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Sorasio R.,Santa Croce e Carle Hospital | Bonferroni M.,Santa Croce e Carle Hospital | Grasso M.,Santa Croce e Carle Hospital | Rapezzi D.,Santa Croce e Carle Hospital | And 11 more authors.
Biology of Blood and Marrow Transplantation

Several algorithms for early prediction of poor-mobilizing patients after chemotherapy and granulocyte colony-stimulating factor administration have been proposed. They generally define peripheral blood cut-off levels of CD34+cells at a fixed day after starting chemotherapy, mostly with cyclophosphamide. To define an algorithm for early addition of plerixafor regardless of the chemotherapy regimen used, we retrospectively analyzed 280 chemomobilization attempts in 236 patients treated at our institution between 2002 and 2012. In multivariate analysis, CD34+absolute count and CD34+percentage upon total leukocyte count at day 1 (defined as the first day in which leukocytes reached a value>1×109/L) were the only factors able to predict a total harvest≥2×106 CD34+/kg. In patients with day 1 CD34+lower than 20/μL, the CD34+percentage was a more reliable predictor of stem cell harvest in the following days than CD34+absolute count. Upon definition of the best CD34+ cut-off value for identification of poor-mobilizing patients, an algorithm was set up to guide plerixafor administration. It was prospectively validated in 20 patients in 2013 with encouraging results in terms of low incidences of both mobilization failure and plerixafor use. Large prospective trials that define the most cost-effective strategy for just-in-time rescue plerixafor are warranted. © 2014 American Society for Blood and Marrow Transplantation. Source

Franchini M.,Immunohematology and Transfusion Medicine | Coppola A.,University of Naples Federico II
Seminars in Thrombosis and Hemostasis

In spite of coagulation impairment, people with congenital bleeding disorders can still develop atherosclerosis and its thrombotic complications. This issue has been particularly addressed in recent years as an increasing number of such patients now reach an elderly age and have to confront age-related comorbidities, including cardiovascular diseases, and as a consequence, challenges concerning the management of concomitant bleeding and atherothrombotic risk. Von Willebrand disease (VWD), caused by quantitative and/or functional defects of von Willebrand factor (VWF), is the most common congenital bleeding disorder, with an estimated prevalence in the general population of 1 to 2%, although clinically significant VWD is much less common. Despite the high population impact of VWD and increasing knowledge of the pathophysiological role of VWF in atherothrombosis, data concerning atherosclerosis and its vascular complications in VWD patients are rather limited, and even more scarce when clinical management is considered. The relevance of this association is certainly underestimated and, possibly, contributes to bleeding complications observed in patients on antithrombotic treatment or undergoing invasive cardiovascular procedures. This review will analyze the available literature data and discuss the implications for management of VWD patients with atherothrombosis, in the light of the information of bleeding risk in the general population and of recent, growing data from hemophilia patients. © 2012 by Thieme Medical Publishers, Inc. Source

Di Minno M.D.,University of Naples Federico II | Ambrosino P.,University of Naples Federico II | Franchini M.,Immunohematology and Transfusion Medicine | Coppola A.,University of Naples Federico II | Di Minno G.,University of Naples Federico II
Seminars in Thrombosis and Hemostasis

Chronic arthropathy is a major complication in severe hemophilia A (Factor [F] VIII < 1%). Almost all adults with severe hemophilia, who have not received prophylaxis with FVIII since their early childhood, suffer from chronic arthropathy. Patients with moderate hemophilia (FVIII activity 1-5%) usually experience fewer joint bleeds than those with severe hemophilia and are thought to rarely develop a significant degree of chronic arthropathy. However, some patients with moderate hemophilia behave like those with the severe form of the disorder, reporting several joint bleeds per year and significant joint impairment. Currently, only little data are available about the prevalence of arthropathy, the degree of quality of life impairment, and the need for orthopedic care/aids in patients with moderate hemophilia. In this systematic review of literature, the prevalence of overt arthropathy ranges between 15 and 77% in patients with moderate hemophilia and prophylactic replacement treatment is prescribed in approximately 30% of these patients, usually after diagnosis of clinically overt arthropathy. Moreover, because of the lack of imaging studies (magnetic resonance and/or ultrasound), the prevalence of subclinical arthropathy cannot be determined. These data confirm that severity of hemophilia should not be defined only according to FVIII levels and that a relevant proportion of patients with nonsevere hemophilia might benefit from a "tailored early prophylaxis. © 2013 by Thieme Medical Publishers, Inc. Source

Coppola A.,University of Naples Federico II | Tagliaferri A.,University of Parma | Di Capua M.,University of Naples Federico II | Franchini M.,Immunohematology and Transfusion Medicine
Seminars in Thrombosis and Hemostasis

Recurrent joint bleeding leading to progressive musculoskeletal damage (hemophilic arthropathy), in spite of on-demand replacement with deficient factor concentrates, is the clinical hallmark of severe hemophilia A and B (i.e., the congenital deficiencies of coagulation factors VIII and IX with circulating levels <1 IU/dL). Fifty years of clinical experience, which began in Northern Europe and then initiated in other European countries and in North America, up to the recent randomized clinical trials, have provided definitive evidence that preventing bleeding from an early age through long-term regular prophylactic concentrate infusions limits the adverse clinical consequences of arthropathy and its complications in the quality of life of hemophilic children. Primary prophylaxis started after the first joint bleed and/or before the age of 2 is now the evidence-based, first-choice treatment in severe hemophilia. Interestingly, recent data also suggest a role for early prophylaxis in preventing inhibitor development, the most serious complication of hemophilia therapy. Secondary prophylaxis is aimed to avoid (or delay) the progression of arthropathy. The earlier the treatment is started, the better the outcomes in joint status and quality of life. Although prophylaxis has radically transformed the natural history of severe hemophilia, relevant barriers to its implementation and diffusion remain. Beyond the obvious economic constraints and problems with venous access and long-term adherence, uncertainties regarding the optimal prophylaxis regimen require further evaluation in prospective studies to optimize approaches based on definite outcome measures and cost-effectiveness/cost-utility analyses. Scientific evidence, current clinical strategies, and open issues of prophylaxis in children with hemophilia will be addressed in this review. © 2012 by Thieme Medical Publishers, Inc. Source

Tagliaferri A.,University of Parma | Franchini M.,Immunohematology and Transfusion Medicine
Journal of Thrombosis and Haemostasis

See also Shetty S, Ghosh K. Cancers in patients with hemophilia: a retrospective study from the Italian Association of Hemophilia Centers: a rebuttal. This issue, pp 1200-1. Tagliaferri A, Di Perna C, Santoro C, Schinco P, Santoro R, Rossetti G, Coppola A, Morfini M, Franchini M, on behalf of the Italian Association of Hemophilia Centers. Cancers in patients with hemophilia: a retrospective study from the Italian Association of Hemophilia Centers. J Thromb Haemost 2012; 10: 90-9. © 2012 International Society on Thrombosis and Haemostasis. Source

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