8 research outputs found

    erebral sinovenous thrombosis and acquired antithrombin, protein C and S deficiency during chemotherapy in a young man: Report of a case

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    L-asparaginase is commonly used in the chemotherapy regimens for acute lymphoblastic leukaemia. Its use is associated with thrombotic complications in 1 to 14 % of the cases. The pathogenesis of this complication is still unclear. However, the decrease of antithrombin seems to play an important role. We report a case of a 17-year old man with a acute lymphoblastic leukaemia, who developed a cerebral sinovenous thrombosis due to an acquired deficiency of antithrombin and protein C and S following L-asparaginase chemotherapy. We discuss the use of prophylactic supplements of antithrombin and the value of screening of thrombophilia based on the recent medical literature.info:eu-repo/semantics/publishe

    Iron status and treatment modalities in transfusion-dependent patients with myelodysplastic syndromes.

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    Transfusion dependency and iron overload are common among patients with myelodysplastic syndromes (MDS) treated with red blood cell (RBC) transfusions. Transfusion dependency is associated with leukemic progression and shorter survival. Guidelines recommend iron chelation therapy to manage iron overload, however little is known about the chelation patterns in daily clinical practice. The objective of this multicenter, retrospective, cross-sectional, observational study was to evaluate iron status and its management in transfusion-dependent MDS patients. A total of 193 patient records from 29 centers were eligible for inclusion. Median patient age was 76, and median age at diagnosis of MDS was 74. Patients had received an average of 13.4 ± 7.6 RBC units in the past 4 months; 44% had received more than 50 units since their MDS diagnosis. Medium serum ferritin was 1,550 μg/L. Ninety patients (46.6%) received iron chelation therapy with either deferoxamine (41%), deferasirox (36%), and deferoxamine followed by deferasirox (23%). There were no statistically significant differences between chelated and nonchelated patients in terms of International Prognostic Scoring System (IPSS), French-American-British (FAB), and/or World Health Organization (WHO) status, though chelated patients had received more RBC transfusions (p = 0.014). Iron chelation therapy may be underutilized in transfusion-dependent patients. Undertreatment can be reduced by complementing sound clinical judgment with the generally accepted guidelines of a serum ferritin level >1,000 μg/L and/or two or more RBC transfusions per month for the past year; considering patients on the basis of their IPSS, FAB, and/or WHO status; and individually tailored treatment regimens. Prospective randomized trials are necessary to establish causally the efficacy of iron chelation therapy in MDS

    Rituximab in auto-immune haemolytic anaemia and immune thrombocytopenic purpura: A Belgian retrospective multicentric study

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    Dierickx D, Verhoef G, Van Hoof A, Mineur P, Roest A, Triffet A, Kentos A, Pierre P, Boulet D, Bries G, Lê P-Q, Janssens A, Delannoy A (University Hospital Gasthuisberg, Leuven; Algemeen Ziekenhuis St. Jan, Brugge; Grand Hôpital de Charleroi, Gilly; ZNA Stuyvenberg, Antwerpen; Centre Hospitalier Universitaire, Charleroi; ULB Hôpital Erasme, Brussels; Cliniques du Sud-Luxembourg, Arlon; CHR Saint Joseph, Mons; Virga Jesseziekenhuis, Hasselt; Children's University Hospital Reine Fabiola, Brussels; and Hôpital de Jolimont, Haine-Saint-Paul and Cliniques Universitaires St Luc, Brussels; Belgium). Rituximab in auto-immune haemolytic anaemia and immune thrombocytopenic purpura:a Belgian retrospective multicentric study. J Intern Med 2009;Objectives. For better characterizing the effect of anti-CD20 therapy, we analysed the use of rituximab in Belgian patients experiencing auto-immune haemolytic anaemia (AIHA) and immune thrombocytopenic purpura (ITP). Design. We performed a retrospective multicentric analysis of patients with AIHA and ITP treated with rituximab in Belgium. Setting. Haematological departments were invited to fill in a questionnaire about patient and disease characteristics. Subjects. All patients with AIHA and ITP, both primary and secondary to other diseases, who received one or more courses of rituximab during their disease course were included. Sixty-eight courses of rituximab in 53 patients with AIHA and 43 courses in 40 patients with ITP were analyzed. Intervention. Response rates, duration of response and factors predictive for response were assessed. Results. All patients were given rituximab after failing at least one previous line of treatment, including splenectomy in 19% and 72.5% of AIHA-patients and ITP-patients respectively. Overall response rates were 79.2% in AIHA and 70% in ITP, with a median follow-up since first rituximab administration of 15 months (range 0.5-62) in AIHA and 11 months (range 0-74) in ITP. Progression free survival at 1 and 2 years were 72% and 56% in AIHA and 70% and 44% in ITP. In this retrospective analysis we were not able to identify pretreatment characteristics predictive for response to rituximab. Nine patients with AIHA and three patients with ITP were given one or more additional courses of rituximab. Most of these patients, who had responded to a previous course, experienced a new response comparable to the previous one, both in terms of quality and of duration of response. Finally, the outcome of patients who failed to respond to rituximab therapy was poor both in terms of response to subsequent therapy and in terms of survival. Conclusions. This study confirms that rituximab induces responses in a majority of previously treated patients with AIHA and ITP. Response duration generally exceeds 1 year. Retreatment with rituximab in responding patients is most often successful. The outcome of patients who fail on rituximab is poor. We were not able to identify pretreatment patient characteristics predicting for response. © 2009 Blackwell Publishing Ltd.SCOPUS: ar.jFLWINinfo:eu-repo/semantics/publishe

    Adequate iron chelation therapy for at least six months improves survival in transfusion-dependent patients wih lower risk myelodysplastic syndromes

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    Background: Most patients with myelodysplastic syndromes (MDS) require transfusions at the risk of ironoverload and associated organ damage, and death. Emerging evidence indicates that iron chelation ther-apy (ICT) could reduce mortality and improve survival in transfusion-dependent MDS patients, especiallythose classified as International Prognostic Scoring System (IPSS) Low or Intermediate-1 (Low/Int-1).Methods: Follow-up of a retrospective study. Sample included 127 Low/Int-1 MDS patients from 28 centersin Belgium. Statistical analysis stratified by duration (≥6 versus <6 months) and quality of chelation (adequate versus weak). Results: Crude chelation rate was 63% but 88% among patients with serum ferritin ≥1000 g/L. Of the 80chelated patients, 70% were chelated adequately mainly with deferasirox (26%) or deferasirox followingdeferoxamine (39%). Mortality was 70% among non-chelated, 40% among chelated, 32% among patientschelated ≥6 m, and 30% among patients chelated adequately; with a trend toward reduced cardiacmortality in chelated patients. Overall, median overall survival (OS) was 10.2 years for chelated and 3.1years for non-chelated patients (p < 0.001). For patients chelated ≥6 m or patients classified as adequatelychelated, median OS was 10.5 years. Mortality increased as a function of average monthly transfusionintensity (HR = 1.08, p = 0.04) but was lower in patients receiving adequate chelation or chelation ≥6 m(HR = 0.24, p < 0.001). Conclusion: Six or more months of adequate ICT is associated with markedly better overall survival. Thissuggests a possible survival benefit of ICT in transfusion-dependent patients with lower-risk MDS
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