Acquired factor XIII deficiency: A review.

Acquired factor XIII (FXIII) deficiency is a rare bleeding disorder that can manifest with spontaneous or delayed life-threatening hemorrhage. Causes of acquired deficiency include immune-mediated inhibition, as well as non-immune FXIII hyperconsumption or hyposynthesis. The occurrence of acquired FXIII deficiency can be idiopathic or may be associated with comorbidities, such as malignancies or autoimmune disorders. Recognition of acquired FXIII deficiency and its underlying cause is imperative, as treatment options vary depending on the etiology. Diagnosis requires quantitative FXIII testing in addition to supplemental inhibitor studies if the clinical situation suggests an immune-mediated pathophysiology. Treatment may involve FXIII replacement, antifibrinolytic administration, and/or inhibitor eradication. However, treatment targets and thresholds are undefined in acquired FXIII deficiency. This review will focus on the clinical characteristics, diagnostic issues and therapeutic options for both immune and non-immune acquired FXIII deficiency. Cases are described to illustrate the clinical features of acquired FXIII deficiency.

Transfusion Safety: The Nature and Outcomes of Errors in Patient Registration.

The transfusion chain is susceptible to error at every step. Accurate patient registration is a key first step that links a patient with their historical medical profile, yet patient registration is marked by its own challenges. Registration errors are deviations from standard operating procedures that occur during the process of patient registration. A frequent consequence of registration errors is the obfuscation of historical information and patient misidentification. Through duplicate registrations, patient information can be spread across multiple records and through hybrid registrations information from multiple patients can be combined into a single record. Patients with the same core identifiers, and the misuse of health insurance information also pose a threat to accurate registration. In the context of transfusion, this can lead to ABO discrepancies, failing to match for previously identified alloantibodies, and redundant serological investigations. Other consequences include missed billing opportunities and the inadvertent sharing of medical information. Reducing the occurrence of registration errors can be achieved through a multifaceted approach combining targeted educational efforts with technological improvements to the registration system. A recent development being the use of biometric identifiers. Despite their frequency, published reports on the occurrence and underlying cause of registration errors are rare. Most reports are found within articles on general medical errors or misidentification events and consequently, the true rate of registration errors among health information systems is not known. Here we summarize literature pertaining to how and why registration errors occur and their implications in the context of blood transfusion.