Search

Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy (TMA) characterized by the development of microangiopathic haemolytic anaemia, thrombocytopenia, and ischaemic organ dysfunction associated with ADAMTS13 levels lower than 10% in most cases. Recently there have been numerous advances in the field of PTT, new, rapid and accessible techniques capable of quantifying ADAMTS13 activity and inhibitors. The massive sequencing systems facilitate the identification of polymorphisms in the ADAMTS13 gene. In addition, new drugs such as caplacizumab have appeared and relapse prevention strategies are being proposed with the use of rituximab. The existence of TTP patient registries allow a deeper understanding of this disease but the great variability in the diagnosis and treatment makes it necessary to elaborate guidelines that homogenize terminology and clinical practice. The recommendations set out in this document were prepared following the AGREE methodology. The research questions were formulated according to the PICO format. A search of the literature published during the last 10 years was carried out. The recommendations were established by consensus among the entire group, specifying the existing strengths and limitations according to the level of evidence obtained. In conclusion, this document contains recommendations on the management, diagnosis, and treatment of TTP with the ultimate objective of developing guidelines based on the evidence published to date that allow healthcare professionals to optimize TTP treatment.

Although frozen platelets are extensively used in remote locations and military environments, scientific evidence of their efficacy is scarce. The objective of this study was to evaluate the in vitro hemostatic efficacy of frozen versus fresh platelet transfusions by rotational thromboelastometry (ROTEM) to ascertain whether the freezing and thawing process impaired platelet contribution to clot strength.An experimental study was performed using platelet in vitro transfusions. Blood samples were collected from 12 patients with non-autoimmune thrombocytopenia. The samples were each transfused with one of 6 pairs of fresh platelet concentrates and platelet concentrates frozen with dimethylsulfoxyde. Optical platelet counts, coagulation studies and ROTEM (EXTEM and FIBTEM) were performed for the baseline and the post-transfusion samples.Only fresh platelet transfusions significantly increased the EXTEM maximum clot firmness (MCF) and maximum clot elasticity (MCE) over baseline (p0.001), achieving values within the normal range. The frozen platelet contribution to MCE was negligible. However, the EXTEM clotting time (CT) was significantly (p0.001) shorter after the frozen platelet transfusion compared with the fresh platelet transfusion. The EXTEM clot formation time (CFT) was significantly shortened after the transfusion of fresh platelets (p=0.002).The ROTEM analysis assessment indicates a dual effect in frozen platelet transfusion: it produces a hypercoagulable state (shortening of CT), and a second, more predominant effect of frozen platelets' functionality impairment compared with fresh platelets (shorter MCF/MCE and longer CFT).

Resumen La hemocromatosis neonatal (HN) es una enfermedad hepatica grave neonatal con alta tasa de mortalidad y recurrencia. Se postula un origen aloinmunitario, resultado de la presencia de un anticuerpo materno aun por determinar que interfiera con el metabolismo ferrico fetal. En esta teoria se basa el tratamiento materno con inmunoglobulinas por via intravenosa en gestaciones sucesivas. Describimos el caso de una paciente con un hijo anterior diagnosticado y fallecido por HN, que tratamos en la siguiente gestacion con inmunoglobulinas por via intravenosa. Es el primer caso descrito en Espana y demuestra el exito de la terapia, tal como describe la literatura cientifica.

La hemocromatosis neonatal es una enfermedad hepatica muy severa del recien nacido y se asocia a una alta mortalidad. Se cree que su etiologia es de tipo aloinmune, debido a la presencia de un anticuerpo materno hasta ahora desconocido que interfiere con el metabolismo ferrico del feto, llegando a producir gran morb i mortal idad. Basandonos en esta teoria, el tratamiento materno con inmunoglobulinas intravenosas en gestaciones sucesivas podria prevenir el desarrollo de un nuevo cuadro de hemocromatosis neonatal. Se describe el caso de una gestante con un hijo anterior diagnosticado y fallecido neonatalmente por hemocromatosis, a la que en el embarazo actual se le trato con inmunoglobulinas intravenosas consiguiendo un hijo sano y vivo. Es el primer caso descrito en Espana y demuestra el exito de esta terapia, tal como describe la literatura.

There is an evident need for procedural protocol for oral surgery patients who undergo oral anticoagulant treatment (OAT) because of: 1) the possible severity of complications and 2) the growing demand for OAT, which in some cases may be as much as 8% of the oral surgery patients that are referred to the hospital from primary care centers. In this study, the authors define the parameters for creating a proto- col applicable to this group of patients. The conclusion is that it is not necessary to suspend OAT before surgery; rather, these procedures should be performed under multidisciplinary medical control.The authors demonstrate that it is possible to perform oral surgery on OAT patients, without having to sus- pend treatment beforehand.A longitudinal study was performed in OAT patients that required some type of oral surgical procedures. After an INR control, the patient underwent surgery and afterwards the patient was given tranexamic acid as a mouth rinse. Postoperative hemorrhage was classified as slight when it lasted less than 5 minutes, moderate when it lasted longer than five minutes, and severe when it required blood transfusion.The study was performed over a 5-year period (1996-2000), by the maxillofacial surgery department. In that time period, 125 patients with OAT were treated; 90 of them were males and 35 were females. Tooth extraction was per- formed in 229 sessions and a total of 367 teeth were extracted, with an average of 1.6% per session. With regards to postoperative hemorrahage, it was slight in 210 cases (91.7%), moderate in 18 (7.9%) and severe only in one case (0.4%). All the variables were compared and no statistically significant differences were found.We believe that OAT should not be suspended before oral surgery, but it surgery should be performed under multidisciplinary control-especially in the case of the elderly (over 65) or with those patients that have other concomitant illnesses such as renal insufficiency or anemia or other medical treatments.