Post-operative of bleeding, haemolysis and coagulation in mechanical circulatory support patients.

There are unique complications arising from mechanical support devices but some of the long-term systemic haematological complications are indistinguishable from management problems affecting the care of other patients receiving intermediate to long term care in the cardiac ICU. The field of mechanical cardiac assist device (MCAD) is evolving. Despite major changes in design of these devices the most feared haematological complications have remained unchanged, namely haemolysis, pump thrombosis or thromboembolism.

Can CSF spectrophotometry for "Xanthochromia" be used to detect leaking subarachnoid aneurysms in patients with sickle cell anemia with negative MRI or CT angiogram despite hyperbilirubinemia?

CSF (Cerebrospinal Fluid) xanthochromia by spectroscopy should not be dismissed in the context of hyperbilirubinemia in a patient with sickle cell anemia. Xanthochromia detected by spectrophotometry offers a vital clue that further invasive diagnosis is required.

Diagnostic high-throughput sequencing of 2396 patients with bleeding, thrombotic, and platelet disorders.

A targeted high-throughput sequencing (HTS) panel test for clinical diagnostics requires careful consideration of the inclusion of appropriate diagnostic-grade genes, the ability to detect multiple types of genomic variation with high levels of analytic sensitivity and reproducibility, and variant interpretation by a multidisciplinary team (MDT) in the context of the clinical phenotype. We have sequenced 2396 index patients using the ThromboGenomics HTS panel test of diagnostic-grade genes known to harbor variants associated with rare bleeding, thrombotic, or platelet disorders (BTPDs). The molecular diagnostic rate was determined by the clinical phenotype, with an overall rate of 49.

Hb Penang [β78(EF2)Leu→Pro, HBB: c.236T>C]: a Novel β-Globin Variant.

We report a novel hemoglobin (Hb) variant with a β chain amino acid substitution at codon 78 (CTG>CCG) (HBB: c.236T>C), detected through prenatal screening via capillary electrophoresis (CE) in an otherwise healthy and asymptomatic 38-year-old female of Southeast Asian ancestry. The variant, named Hb Penang after the proband's Malaysian city of origin, underwent further characterization through high performance liquid chromatography (HPLC), reversed phase HPLC, Sanger sequencing, isopropanol stability testing and isoelectric focusing (IEF).

Acquired hypofibrinogenemia: current perspectives.

Acquired hypofibrinogenemia is most frequently caused by hemodilution and consumption of clotting factors. The aggressive replacement of fibrinogen has become one of the core principles of modern management of massive hemorrhage. The best method for determining the patient's fibrinogen level remains controversial, and particularly in acquired dysfibrinogenemia, could have major therapeutic implications depending on which quantification method is chosen.

Hb Kalavasos [HBA2: c.275T > A; p.Leu92His]: a Novel α Chain Hemoglobin Variant.

We present a case of a novel pathogenic variant, Hb Kalavasos [α91(FG3)Leu→His (α2); HBA2: c.275T > A; p.Leu92His (NM_000517.

Agreement between ACT and aPTT during extracorporeal membrane oxygenation shows intra- and inter-individual variation.

Aim: We explored the relationship between activated clotting time (ACT) and activated partial thromboplastin time (aPTT) when used to monitor anticoagulation in patients undergoing extracorporeal membrane oxygenation (ECMO) support.

Methods: Data obtained in patients undergoing ECMO support between October 2012 and August 2013 in a single centre were reviewed. Clinical data were extracted from our Clinical Information System and ECMO database.

The place of viscoelastic testing in clinical practice.

Hellmut Hartert was the first person to exploit the viscoelastic properties of clotting blood to measure blood coagulation in 1948. Since then, the technology has improved, allowing these analyses to be performed as point-of-care tests with immediately-available results. The addition of several activators and inhibitors to the original assay creates a panel of tests able to quantify the different aspects of blood clotting that can rival conventional laboratory assays.

Clinical use of low haemoglobin density, transferrin saturation, bone marrow morphology, Perl's stain and other plasma markers in the identification of treatable anaemia presenting for cardiac surgery in a prospective cohort study.

Objectives: The differential diagnosis between iron deficiency anaemia (IDA) and anaemia of chronic disease (ACD) with or without associated iron deficiency can be challenging. We assessed the use of different parameters, both classical like ferritin, transferrin saturation and stainable bone marrow iron stores, and novel markers such as low haemoglobin density (LHD) and hepcidin to help discriminate between the three entities. This would allow the detection of patients with ACD with associated iron deficiency, which could benefit from iron supplementation that would have otherwise remained undetected.

An exploratory cohort study comparing prothrombin complex concentrate and fresh frozen plasma for the treatment of coagulopathy after complex cardiac surgery.

Background: Administration of coagulation factor concentrates to treat bleeding after cardiac surgery with cardiopulmonary bypass might be a strategy for reducing allogeneic blood transfusions, particularly for patients treated with warfarin preoperatively. We performed an exploratory analysis on whether the use of prothrombin complex concentrate (PCC) is safe and effective compared with fresh frozen plasma (FFP) to treat coagulopathy after pulmonary endarterectomy surgery with deep hypothermic circulatory arrest.

Methods: Consecutive adult patients who underwent pulmonary endarterectomy surgery between January 2010 and September 2012 and received PCC or FFP to treat coagulopathy were studied.

Primary percutaneous coronary intervention in a patient with haemophilia a.

Haemophilia A is a rare genetic condition leading to coagulation factor VIII deficiency and thus predisposing to bleeding diathesis. Due to advances in treatment, life expectancy of haemophilia A sufferers is increasing, and the incidence and prevalence of coronary artery disease are rising. There have been many reported cases of acute myocardial infarction in such patients, who subsequently undergo elective percutaneous coronary intervention.

Point-of-care assessment of hypothermia and protamine-induced platelet dysfunction with multiple electrode aggregometry (Multiplate®) in patients undergoing cardiopulmonary bypass.

Background: Coagulopathy is common after cardiopulmonary bypass (CPB), and platelet dysfunction is frequently considered to be a major contributor to excessive bleeding. Exposure to hypothermia may exacerbate the platelet function defect. We assessed platelet function during and after deep hypothermia with multiple electrode aggregometry (Multiplate(®); Verum Diagnostica GmbH, Munich, Germany).

The coagulopathy of cardiopulmonary bypass.

There have been numerous publications on the coagulopathy of cardiopulmonary bypass (CPB). This review provides an introduction to the history and main components of current CPB circuits and summarizes the current knowledge of pathogenesis, prevention, and treatment of the CPB coagulopathy. It encompasses an overview of intra- and postoperative monitoring of coagulation with special emphasis on the near-patient testing, its main complications, and the transfusion support, while taking into account the major changes in the technology used and supportive care provided since its inception.