Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, progressive, and life-threatening hematopoietic stem cell disorder characterized by complement-mediated intravascular hemolysis and a prothrombotic state. Patients with PNH might have slightly increased risk of infections due to complement-associated defects subsequent to CD59 deficiency. Here, we report a rare case of a 65-year-old male patient with necrotic ulcers on both legs, where the recognition of pancytopenia and microthrombi led to the diagnosis of PNH based on FLAER (FLuorescent AERolysin) flow cytometric analysis. He was subsequently started on eculizumab therapy, with starting and maintenance doses set as per drug labelling. Progression of the patient’s leg ulcers during follow-up, with fulminant tissue destruction, purulent discharge, and necrotic patches, led to a later diagnosis of necrotizing fasciitis due toPseudomonas aeruginosaandKlebsiella pneumoniainfection. Courses of broad-spectrum antibiotics, surgical debridement, and superficial skin grafting were applied with successful effect during ongoing eculizumab therapy. This case highlights the point that it is important to maintain treatment of underlying disorders such as PNH in the presence of life-threatening infections like NF.
The aim is to evaluate if patients reach the level of competence that enables them to make the best decision for themselves with oral and written informed consent process that is legally valid. This study included 62 patients who applied to Ege University Hospital Department of General Surgery Endocrine Surgery Clinics and in whom oral and written informed consent was obtained by a surgeon. Patients who were willing to participate in the study were asked to fill in a questionnaire containing questions regarding the concept of consent. Seventy-one percent of patients were female and 29% were male, with a mean age of 50.4±17.9 years. Six percent of patients were illiterate, 51.6% had primary education and 16.1% were college graduates. One in every two patients stated that they have never heard of informed consent concept before, 16% stated that they signed the consent without reading it. Among these patients, 88% reported that they trusted the physician and did not bother reading because they have already been verbally informed. Verbal briefing by the doctor was detected as 92%. Similarly, 91% of the patients reported that the time allocated to read and fill-in the form was enough. Informing is not composed simply of conveying information, but also to elevate patients to a proficiency level where they could decide with their best interest. It is thought that the results obtained in this study might guide studies to improve the quality of information in patients undergoing surgery.