1,721,006 research outputs found
Direct oral anticoagulants for the treatment of Mondor's disease not responding to low-molecular weight heparin
No full textMondor's disease is a rare condition and usually treated with low-molecular weight heparin and non-steroidal anti-inflammatory drugs. Because of paucity of cases and for the usually spontaneous resolution, there is not a standard treatment strategy and the use of oral anticoagulation in controversial. We reported the efficacy of direct oral anticoagulants in the recurrent Mondor's disease refractory to standard therapy
Right elbow arthropathy in a patient with severe haemophilia A
No full textA 56‐year‐old male had been diagnosed with severe haemophilia A at the age of one year (missense mutation, exon 10, variant NM_000132.3c.1537+1G). He had been treated on demand with plasma‐derived factor VIII (pdFVIII) concentrate for most of his life; he was not compliant with prophylaxis and attended the clinic only when he thought it was strictly necessary. In April 2017 he underwent an orthopaedic assessment, which showed severe arthropathy of both elbows, knees and ankles. In August 2017, he attended the clinic with a major haemarthrosis of the right elbow, which had not resolved with self‐medication. On physical examination, the right elbow was deformed with multidirectional laxity; paradoxically the range of motion was preserved. An X‐ray of the right elbow showed severe arthropathy with complete joint dislocation (upper images). There were also widespread areas of erosion and absorption of the articular surfaces; these were confirmed on computed tomography (CT) scanning (lower left image). The patient was treated for six days with pdFVIII concentrate at a dosage of 50 U/kg/day, and then the frequency of infusions was decreased to every other day. After the acute phase, he continued with pdFVIII 40 U/kg every other day with continued clinical improvement. A peripherally inserted central catheter was used to improve compliance with intravenous infusions. In April 2018 a switch was made to extended half‐life FVIII concentrate (recombinant FVIII‐Fc fusion protein, rFVIII‐Fc, efmoroctocog alpha) to reduce the frequency of infusions. Thereafter the patient accepted prophylaxis with rFVIII‐Fc, at a dosage of 50 U/kg every 96 h, with a FVIII trough level of approximately 10% and stable joint status.
Such severe joint damage is now uncommon in patients with haemophilia A. Our patient is a reminder of the possible consequences if prophylaxis is either unavailable or is not accepted by the patient
Immune thrombocytopenia management during COVID‐19 pandemic: An Italian monocentric experience
No full textOver the last 2 years, different cases of immune thrombocytopenia (ITP) in patients affected by SARS‐CoV2 have been reported. The management of SARS‐CoV2 in subjects with simultaneous or previous ITP can be challenging because of the great involvement of the haemostatic system in this viral infection. In this report, we describe the management and outcome of patients with newly diagnosed (ND), chronic and previous ITP, infected by COVID‐19, referred to the Haematology Institute of University Hospital Policlinico Umberto I in Rome. Steroids + immunoglobulins for ND or relapsed ITP and continuation of home therapy for chronic ITP are advised, although further knowledge is required
Oral anticoagulant therapy in Italian patients 80 yr of age or older with atrial fibrillation: a pilot study of low vs. standard PT/INR targets.
No full textBACKGROUND:
Oral anticoagulation therapy (OAT), which aims to prevent thromboembolism in patients with atrial fibrillation (AF), is underused in subjects who are over the age of 80 yr because of the associated bleeding risk. The aim of this study was to evaluate the efficacy and safety of OAT with low (2.0) vs. standard (2.5) PT/international normalised ratio (INR) targets in patients over the age of 80.
MATERIALS AND METHODS:
Of 233 patients aged 80 yr or older with AF on OAT, 58 had unstable PT/INR values and achieved reduced targets. These patients were enrolled as a group (A) in a case-control study and were treated with a low (2.0) PT/INR target. They were compared with a second group (B) of 58 additional patients who were matched for age and CHADS scores and treated with a standard (2.5) PT/INR target. Group A OAT parameters were also compared before and after the PT/INR reduction. The time in the therapeutic range (TTR%), PT/INR values >5, haemorrhages and strokes were prospectively evaluated in the two groups after 2 yr of follow-up.
RESULTS:
Of the 116 enrolled patients, 55 group A and 57 group B patients were evaluated. The TTR was 72.59% in group A and 64.43% in group B (P 5 was 0.68% for group A and 1.42% for group B (P 5 (1.72% vs. 0.68%; P < 0.001).
CONCLUSIONS:
A low PT/INR target seems effective and safe in Italian patients with AF over the age of 80. Further trials are needed to confirm the hypothesis generated by this study
Satisfaction, quality of life and therapy adherence assessment in real life patients transitioning from vitamin K antagonists to direct oral anticoagulants
No full textAnticoagulant therapy has undergone a significant change since direct oral anticoagulants (DOACs) introduction. Their obvious advantages including the fixed dose, the few interactions and less frequent controls, have made them the first choice anticoagulant therapy. More and more patients have therefore switched from therapy with vitamin K antagonists (VKAs) to DOACs. Aim of our study was to assess the satisfaction, quality of life (QoL) and therapy adherence of patients who switched from VKA to DOACs therapy. This single center study evaluated satisfaction and QoL of 107 patients who switched from VKA to DOACs therapy through Anti-Clot Treatment Scale and SF-36 respectively. The questionnaires were administered before therapy change, after 3 months of DOACs therapy and then annually. We also evaluated DOACs therapy adherence with a questionnaire administered each visit and through the measures of DOACs plasma levels. Patients' satisfaction and QoL were high during VKA therapy, but with DOACs we observed an improvement after the first 3 months and then maintained over the time of DOACs therapy. DOACs adherence was excellent, also confirmed by DOACs plasma levels
Different regimens of prophylaxis treatment in young severe hemophilia A patients: comparisons on efficacy, FVIII consumption, and therapy compliance
No full textPlasma vitamin K1 levels in Italian patients receiving oral anticoagulant therapy for mechanical heart prosthesis: a case-control study
No full textBackground
Oral anticoagulant therapy (OAT) with a vitamin K antagonist (VKA) is the choice of treatment for preventing thromboembolism in patients with mechanical heart valve prosthesis (MHP). The percentage of time in the therapeutic range (TTR%) expresses the OAT quality. We planned a case-control study in order to determine vitamin K1 plasmatic concentrations in MHP patients and to correlate these with TTR%.
Materials and Methods
Of 756 MHP patients receiving OAT, 125 patients (61 younger than 65 years, and 64 older than 65 years) and 120 healthy blood donors, matched for sex and age, were enrolled in the study. All subjects completed a living questionnaire regarding diet, and underwent blood collection. Vegetable and fruit intake was categorized as optimal or suboptimal, and the high-performance liquid chromatography method was used to determine vitamin K1 levels.
Results
Neither the patients nor controls had been taking vitamin supplements prior to the start of the study. The median vitamin K1 level was 290 pg/L in 72 controls with optimal intake, and 274 pg/L in 48 controls with suboptimal intake, while the median vitamin K1 level in MHP patients with optimal intake was 409 pg/L, significantly higher (p < 0.001) than the 133.5 pg/L in patients with suboptimal intake. Vitamin K1 concentration in MHP patients appears to be linked to an agerelated threshold: in patients younger than 65 years of age, the median vitamin K1 level was 431 pg/L, significantly higher (p < 0.05) than the 290 pg/L in patients older than 65 years of age. No clear relation was found between vitamin K1 levels and TTR% (Pearson = 0.14). However, patients with vitamin K1 > 160 pg/L showed a TTR% > 60 %. Among patients younger than 65 years, subjects with vitamin K1 > 160 pg/L showed a median TTR of 66 %, this being significantly higher (p < 0.001) than the 46 % level shown by patients with vitamin K1 > 160 pg/L.
Conclusions
Vitamin K1 concentrations in MHP patients seem to be related to both diet and age. © Springer International Publishing Switzerland 2016
Secondary prophylaxis of venous thromboembolism (VTE) with low dose apixaban or rivaroxaban in major-thrombophilia carriers
No full textDirect oral anticoagulants (DOACs) are widely used for treatment and secondary prophylaxis of venous thromboembolism (VTE) and represent the gold standard for VTE secondary prophylaxis, with low-intensity DOACs administration becoming increasingly used worldwide in such scenario. Albeit widespread DOACs usage there are few literature data regarding their efficacy and safety in major thrombophilia carriers and almost no data is available for low intensity apixaban and rivaroxaban as secondary VTE prophylaxis in such patients. The aim of our study is to evaluate and confront the efficacy and safety of low-dose DOACs for VTE secondary prophylaxis, in major thrombophilia carriers vs patients at high risk of VTE recurrence for other reasons. We retrospectively evaluated patients who required long-term anticoagulant secondary prophylaxis to prevent recurrent VTE, treated with apixaban 2.5 mg BID or rivaroxaban 10 mg daily and that were screened for thrombophilia. The examined patients were 339. Baseline characteristics such as sex, age, obesity rate, smoking, number of previous VTEs and comorbidities (such as cardiovascular diseases, diabetes, mild CKD) were equally distributed in either group. The median low-dose DOACs administration time was 19.20 months (IQR 12.17–35.67). During low-dose DOACs treatment, 13 (3.8%) VTE recurrences were observed; 14 bleeding events were registered (4,1%), with no major bleeding (MB), 6 clinically relevant non major bleeding (CRNMB) (1.8%) and 8 minor bleeding (2.3%). No statistically significant difference in the rate of VTE recurrence and/or bleeding events emerged between major thrombophilia carriers and non-major thrombophilia carriers. In multivariate analysis increased risk of VTE recurrence was found for patients with cardiovascular comorbidities (HR 4.00 – p = 0.034) and for patients with more than one previous VTE episode (HR 5.14 — p = 0.034). Our data suggest that low-dose DOACs may be effective and safe in secondary VTE prophylaxis for carriers of major thrombophilia with no increase in VTE recurrence and/or bleeding risk
Severe Thrombotic Complications in Congenital Afibrinogenemia: A Pathophysiological and Management Dilemma
No full textCongenital afibrinogenemia (CA) is a disease characterized by a complex pathophysiology, involving both the procoagulant and fibrinolytic systems, as well as platelet activity. Although hemorrhagic diathesis represents the most frequent clinical presentation of this disorder, severe thrombotic events can occur. It is not yet clear if these events are strictly related to the disease itself or to the fibrinogen replacement therapy. Different hypotheses on the pathophysiological mechanisms have been proposed. It is well known that fibrinogen/fibrin has a role in the downregulation of thrombin generation in plasma. In the absence of circulating fibrinogen, this "antithrombin" activity is missing and plasma thrombin levels rise; this excess of thrombin could promote clotting of the infused fibrinogen, initiating the thrombotic process. Furthermore, the observation of impaired plasmin generation in the plasma of CA patients has raised the hypothesis of a fibrinolytic system deficiency. We report the case of a CA male patient who at the age of 36 years experienced an arterial thrombosis in his left lower limb. Despite an aggressive medical treatment with low-molecular-weight heparin, fibrinolytic and antiplatelet agents, the arterial thrombosis progressed to the obstruction of the whole left arterial district and the patient underwent the amputation of the left lower limb. This case demonstrates the complexity of pathophysiology and clinical management of a "so-called" bleeding disorder as CA
Fostamatinib combined with TPO‐RAs or steroids as a bridge to monotherapy or as time‐limited continuous treatment in relapsed chronic ITP: A single‐centre case series
No full text- …
