Acquired von Willebrand syndrome is common in infants with systemic-to-pulmonary shunts: Retrospective case-series.

Background: Although acquired von Willebrand syndrome (aVWS) has been described in congenital heart disease before, anatomical features leading to aVWS with characteristic reduction or loss of high molecular weight von Willebrand multimers (HMWM) are not well known. This study assesses the prevalence and effects of aVWS in infants with systemic-to-pulmonary shunts (SPS).

Methods: This retrospective single-center study analyzes diagnostic data of infants with complex congenital heart defects requiring palliation with SPS.

Perioperative diagnosis and impact of acquired von Willebrand syndrome in infants with congenital heart disease.

Acquired von Willebrand syndrome (aVWS) has been reported in patients with congenital heart diseases associated with shear stress caused by significant blood flow gradients. Its etiology and impact on intraoperative bleeding during pediatric cardiac surgery have not been systematically studied. This single-center, prospective, observational study investigated appropriate diagnostic tools of aVWS compared with multimer analysis as diagnostic criterion standard and aimed to clarify the role of aVWS in intraoperative hemorrhage.

Multi-Modal Characterization of the Coagulopathy Associated With Extracorporeal Membrane Oxygenation.

Objectives: Extracorporeal membrane oxygenation is used to stabilize severe cardiocirculatory and/or respiratory failure. However, extracorporeal membrane oxygenation is associated with a coagulopathy characterized by thromboembolic and hemorrhagic complications. This study aimed to characterize the pathomechanism of the extracorporeal membrane oxygenation-associated coagulopathy and identify options to optimize its monitoring and therapy.

Disease progression and defects in primary hemostasis as major cause of bleeding in multiple myeloma.

Background And Objectives: In patients with multiple myeloma (MM), unexpected bleeding complications remain a major issue. Since routine coagulation parameters are often inconspicuous, diagnosis and treatment of the underlying coagulation disorders are challenging.

Patients And Methods: In our single-center observational study, we analyzed 164 patients with MM for coagulation disorders and bleeding complications.

Low plasma protein Z levels are associated with an increased risk for perioperative bleedings.

Background And Objectives: Protein Z (PZ) deficiency has been implicated both in bleeding diatheses and in thrombophilia. Considering its ambiguous nature and the conflicting clinical data so far, we set out to evaluate the impact of low PZ on perioperative bleeding in patients who underwent surgical (ENT) interventions involving a high risk of bleeding.

Patients And Methods: After exclusion of other coagulation disorders, 154 Patients were stratified into quartiles according to PZ plasma concentrations to evaluate the relation between PZ and bleeding complications.

[Vaginal bleeding as symptom of a medical emergency--pitfalls of therapy with new oral anticoagulants - case 5/2014].

History And Admission Findings: A 90-year-old female patient treated with dabigatran for atrial fibrillation presented emergently with a spontaneous vaginal bleeding due to endometrium carcinoma.

Investigations: Laboratory analysis revealed azotemia consistent with acute-on-chronic renal failure. Coagulation was deranged (prolongation of activated partial thromboplastin time (aPTT) by 3.

Management of disseminated intravascular coagulopathy with direct factor Xa inhibitor rivaroxaban in Klippel-Trénaunay syndrome.

Klippel-Trénaunay syndrome (KTS) is a rare congenital anomaly characterized by malformation of lymph and blood vessels as well as growth disturbance of soft tissue and bone. The clinical picture is variable and associated with an increased risk of thromboembolic events mediated by intravascular coagulopathy in venous malformations. Here, we report on a male patient with KTS suffering from recurrent deep vein thrombosis (DVT) and life-threatening bleeding due to consumptive coagulopathy.

[Unusual cause of macrohematuria in a 29-year-old woman].

History And Admission Findings: We report on the case of a young women presenting with macrohaematuria, petechiae and strong headaches.

Investigations: Laboratory showed a thrombotic microangiopathy with helmet cells, increased LDH levels (>600 U/l), and thrombocytopenia (<40,000/μl).

Diagnosis, Treatment And Course: Due to strong haemolytic activity and headache with blurred vision, immediate plasma separation with fresh frozen plasma was commenced.

[Severe hemorrhage in a patient with metastatic colorectal cancer - case 8/2012].

History And Admission Findings: We report on a 65-year-old female patient with a recent diagnosis of adenocarcinoma of the sigmoid colon and massive hematochezia in the context of a general bleeding disorder.

Investigations: Disseminated malignant disease with hepatic metastases as well as bone marrow involvement was demonstrated. Moreover, circulating tumor cells were demonstrated by flow cytometry.

Acquired coagulopathy caused by intoxication with the superwarfarin-type anticoagulant rodenticide flocoumafen.

A 28 year-old heretofore healthy woman was transferred to our hospital with a two-month history of recurring episodes of bleeding. Administration of vitamin K and prothrombin complex concentrates in the transferring hospital had only temporarily corrected both the markedly elevated international normalized ratio (INR) and the prolonged activated partial thromboplastin time (aPTT). The patient's medical and family history revealed no reason for these abnormalities.

Human plasma thrombopoietin levels are regulated by binding to platelet thrombopoietin receptors in vivo.

Background: Data from several studies support the hypothesis that thrombopoietin (TPO) plasma levels are regulated via circulating platelet (PLT) numbers by binding to PLT TPO receptors (TPO-Rs). In this study, PLT numbers and TPO plasma levels were measured following the transfusion of unmanipulated, sham-saturated, and TPO-R-saturated PLT preparations to provide additional in vivo evidence for this regulatory mechanism.

Study Design And Methods: Following in vitro experiments to characterize pegylated recombinant human megakaryocyte growth and development factor (PEG-rHuMGDF) binding characteristics, PLT numbers and TPO plasma levels were measured following the transfusion of unmanipulated, sham-saturated, and TPO-R-saturated PLT preparations in thrombocytopenic patients.