Many patients worldwide receive platelet components (PCs) through the transfusion of diverse types of blood components. PC transfusions are essential for the treatment of central thrombocytopenia of diverse causes, and such treatment is beneficial in patients at risk of severe bleeding. PC transfusions account for almost 10% of all the blood components supplied by blood services, but they are associated with about 3.25 times as many severe reactions (attributable to transfusion) than red blood cell transfusions after stringent in-process leukoreduction to less than 106 residual cells per blood component. PCs are not homogeneous, due to the considerable differences between donors. Furthermore, the modes of PC collection and preparation, the safety precautions taken to limit either the most common (allergic-type reactions and febrile non-hemolytic reactions) or the most severe (bacterial contamination, pulmonary lesions) adverse reactions, and storage and conservation methods can all result in so-called PC "storage lesions". Some storage lesions affect PC quality, with implications for patient outcome. Good transfusion practices should result in higher levels of platelet recovery and efficacy, and lower complication rates. These practices include a matching of tissue ABH antigens whenever possible, and of platelet HLA (and, to a lesser extent, HPA) antigens in immunization situations. This review provides an overview of all the available information relating to platelet transfusion, from donor and donation to bedside transfusion, and considers the impact of the measures applied to increase transfusion efficacy while improving safety and preventing transfusion inefficacy and refractoriness. It also considers alternatives to platelet component (PC) transfusion.
Background: Little is known about autoimmune myelofibrosis (AIMF), a rare entity that can occur alone or in association with another autoimmune disease (AID) and is responsible for bone marrow (BM) failure and life-threatening complications. Objectives: We conducted a nationwide retrospective observational study of AIMF cases to better characterize the epidemiology, clinical presentation and evolution of this rare entity. Methods: The aim of the study was to analyze the characteristics of AIMF and the nature and indication of treatments currently used. Response to treatment was evaluated by the revised Tefferi et al. response criteria. Results: Among 30 cases of AIMF, the sex ratio (F/M) was 4:1 and the median age at diagnosis was 37 years (interquartile range 30–49). AIMF was diagnosed after the onset of an associated AID in 12 cases and concomitant to an AID in the remaining 18 cases. The most frequently associated AID was systemic lupus erythematous, followed by Sjögren syndrome. All cases consisted of reticulin fibers, and no collagen fibrosis was described. More than 50% of cases showed complete response after first-line therapy, with glucocorticoids (GC) in 28 cases. Half of the cases had treatment complications mainly related to GC therapy. Conclusion: Diagnosis of AIMF remains challenging in the absence of a validated set of diagnosis criteria, and must always be searched in the presence of hematological abnormalities at onset or during follow-up of AID. Clinical context, search for mutations and pathology findings can help differentiating this rare disease from a clonal pathology. GC is currently an effective first-choice therapy for AIMF, but a high rate of GC dependency and long-term complications indicate the need to find new sparing drugs. Disclosure of Interests: PHILIPPE MERTZ: None declared, Emilie Chalayer: None declared, Jean Sibilia: None declared, Jacques-Eric Gottenberg Grant/research support from: BMS, Pfizer, Consultant of: BMS, Sanofi-Genzyme, UCB, Speakers bureau: Abbvie, Eli Lilly and Co., Roche, Sanofi-Genzyme, UCB, Anne-Sophie Korganow: None declared, Laurent Arnaud: None declared, Thierry Martin: None declared
Venous thromboembolism (VTE) remains a critical issue in the management of patients with multiple myeloma (MM), particularly when immunomodulatory drugs (IMiDs) combined with dexamethasone therapy are being prescribed as first-line and relapse therapy. One possible explanation for the persistent high rates of VTE, is the use of inappropriate thromboprophylaxis strategies for patients starting antimyeloma treatment. To tackle the issue, the Intergroupe francophone du myélome (IFM) offered convenient guidance for VTE thromboprophylaxis in MM patients initiating systemic therapy. This guidance is mainly supported by the results of a large survey on the clinical habits regarding VTE of physicians who are substantially involved in daily care of MM patients. VTE prophylaxis should be considered for all patients treated with IMiDs in combination with dexamethasone, in the absence of significant comorbidities, such as renal failure or bleeding risk. Anticoagulant should be preferred to antiplatelet agents for thromboprophylaxis. Despite the absence of large randomized controlled trials comparing those attitudes/options, available data on direct oral anticoagulants, which are already used in daily management of MM patients, are consistent with their potential usefulness for VTE prophylaxis in such patients. However, in order to implement a personalized continuous improvement strategy, clinicians must to be organized to collect all the data regarding this management. In other situations, thromboprophylaxis should be evaluated by using risk models and after careful evaluation of the risk/benefit ratio.
