Correlation to FVIII:C in Two Thrombin Generation Tests: TGA-CAT and INNOVANCE ETP.

Introduction: Several thrombin-generation tests are available, but few have been directly compared. Our primary aim was to investigate the correlation of two thrombin generation tests, thrombin generation assay-calibrated automated thrombogram (TGA-CAT) and INNOVANCE ETP, to factor VIII levels (FVIII:C) in a group of patients with hemophilia A. The secondary aim was to investigate inter-laboratory variation for the TGA-CAT method.

'On-demand' romiplostim therapy in immune thrombocytopenia.

What Is Known And Objective: Although thrombopoietin receptor agonists are a second-line treatment for refractory immune thrombocytopenia (ITP), we lack guidelines recommending maintenance modality in patients who achieve complete remission (CR).

Case Summary: We report a patient with refractory ITP who achieved CR on romiplostim. Obtaining romiplostim for 6 months of therapy, we decided to try extending this by modifying the standard treatment regimen.

Myeloid sarcoma of the skin in a patient with myelodysplastic syndrome.

We report the case of a 76-year-old woman who presented with asymptomatic extensive erythematous. Firm plaques were noted over the right cheek. Complete blood count was normal, as was a peripheral smear.

Comparison of standard fibrinogen measurement methods with fibrin clot firmness assessed by thromboelastometry in patients with cirrhosis.

Background: The Clauss fibrinogen method and thrombin clotting time (TCT) are still routinely used in patients with cirrhosis to define fibrinogen concentration and clotting potential. The thromboelastometric functional fibrinogen FIBTEM assay evaluates the strength of fibrin-based clots in whole blood, providing information on both quantitative deficit and fibrin polymerization disorders.

Objective: To compare these three methods of assessing fibrinogen in patients with cirrhosis of different aetiologies, characterized by impairment in fibrinogen concentration as well as functional aberrance.

Diagnostic challenges during pretreatment long-term follow-up in a patient with FIP1L1-PDGFRA-positive eosinophilia.

Obtaining a precise characterization of eosinophilia is crucial, as successful treatment relies on the underlying etiology of the disease. Platelet-derived growth factor receptor alpha-related disorders were first specified in 2008 as a distinct group of clonal eosinophilic disorders with exceptional responsiveness to imatinib. We herein present the case of a man with myeloid neoplasm and eosinophilia in whom a definitive diagnosis could not be adequately made based on histopathological features who was ultimately diagnosed only after extensive molecular analyses and successfully treated with imatinib.

Thrombotic events in acute promyelocytic leukemia.

Introduction: Thrombotic events (TE) appear to be more common in acute promyelocytic leukemia (APL) than in other acute leukemias, with reported prevalence ranging from 2 to 10-15%.

Materials And Methods: We retrospectively analyzed the data on TE appearance in 63 APL patients.

Results: TE occured in 13 (20.

Rationale for individualizing haemophilia care.

Owing to the heterogeneity in the clinical phenotype of haemophilia A and B, it is now recognized that disease severity (based on factor VIII/IX activity) may no longer be the most appropriate guide for treatment and that a 'one-size-fits-all' approach is unlikely to achieve optimal therapy. Based on the present literature and consensus views of a group of experts in the field, this article highlights key gaps in the understanding of the diverse relationships between bleeding phenotype and factors such as joint health, genetic susceptibility, laboratory parameters, quality of life and management of pain. Early prophylaxis is a potential 'gold standard' therapy and issues surrounding inhibitor development, variations in its clinical use and long-term outcomes are discussed.

The importance of cardiovascular risk factors for thrombosis prediction in patients with essential thrombocythemia.

The current widely accepted stratification defined by age and previous thrombosis in patients with essential thrombocythemia (ET) probably deserves deeper analysis. The aim of our study was to identify additional factors at the time of diagnosis, which have an impact on the thrombosis prediction. We conducted a study of 244 consecutive ET patients with median follow-up of 83 months.

Comparison of two laboratory assays in monitoring the efficacy of different prophylaxis regimens for severe haemophilia.

Background: The goal of this study was to compare the validity of two laboratory assays, rotation thromboelastometry (ROTEM) and endogenous thrombin potential (ETP), in monitoring and evaluating different prophylactic treatment regimens in patients with severe haemophilia.

Methods: Twenty adult patients with severe haemophilia were divided into three groups according to treatment regimen with concentrate of factor (F) VIII/IX: full-dose prophylaxis (5 patients), intermediate-dose prophylaxis (5 patients), and on demand treatment (10 patients).

Results: The ROTEM for the group treated with full-dose prophylaxis was significantly lower than ROTEM for the group treated with intermediate-dose prophylaxis (p = 0.

[Obstetric and gynecological intervention in women with Bernard-Soulier syndrome: report of two cases].

Unlabelled: Introduction Bernard-Soulier syndrome (BSS) is a rare inherited bleeding disorder characterized by giant platelets thrombocytopenia, prolonged bleeding time, frequent hemorrhages with considerable morbidity. Data on the outcome of pregnancy and gynecological intervention in BSS are rare and there are no general therapeutic recommendations.

Cases Outline: We report two cases of BSS.

Complex haemostatic abnormalities as a cause of bleeding after neurosurgery in a patient with Gaucher disease.

We report a treatment-naïve patient with Gaucher disease (GD) who experienced repeated bleeding after three neurosurgeries for a brain tumour, identified as an oligoastrocytoma. The patient had normal values on basic haemostatic tests: prothrombin time, 75-105%; activated partial thromboplastin time, 30.3-34 s; and mild thrombocytopaenia, 96-115 × 10(9 )cells/l.

Multicentre, randomized, open-label study of on-demand treatment with two prophylaxis regimens of recombinant coagulation factor IX in haemophilia B subjects.

Few randomized studies have reported on the use of factor IX (FIX) for secondary prophylaxis in haemophilia B patients. This study aimed to evaluate the efficacy and safety of two secondary prophylaxis regimens of recombinant coagulation FIX, nonacog alfa, compared with on-demand therapy. Male subjects aged 6-65 years with severe or moderately severe haemophilia B (FIX:C ≤ 2, n = 50) and ≥12 bleeding episodes (including ≥6 haemarthroses episodes) within 12 months of study participation were enrolled in this multicentre, randomized, open-label, four-period crossover trial.

The effect of paraprotein on platelet aggregation.

Background: Some patients with paraproteinemia have platelet aggregation disorders and the aim of this study was to examine disturbance of platelet aggregation in healthy blood donors by isolated paraprotein in vitro.

Methods: Using Rivanol, paraprotein was separated from the serum of ten patients with paraproteinemia, who had decreased platelet aggregation with several inducers. Platelet aggregation in ten healthy donors was measured with and without addition of the isolated induced paraprotein.

Acquired von Willebrand syndrome in patients with Gaucher disease.

Although various coagulation abnormalities occur in patients with Gaucher disease (GD), von Willebrand factor (vWF) deficiency has rarely been reported. A retrospective review of six treatment naïve cases with GD and concomitant vWF deficiency over a 12-year-period in a single center is presented. All patients had a personal history of prior hemorrhages.

Improvement of fibrin clot structure after factor VIII injection in haemophilia A patients treated on demand.

Patients with haemophilia A have seriously impaired thrombin generation due to an inherited deficiency of factor (F)VIII, making them form unstable fibrin clots that are unable to maintain haemostasis. Data on fibrin structure in haemophilia patients remain limited. Fibrin permeability, assessed by a flow measurement technique, was investigated in plasma from 20 patients with severe haemophilia A treated on demand, before and 30 minutes after FVIII injection.

Specific binding of paraprotein to platelet receptors as a cause of platelet dysfunction in monoclonal gammopathies.

The study included 48 untreated patients with monoclonal gammopathies (MG). Paraprotein was isolated from the serum of 10 patients with decreased platelet aggregation. Platelet aggregation was measured before and after the addition of the isolated paraprotein to platelet-rich plasma (PRP) from 10 healthy donors, in vitro.

International Society of Thrombosis and Hemostasis Scoring System for disseminated intravascular coagulation ≥ 6: a new predictor of hemorrhagic early death in acute promyelocytic leukemia.

High-hemorrhagic early death (ED) rate is a major impediment in the managing of acute promyelocytic leukemia (APL). In our group of 56 newly diagnosed APL patients, ED occurred in 12 subjects, due to endocranial bleeding (8/12), differentiation syndrome (2/12), or infection (2/12). Predictors of hemorrhagic ED were as follows: white blood cells count ≥ 20 × 10(9)/L (P = 0.

Is there a "gold" standard treatment for patients with isolated myeloid sarcoma?

Isolated myeloid sarcoma is an extramedullary tumor of immature myeloid cells defined by the absence of leukemia history, myelodisplastic syndrome, or myeloproliferative neoplasma with a negative bone marrow biopsy. Myeloid sarcoma is a very rare condition, and few cases have been reported. We reviewed data of 12 patients with isolated myeloid sarcoma managed at a single center to determine the possible prognostic factors affecting patient survival, such as age, sex, type, localization, and treatment options.

Successful non-standard approaches to massive hemoptysis in invasive pulmonary aspergillosis.

Introduction: Invasive pulmonary aspergillosis (IA) is the most frequent invasive fungal infection in patients with hematological malignancies. Massive hemoptysis (MH) with blood loss more than 300-600 ml in 24 hours is a rare (5-10% of IA patients) but frequently fatal complication. Standard treatment of MH, such as oxygenation, a semi-sitting position with the bleeding site down, bronchoscopical suctioning, antifungal therapy, transfusion support and surgical resection might be either ineffective or not feasible in some cases.

Beyond patient benefit: clinical development in hemophilia.

Historically in hemophilia, outcome measures have not been collected systematically. Hence, there are insufficient clearly defined, evidence-based measures that can be applied consistently across hemophilia trials. This review focuses on some key challenges to evaluating patient outcomes and performing trials identified by experts at the Fourth and Fifth Zurich Haemophilia Forums.

Two global haemostatic assays as additional tools to monitor treatment in cases of haemophilia A.

Haemophilia A patients with similar levels of factor VIII (FVIII) may have different bleeding phenotypes and responses to treatment with FVIII concentrate. Therefore, a test which determines overall haemostasis may be appropriate for treatment monitoring in some patients. We studied two global haemostatic methods:endogenous thrombin potential (ETP) and overall haemostatic potential(OHP) before and after injection of FVIII concentrate in patients with haemophilia A treated prophylactically and on-demand.