Peyrard T, Pham B-N, Le Pennec P-Y, Rouger P
Inserm, U665, centre national de référence pour les groupes sanguins, institut national de la transfusion sanguine, 20, rue Bouvier, BP 79, 75522 Paris cedex 11, France.
Transfus Clin Biol. 2008 Jun;15(3):109-19. doi: 10.1016/j.tracli.2008.02.001. Epub 2008 Apr 10.
A rare blood group is usually defined as the absence of a high prevalence antigen or the absence of several antigens within a single blood group system, if its prevalence in France is 4/1000 or less in the general population. An individual with a rare blood phenotype can develop a naturally-occurring or immune antibody corresponding to his rare specificity. In case an extremely low stock of compatible blood is available at the national level, a so-called "transfusion deadlock" is described. Most of the individuals with a rare blood group are coincidently identified when a routine pretransfusion testing or pregnancy follow-up is performed, if the antibody(ies) corresponding to the rare specificity is(are) present. Other individuals are discovered following a systematic red cell typing, or family investigations in siblings. One hundred and twenty-one rare blood specificities and 42 rare blood genotypes are currently defined at the French National Reference Laboratory for Blood Groups (CNRGS-Paris). The French national registry of individuals with a rare blood phenotype/genotype includes about 9600 people, who are urged to regularly donate blood for the National Rare Blood Bank. This bank, based on a homologous blood transfusion program, is in charge of the long-term storage of rare frozen blood units, that can only be delivered after receiving authorization from the CNRGS. The global and individual care management of the individuals with a rare blood group, concerning potentially several hundred thousand people in France, requires a close cooperation between all the protagonists within the transfusion chain.
稀有血型通常定义为缺乏高流行率抗原，或在单一血型系统中缺乏几种抗原，前提是其在法国普通人群中的流行率为千分之四或更低。具有稀有血型表型的个体可产生与其稀有特异性相对应的天然或免疫抗体。如果全国范围内可获得的相容血液库存极低，就会出现所谓的“输血僵局”。大多数稀有血型个体是在进行常规输血前检测或孕期随访时，若存在与稀有特异性相对应的抗体而被偶然发现的。其他个体则是在进行系统的红细胞分型或对兄弟姐妹进行家族调查后被发现的。法国国家血型参考实验室（巴黎CNRGS）目前定义了121种稀有血型特异性和42种稀有血型基因型。法国稀有血型表型/基因型个体国家登记册约有9600人，他们被敦促定期为国家稀有血库献血。该血库基于同源输血计划，负责长期储存稀有冷冻血液单位，只有在获得CNRGS授权后才能发放。对法国可能涉及数十万的稀有血型个体进行全面和个性化的护理管理，需要输血链中所有相关方密切合作。
