RESUMO
Factor XII (FXII) deficiency is a congenital disorder inherited as an autosomal recessive condition. In his heterozygous form, it is relatively common in the general population. However, a total absence of FXII as seen in homozygous patients, is rare, with an incidence of approximately 1/1,000,000 individuals. Surprisingly, FXII deficiency is rather associated with thromboembolic complications. Patients do not experience a higher risk of surgical bleeding despite a markedly prolonged activated partial thromboplastin time. Given its low incidence in the general population, the finding of an unknown FXII deficiency is rare during cardiac surgery. This unique case describes a patient with an unanticipated prolonged baseline activated clotting time (ACT) during cardiac surgery in which his bleeding history and rotational thromboelastometry tracings lead us to the diagnosis of a FXII deficiency. The finding of a hypocoagulable INTEM tracing and a concurrent normal EXTEM tracing in a sample of a patient with prolonged ACT and adverse anamnestic bleeding history should prompt clinicians to consider a FXII deficiency. It may help clinicians in further perioperative management where there is not enough time to wait for the results of individual coagulation factor testing.
Assuntos
Procedimentos Cirúrgicos Cardíacos , Deficiência do Fator XII , Fator XII , Deficiência do Fator XII/complicações , Deficiência do Fator XII/diagnóstico , Humanos , Tempo de Tromboplastina ParcialRESUMO
INTRODUCTION: Cancer induced bone pain (CIBP) is frequent in patients with non-small cell lung cancer (NSCLC). Radiation therapy continues to be the gold standard for treatment of painful bone metastases, however only a limited number of metastases can be irradiated. We evaluated non-radiation based early CIBP relief options in NSCLC through a systematic review. METHODS: Systematic review including all prospective articles published between 01-1994 and 06-2020 on Pubmed, Cochrane Library and ClinicalTrials.gov database. Inclusion: non-radiation based trials evaluating CIBP early pain relief options (initially defined as pain score evaluated within two weeks, because of no randomized trials, later inclusion broadened to pain score evaluated within six weeks) in ≥10 NSCLC patients. Radioisotope trials were excluded as these treatments have interactions with systemic anticancer therapy. RESULTS: 188 articles were found; 10 articles (6 randomized controlled (4 double blinded), 1 phase II single-arm, and 3 prospective trials) fulfilled the inclusion criteria. Six of these trials consisted of ≥2 treatment arms, whereas the others were single-arm studies. In total, 554 NSCLC patients were evaluated in these trials. The included trials were very heterogeneous regarding evaluated treatment options, methods of pain measuring, and endpoints. No high-level evidence for specific early pain relief treatment options was found. DISCUSSION: Non-radiation based studies evaluating treatment options to rapidly reduce CIBP in NSCLC are scarce. This systematic review shows that there is no high-level evidence to recommend a specific treatment for early pain relief. Future research should focus on early pain relief treatment options for CIBP in NSCLC.