The results of the 2005 Survey of the Italian Society for Apheresis and Cell Manipulation (SIdEM) reporting on the pediatric procedures carried out in 18 Italian Apheresis Units are presented here. Utilizing a standardized questionnaire, the survey collected data on techniques, types of blood separators, clinical indications, and adverse events. A total of 1,693 apheresis procedures were carried out in 355 pediatric patients: 219 plasma-exchange, 291 peripheral blood stem cell collections, 791 extracorporeal photochemotherapy (ECP), 265 LDL-apheresis, 71 erythro-exchange, 9 cytoreductive apheresis, 47 immunoadsorption sessions. Adverse events were registered in 94 procedures (5.6%), most of which of mild entity, e.g., insufficient flow rate (50.0%) and symptomatic hypocalcemia (24.4%). Our data indicate that all types of apheresis procedures can be safely carried out in children. ECP, utilized primarily for the treatment of graft versus host disease (GvHD) and rejection of solid organ transplantation, are burgeoning procedures in pediatric patients, whereas plasma exchange, which is a common treatment in adults, is infrequently utilized in pediatric medicine.
A family with a new factor X defect is reported. The proposita is a 56-year-old female. She is asymptomatic and no consanguinity is present between the parents. The main features of the defect are: prolongation of prothrombin time and derivative tests but normal partial thromboplastin time. Factor X was found to be low (about 25–30% of normal) only if tissue thromboplastins were used in the assay system. Chromogenic substrate S-2222 also yielded decreased factor X levels. However, factor X activity was normal with cephalin and cephalin-RVV mixture. Factor X antigen was normal in three immunological systems (electroimmunoassay, an Elisa method and laser nephelometry). Crossed immunoelectrophoresis and antigen-antibody kinetics recorded in a laser nephelometer failed to show major differences from normal factor X. Both sons of the proposita, the father and other family members showed slightly decreased factor X levels and normal factor X antigen and were considered heterozygous for the abnormality. The toponym factor X Padua is proposed to indicate this peculiar abnormality.
Data collection on apheresis activities in Italy throughout 2005 including techniques, types of blood cell separators, clinical indications and adverse effects was performed by means of a standardized questionnaire. These data provided by 83 Apheresis Units from 16 Italian regions, albeit rough, are sufficiently informative, mainly in comparison with previous surveys on these statistics (1997 and 2000). In 2005 a total number of 204,746 apheresis procedures were carried out, with a clear-cut prevalence of apheresis production (87.7%), performed by 66 out of 83 Apheresis Units (79.5). Lombardy, Veneto and Tuscany were the most active regions for therapeutic apheresis (51.1% of the total national procedures). An increasing number in extracorporeal photochemotherapy as compared to the 2000 national survey (3,386 vs. 704 procedures) is the most striking observation to emerge from the 2005 data collection on therapeutic apheresis in Italy. Adverse effects, predominantly mild ones (i.e., paresthesia due to citrate-induced hypocalcemia), occurred in 0.12% of apheresis production and 6.04 of therapeutic sessions, particularly in the course of peripheral blood stem cell collection (20.79%), as already reported in the 2000 national survey.
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