Implementation of a clinical decision support system improves compliance with restrictive transfusion policies in hematology patients.

BACKGROUND: There is increasing evidence for restrictive red blood cell (RBC) transfusion but compliance with recommended transfusion triggers is variable. A clinical decision support system (CDSS) has been found to reduce unnecessary transfusion in some clinical settings when physicians are advised they are noncompliant with the current guidelines. The objective was to assess the impact of a CDSS for blood product ordering in patients with hematologic disease. STUDY DESIGN AND METHODS: All platelet (PLT) and RBC transfusions were identified in hematology patients in three periods: before (baseline), immediately after (CDSS1), and 7 months after implementation of CDSS for blood ordering (CDSS2). Compliance with the recommended transfusion triggers was monitored for all orders made by CDSS or non-CDSS methods during each period. RESULTS: Ninety-seven patients with a variety of hematologic diagnoses received 502 RBC and 572 PLT transfusions during the three periods with no significant difference in 1) the mean number of transfusions per patient, 2) the proportion of patients transfused, 3) posttransfusion hemoglobin (Hb), and 4) pre- and posttransfusion PLT count, although mean pretransfusion Hb decreased. The proportion of noncompliant RBC and PLT transfusion requests improved from baseline to CDSS2 (69.0% to 43.4% p ≤ 0.005 for RBCs; and 41.9% to 31.2%, p = 0.16 for PLT) when all orders were compared, although this improvement was not significant at the 5% level for PLTs. CONCLUSIONS: The introduction of CDSS for blood product ordering supported by education and physician feedback in the hematology setting had an immediate impact on improving compliance with guidelines for restrictive transfusion practice.

The Bloom's syndrome helicase: keeping cancer at bay.

Bloom's syndrome, a very rare inherited disorder, predisposes its sufferers to the full range of cancers that afflict humanity. This predisposition is rooted in just one defective gene on chromosome 15. It encodes the BLM helicase - an enzyme that ordinarily protects against DNA damage arising during replication.

Premature aging in RecQ helicase-deficient human syndromes.

The RecQ family of DNA helicases have potential roles in DNA repair, replication and/or recombination pathways. In humans, a defect in the RecQ family helicases encoded by the BLM, WRN and RECQ4 genes gives rise to Bloom's (BS), Werner's (WS) and Rothmund-Thomson (RTS) syndromes, respectively. These disorders are associated with cancer predisposition and/or premature aging. In Bloom's syndrome, affected individuals are predisposed to many types of cancer at an early age. Werner's syndrome is a premature aging disorder with a complex phenotype, which includes many age-related disorders that develop from puberty, including greying and thinning of the hair, bilateral cataract formation, type II diabetes mellitus, osteoporosis and atherosclerosis. The phenotype of Rothmund-Thomson syndrome patients also consists of some features associated with premature aging, as well as predispositon to certain cancers. Here, we discuss the molecular basis of these RecQ helicase-deficient disorders.