KLF1 gene is the cause of the In(Lu) (Inhibitor of Lutheran) Lu(a-b-) phenotype and more than 60 alleles have been associated with this phenotype. Here we describe findings from investigation of seven cases: six presenting with a Lu(a-b-) phenotype including the historical index case and one referred from a patient with chronic anemia.
STUDY DESIGN AND METHODS: Serologic testing was by standard methods. DNA testing included amplification and sequencing of KLF1 and LU coding regions. A StuI polymerase chain reaction-restriction fragment length polymorphism was designed to target c.304T>C in KLF1.
RESULTS: Five different KLF1 alleles were identified. Three are new: KLF1*90A (p.Trp30Ter), KLF*911A (p.Thr304Lys), and KLF1*304C,318G (p. Ser102Pro, Tyr106Ter) present in two unrelated individuals. Two, including the index case, had c.954dupG (p.Arg319Glufs*34), that is, KLF1*BGM06. The child with unexplained anemia had c.973G>A (p.Glu325Lys), associated with congenital dyserythropoietic anemia. The common c.304T>C was found in two of the seven samples investigated and in 60 of 100 blood donors.
CONCLUSION: Mutations in KLF1 are pleiotropic and although most are benign, others are associated with hematologic abnormalities. We report three new KLF1 alleles associated with benign In(Lu) and document both the molecular basis of the original In(Lu) phenotype using a frozen sample stored for more than 50 years and the cause of unexplained anemia in a child. We also confirm previous observations that c.304C (p.102Pro) is not, by itself, associated with an In(Lu) phenotype in donors self-identified as U.S. minorities.

PMID: 29047116 [PubMed - as supplied by publisher]




An international investigation into AB plasma administration in hospitals: how many AB plasma units were infused? The HABSWIN study.
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An international investigation into AB plasma administration in hospitals: how many AB plasma units were infused? The HABSWIN study.

Transfusion. 2017 Oct 14;:

Authors: Zeller MP, Barty R, Dunbar NM, Elahie A, Flanagan P, Garritsen H, Kutner JM, Pagano MB, Pogłód R, Rogers TS, Staves J, van Wordragen-Vlaswinkel M, Zwaginga JJ, Murphy MF, Heddle NM, Yazer MH, Biomedical Excellence for Safer Transfusion (BEST) Collaborative

Abstract
BACKGROUND: Typical practice is to transfuse group-specific plasma units; however, there are situations where group AB plasma (universal donor) is issued to group A, B, or O recipients. If demand for group AB plasma exceeds collections, there is potential for shortage. This project explored the patterns of group AB plasma utilization at hospitals around the world.
STUDY DESIGN AND METHODS: The study had two phases: a survey that inquired about hospital group AB plasma inventory, policies, and transfusion practices and a retrospective review of 2014 calendar year data where participants submitted information on plasma disposition including ABO group of unit and recipient, transfusion location, and select indications. Recruitment occurred through snowball sampling. Descriptive analyses were performed.
RESULTS: Survey data were received from 25 centers across 10 countries; of those, 15 participants contributed to the data collection component. These 15 centers transfused a total of 43,369 AB plasma units during the study period. Only 1496 of 5541 (27%) group AB plasma units were transfused to group AB recipients. Transfusion policies, practices, and patterns were variable across sites.
CONCLUSION: Group AB plasma units are frequently transfused to non-AB recipients. Whether transfusing 73% of group AB plasma units to non-AB recipients is the ideal inventory management strategy remains to be determined.

PMID: 29030954 [PubMed - as supplied by publisher]




Fetal/neonatal alloimmune thrombocytopenia due to anti-CD36 antibodies: antibody evaluations by CD36-transfected cell lines.
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Fetal/neonatal alloimmune thrombocytopenia due to anti-CD36 antibodies: antibody evaluations by CD36-transfected cell lines.

Transfusion. 2017 Oct 13;:

Authors: Lin M, Xu X, Lee HL, Liang DC, Santoso S

Abstract
BACKGROUND: Isoantibodies against CD36 (platelet glycoprotein 4), developed in Type I CD36-deficient mothers are frequently reported as the cause of fetal/neonatal alloimmune thrombocytopenia in the Asian population. Therefore, further detailed characterization of anti-CD36-mediated fetal/neonatal alloimmune thrombocytopenia is warranted. Here, we report the characterization of a patient with fetal/neonatal alloimmune thrombocytopenia in a Taiwanese family caused by anti-CD36 isoantibodies using a novel antigen-capture method.
STUDY DESIGN AND METHODS: Platelets and monocytes were analyzed for CD36 expression by flow cytometry. Sequencing analysis of the CD36 gene was performed to identify the mutation underlying the CD36 deficiency. Stable transfected human embryonic kidney HEK293 cells expressing recombinant CD36 were established. These cells were used for the characterization of anti-CD36 isoantibodies by flow cytometry, immunoprecipitation, and antigen-capture assay.
RESULTS: Flow cytometry analysis revealed a total absence of CD36 on both platelets and monocytes of the mother (Type I CD36-deficient) caused by heterozygous deletions of the CD36 gene (332_333delCA and c.1254 + 6_1254 + 11delTATTTG). Analysis of maternal serum with CD36-transfected HEK293 cells by flow cytometry, immunoprecipitation, and antigen-capture assay demonstrated the presence of anti-CD36 isoantibodies in maternal serum. Interestingly, this antibody could not be detected by the monoclonal antibody immobilization of platelet antigens assay when anti-CD36 monoclonal antibody (clone FA6-152) was used as the capture antibody.
CONCLUSION: This case reemphasizes the role of anti-CD36 isoantibodies on the pathomechanism of fetal/neonatal alloimmune thrombocytopenia. The fact that the monoclonal antibody immobilization of platelet antigens assay does not seem to be reliable for the identification of all anti-CD36 antibodies indicates that screening of anti-CD36 isoantibodies by a monoclonal antibody-independent method, as presented here, should be considered.

PMID: 29030871 [PubMed - as supplied by publisher]




Funding blood safety in the 21st century.
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Funding blood safety in the 21st century.

Transfusion. 2017 Oct 13;:

Authors: Ifland L, Bloch EM, Pitman JP

Abstract
BACKGROUND: Since 2000, there has been an historic increase in international development assistance, including blood safety projects. The result has been increased blood donations and infectious disease screening in many beneficiary countries. A comprehensive examination of international development assistance for blood safety has yet to be completed.
STUDY DESIGN AND METHODS: This report examines publicly available information, including donor agency websites and databases and data from the 2008 and 2012 World Health Organization Global Database on Blood Safety.
RESULTS: Between 2000 and 2015, from $602.4 million to $2.1 billion in international development assistance was allocated to blood safety programs worldwide, mostly as part of the global response to the human immunodeficiency virus/acquired immunodeficiency syndrome epidemic. The US President's Emergency Plan for AIDS Relief and the Global Fund to Fight AIDS, Tuberculosis, and Malaria were responsible for the majority of blood safety funding, which peaked in 2010 and declined through 2015.
CONCLUSION: Between 2000 and 2015, countries with high burdens of human immunodeficiency virus/acquired immunodeficiency syndrome received funding and technical assistance to improve national laboratories, increase blood component production, and strengthen clinical practice. Global trends in international development assistance at large, including aid for blood safety, suggest that funding will not rebound.

PMID: 29030857 [PubMed - as supplied by publisher]