The best strategy of blood transfusion in patients with Rhnull syndrome
  
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DOI:10.46701/APJBG.20170217026
KeyWord:Rhnull syndrome, Rh associated glycoprotein, transfusion strategy
                             
AuthorInstitution
Shuhong Yu Blood Transfusion Department, Affiliated Yantai Yuhuangding Hospital of Qingdao University, Yantai, Shangdong 264000, China.
Jie Xiao Blood Transfusion Department, Chengdu Military General Hospital, Chengdu, Sichuan 610000, China.
Hong Xu Blood Transfusion Department, Chengdu Military General Hospital, Chengdu, Sichuan 610000, China.
Yushiang Lin Department of Clinical Medicine, School of Basic Medical Sciences, Peking University Health Science Center, Beijing, 100191, China.
Jhysheng Chang Taiwan Society of Blood Transfusion, Taiwan,China.
Yanbin Li Blood Transfusion Department, Affiliated Yantai Yuhuangding Hospital of Qingdao University, Yantai, Shangdong 264000, China.
Dezhen Gao Blood Transfusion Department, Affiliated Yantai Yuhuangding Hospital of Qingdao University, Yantai, Shangdong 264000, China.
Shuai Pang Blood Transfusion Department, Affiliated Yantai Yuhuangding Hospital of Qingdao University, Yantai, Shangdong 264000, China.
Zhiyuan Xu Blood Group Lab, Beijing Red Cross Blood Center, Beijing 100088, China.
Tianhong Miao Blood Group Lab, Beijing Red Cross Blood Center, Beijing 100088, China.
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Abstract:
      Rhnull syndrome is a very rare disease. Patients with this syndrome present with negative serological Rh typing of E, e, C, c, and D antigens. Only one study has previously discussed Rhnull syndrome in Chinese individuals. We experienced two patients with Rhnull syndrome in China, Rh genotypes being CcDEe in the first patient and CCDee in the second patient. The first patient was a pregnant woman (gravida 2, para 1) with a negative red blood cell (RBC) antibody screen test. The second patient was a middle-aged man, transfused with ccdee, ccdEe, and ccdee RBC products, the pre-transfusion specimen was negative and post-transfusion specimen was anti-c,e, respectively. The hemoglobin level continued to increase in the second patient after being transfused with ccdEe RBC products. In the first patient, the result of the antibody screen test was still negative after artificial abortion. In patients with Rhnull syndrome, RBC products that have the same Rh genotype as the patient can be safely transfused.
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