L’objectiu d’aquestes pàgines se centra a analitzar, molt succintament, les principals disposicions normatives d’incidència en dret lingüístic que s’han publicat al Diari Oficial de la Generalitat de Catalunya (en endavant DOGC) durant el primer semestre d’enguany. Per tant, el període estudiat s’inclou dins la X legislatura del Parlament català, nascuda dels comicis anticipats celebrats el 25 de novembre de 201
Acquired haemophilia A (AHA) is a rare but clinically relevant bleeding disorder due to autoantibodies (inhibitors) against coagulation factor VIII (FVIII)1–3. AHA is usually triggered by infections, malignancies, autoimmune diseases, or pregnancy. Some cases associated with drug intake have also been described, but about half of cases remain unexplained and are classified as “idiopathic”. According to a 2-year prospective surveillance study from the UK, the incidence is approximately 1.5 cases per million persons per year1; the condition is most frequent in the elderly and the reported median age of affected individuals is above 70 years1,4,5. This acquired bleeding disorder should be suspected in subjects without a personal or family history of bleeding who have unexplained haemorrhages, prolonged activated partial thromboplastin time (APTT) and a normal prothrombin time. A mixing test which does not correct the APTT, in the absence of lupus anticoagulant and heparin administration, together with low FVIII levels and circulating FVIII inhibitor, confirm the diagnosis3. In the majority of cases bleeding is spontaneous and can range from mild to life-threatening1,3–5. Bleeding sites in patients with AHA differ from those associated with congenital haemophilia, traumatic muscle bleeds and haemarthroses being uncommon. Indeed, most events are spontaneous subcutaneous, deep muscle and retroperitoneal bleeds, although mucosal (gastrointestinal, lung, urogenital) and intracranial bleeds also occur. Bleeding is the main cause of death in the early phase of AHA and its severity does not correlate strictly with FVIII levels or inhibitor titre.The management of AHA is directed at controlling the bleeding, eradicating inhibitors in order to prevent subsequent haemorrhagic complications, and treating any underlying associated disease2,3. Early treatment of bleeds is recommended. Available haemostatic agents do not have predictable efficacy; therefore, clinical review by physicians experienced in the management of inhibitors, supported by appropriate imaging and laboratory studies, is important6–8. Among the different approaches to control haemorrhages in AHA patients, the use of desmopressin and antifibrinolytics is mostly anecdotal and reserved only for minor bleeding. In selected cases FVIII concentrates, either recombinant or plasma-derived, could be appropriate9.According to international recommendations, bypassing agents, i.e. recombinant activated factor VII (rFVIIa) and activated prothrombin complex concentrate (aPCC), are considered the first-line approach for the treatment of bleeding episodes6,7. The dosages and regimens of rFVIIa (Novoseven®) that have been reportedly used in AHA are widely heterogeneous3,10–13, but current recommendations suggest bolus administrations of 90 μg/kg every 2–3 hours, until haemostasis is achieved6–7, similar to the treatment in patients with congenital haemophilia and inhibitors. The largest available collection of treatment records, the European Acquired Haemophilia (EACH2) registry, showed consistent concordance among physicians (rFVIIa dosage range 84.7–102.9 μg/kg every 2–6 hours)13. Unfortunately, due to the rarity of AHA, no methodologically rigorous study has been conducted to determine the most effective and safest dosage in this setting. Bypassing agents are generally well tolerated in AHA patients, although severe thrombotic complications may occur, such as myocardial infarction, disseminated intravascular coagulation, arterial and venous thrombosis, pulmonary embolism and stroke11,13. Although a causal relationship between bypassing agents and such thrombotic complications cannot be established in most cases, caution is required, in particular taking into account that the elderly age and frequent comorbidities of patients treated with these agents increase the risk of thromboembolism3,9,14.Here we describe four patients with AHA whose bleeds were treated with lower dosages and/or longer intervals of bolus administrations of rFVIIa than those recommended. These regimens were chosen because of the high risk of thrombotic complications, given the patients’ risk profile, concomitant with the need to treat severe, even life-threatening, haemorrhagic events.
Dental extractions in haemophiliacs may cause secondary bleeding, requiring repeated surgical and haematological interventions. As a local haemostatic, fibrin glue has recognised efficacy but, as a plasma-derived product, it carries the risk of viral infections. We, therefore, compared fibrin glue with an autologous haemostatic, plasma rich in growth factors (PRGF), in a controlled trial.
The indications for transfusion therapy in the neonatal period are based on specific knowledge of various aspects of this particular period of life, such as the dynamic interaction of the mother-placenta-foetus/neonate, the pathophysiological changes in the perinatal and neonatal periods and the profound haematological modfications that are characteristic of the first weeks of life. Furthermore, the physiological immaturity of various organs and systems can expose neonates, in particular those with a very low birth weight (VLBW) (Appendix I), to metabolic alterations following the transfusion of various blood components and the additives in them, and to infectious and immunological risks, such as Graft-versus-Host disease (GVHD).\ud\udThis implies the need for close and continuous collaboration between paediatricians-neonatologists and transfusion medicine specialists in order to obtain “dedicated” blood components, both with regards to quality and quantity, able to meet the particular needs of the neonate, especially considering the now increased survival of extremely low birth weight (ELBW) babies.\ud\udELBW and “critically ill” neonates are categories of patients with high transfusion needs, even though the number of transfusions given to premature neonates has progressively decreased over the last decade. It is, however, essential to establish appropriate transfusion criteria for these subjects.\ud\udThe scientific contributions on transfusion medicine in the neonatal period derive predominantly from consensus of opinions rather than controlled studies and the lack of clear scientific evidence makes it difficult to formulate high-grade recommendations based on solid levels of evidence. Furthermore, it should be appreciated that neonatal transfusion medicine is, like all other scientific fields, a continuously evolving discipline. These Recommendations, which represent the opinions of the authors and include evidence-based data, when available, have been formulated to facilitate the implementation of uniform transfusion practices. They are not intended to provide absolute indications, but aim to be a “guide” which nevertheless guarantees individual healthcare professionals freedom of choice in the various different clinical situations.\ud\udThis document deals with pre-transfusion tests, indications for the transfusion of blood components, characteristics of the blood components and methods of their administration for neonates. Details on the levels of evidence and strengths of the recommendations are provided in Appendix II.\ud\udThis document does not consider the indications for the use of blood derivatives and some highly specialised, life-saving techniques used in particular emergencies, such as extracorporeal membrane oxygenation and cardiopulmonary bypass