[Molecular genetic analysis of a family trio with weak B phenotype].

OBJECTIVE: To explore serological and molecular characteristics of a family trio with weak B phenotype. METHODS: ABO blood type of the family was determined with a serological method. Genotypes of the ABO gene were determined with PCR-sequence specific primer (PCR-SSP). Sequences of exons 6 and 7 of the ABO gene were analyzed by PCR sequence-based testing. RESULTS: Serologically, the proband and her father were determined as B subtype (Bw), while her mother was of O group. The genotypes of the proband and her father were Bw12/O, while her mother was O01/O01. Sequencing of exons 6 and 7 of the ABO gene confirmed that the proband and her father were Bw12/O01. Compared with B101/O01, the Bw12 subtype carried a missense mutation (278C>T) in exon 6 of the ABO gene. CONCLUSION: The 278C>T mutation probably underlies the Bw phenotype and can be transmitted stably.

Use of Low-Dose Recombinant Factor â ¦a for Uncontrolled Perioperative Bleeding.

BACKGROUND: Recombinant activated factor VIIa (rFVIIa) is a prohemostatic agent initially approved for use in hemophilia patients and has also been used for a diverse range of off-label indications in the context of massive uncontrolled blood loss; however, no convincing evidence exists regarding the optimal dose of rFVIIa to treat uncontrolled bleeding in surgical patients. AIM: To evaluate the effects and safety of a very low dose of rFⅦa in patients with uncontrolled perioperative bleeding in the surgical intensive care unit (ICU). METHODS: 55 patients from Beijing Hospital, who received rFⅦa between July 2004 and November 2018 for uncontrolled perioperative bleeding were included. The controls were matched for age, sex, severity, and operation type. The baseline demographics, survival, changes in bleeding and transfusion, coagulation parameters and complications were analyzed. RESULTS: A low dose of rFⅦa (2.0∼3.6 mg, with a median dose of 39.02 µg/kg) appears to be effective in controlling massive hemorrhage (with an effective rate of 74.55%), and can reduce volume of red blood cell transfusion, improve coagulation status, while has a relatively low risk of thromboembolic complications (3.6%). CONCLUSION: In patients with uncontrolled perioperative bleeding, a low dose of rFⅦa could be used when traditional methods are ineffective.

Plasma Transfusion in Patients With Cirrhosis in China: A Retrospective Multicenter Cohort Study.

Patients with cirrhosis used to be associated with frequent use of blood components because of their complex disorder of hemostasis and bleeding complications. Recent findings have indicated that patients with cirrhosis have a state of "rebalanced" or even procoagulant hemostasis and have questioned the prophylactic use of plasma. To evaluate the current status of plasma use in patients with cirrhosis, we conducted a retrospective survey in 11 tertiary-care hospitals in China from September 1 to October 31, 2013. All patients admitted with cirrhosis during the study period were included in the study. The survey collected information including patients' diagnostic and demographic data, clinical course including bleeding complications and invasive procedures, laboratory results, and plasma transfusion data. Among 1595 patients with cirrhosis admitted to the 11 hospitals, 236 (14.8%) patients received 1 or more plasma transfusions during the study period. The number of plasma transfusions is defined as the number of transfusion orders. A total of 1037 plasma transfusions were administered to these patients, with a mean of 4.4 transfusions per transfused patient, ranging from 1 to 22 transfusions per transfused patient. Most plasma transfusions (760/1037; 73.3%) were given to patients without bleeding, for treatment of coagulopathy either without planned invasive procedures (70.4%) or before invasive procedures (2.9%). The median dose of plasma transfusion was 3.8 mL/kg. The rate of plasma transfusion of participating hospitals varied from 5.3% to 31.8%. It is encouraging to see that in one teaching hospital, 85.7% plasma transfusions were given to patients with bleeding indication, showing a promising sign in appropriate transfusion. Prophylaxis or empirical plasma transfusion is still a common problem in managing patients with liver cirrhosis. Wide variations are found in plasma transfusion practice among hospitals. Effective measures to control and reduce empirical correction of abnormal coagulation tests through transfusing plasma should be strengthened urgently.

A multicenter study of blood component transfusion in patients with liver cirrhosis in China: Patient characteristics, transfusion practice, and outcomes.

BACKGROUND: Cirrhosis is a complex acquired disorder of hemostasis and patients frequently receive blood transfusions. But there is very limited data on patterns of blood use at a patient level. AIMS: To characterize blood use in cirrhotic patients in China and compare with recommendations to help identify areas where quality improvement strategies can be targeted. We also compared findings to a similar study undertaken in UK. METHODS: A cross-sectional study was conducted in 11 hospitals over a 2-month period. Data were collected prospectively on each hospitalized cirrhotic patient to day 28. RESULTS: 1595 cirrhotic patients were included and 20.6% were transfused. 48.2% of transfused patients received transfusion for bleeding, most commonly gastrointestinal bleeding (65.8%). The remaining 51.8% were transfused for non-bleeding indications. 32.5% of patients transfused for gastrointestinal bleeding with red blood cells had a pre-transfusion haemoglobin >7g/dL. 89.1% of patients transfused frozen plasma for non-bleeding indications received them in the absence of a planned procedure. The patterns of blood transfusion in cirrhosis were different between China and UK. Of note, empirical prophylactic use of frozen plasma was more common in the Chinese study (89%) than in the UK (24%). CONCLUSION: Education and research should be implemented to improve patient blood management, especially in prophylactic frozen plasma use area.