关键词: RBC antibody RBC transfusion anti‐Hr0 anti‐Rh17 hemoglobin‐based oxygen carriers  transfusion practices (adult)

Mesh : Humans Female Middle Aged Hemoglobins Isoantibodies / immunology Rh-Hr Blood-Group System / immunology Precursor Cell Lymphoblastic Leukemia-Lymphoma / therapy Blood Substitutes / therapeutic use Erythrocyte Transfusion

来  源:   DOI:10.1111/trf.17855

Abstract:
BACKGROUND: A 54-year-old Hispanic OPos female with known history of anti-Rh17 antibodies was diagnosed with Philadelphia-Chromosome positive (Ph+) acute lymphoblastic leukemia (ALL). Rh17, also known as Hr0, is a high-frequency antigen composed of several epitopes on the RhCE protein. Anti-Rh17 antibodies can be made by individuals with missing or varied C/c, E/e antigens. Anti-Rh17 antibodies are clinically significant given multiple case reports of hemolytic disease of the fetus and newborn (HDFN). Finding compatible units for patients with anti-Rh17 can be particularly difficult given that only 1 in 100,000 people are Rh17 negative.
METHODS: Search for compatible units was conducted by the American Rare Donor Program (ARDP) with no leads. After chemotherapy induction and despite erythropoiesis stimulating agent administration, the patient\'s hemoglobin continued to trend down to a nadir of 2.8 g/dL. Here we report transfusion of incompatible pRBC to this patient with critically symptomatic anemia. HBOC-201 (Hemopure) was obtained and administered under an emergency compassionate/expanded access designation from the Food and Drug Administration (FDA) under an emergency Investigational New Drug (IND) application.
CONCLUSIONS: Overall difficulties in this case included the challenge of finding compatible units, dilemma of transfusing incompatible units in a patient with severe anemia and obtaining alternatives to blood products. This case report demonstrates the successful use of HBOC-21 in treating life-threatening anemia.
摘要:
背景:一名54岁的西班牙裔OPOS女性,具有已知的抗Rh17抗体病史,被诊断为费城染色体阳性(Ph+)急性淋巴细胞白血病(ALL)。Rh17,也称为Hr0,是由RhCE蛋白上的几个表位组成的高频抗原。抗Rh17抗体可以由缺失或不同C/c的个体产生,E/e抗原。鉴于胎儿和新生儿溶血病(HDFN)的多个病例报告,抗Rh17抗体具有临床意义。考虑到100,000人中只有1人是Rh17阴性的,为抗Rh17患者寻找兼容的单位可能特别困难。
方法:由美国稀有捐赠者计划(ARDP)进行无导线的兼容单位搜索。化疗诱导后,尽管施用红细胞生成刺激剂,患者的血红蛋白继续下降至最低点2.8g/dL。在这里,我们报告了向患有严重症状性贫血的患者输注不相容的pRBC。HBOC-201(Hemopure)是在紧急研究新药(IND)申请下,根据食品和药物管理局(FDA)的紧急同情/扩展访问指定获得和管理的。
结论:这种情况下的总体困难包括寻找兼容单位的挑战,在患有严重贫血的患者中输注不相容单位并获得血液制品替代品的困境。该病例报告证明了HBOC-21在治疗危及生命的贫血中的成功使用。
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