ABSTRACT
We report the case of a 39-year old male patient who presented with anaphylactoid shock and diffuse bleeding with prolonged activated partial thromboplastin time at the emergency room. The diagnosis of aggressive mastocytosis was suspected and then confirmed by raised tryptase level and mastocytic infiltration of the bone marrow. The outcome was favorable with supportive measures, antihistamine drugs, and imatinib mesylate.
ABSTRACT
Coronary surgery with diabetes and severe hemophilia B is a challenging situation requiring specific and adequate therapeutic considerations. We herein report the case of a 52-year-old diabetic patient with severe factor IX deficiency and impaired ventricular function, who was scheduled for myocardial revascularization because of exertional angina and recurrent myocardial infarctions following infusions of factor IX concentrate. The patient underwent a successful off-pump direct myocardial revascularization with neither hemorrhagic nor thrombotic complications. Hematological protocols and operative strategies are assessed and discussed.
Subject(s)
Coronary Artery Bypass, Off-Pump/methods , Diabetes Complications , Hemophilia B/complications , Ventricular Dysfunction, Left/surgery , Factor IX/administration & dosage , Humans , Male , Middle Aged , Postoperative Complications/prevention & control , Treatment Outcome , Ventricular Dysfunction, Left/complicationsABSTRACT
The aim of the study was to assess the respective roles of the half-life of elimination of oral anticoagulants and patient education as causes of instability of anticoagulation level in patients on oral anticoagulant therapy. Patients were randomised to receive either warfarin (long half-life) or acenocoumarol (short half-life) and either intensive or standard education, according to a factorial design. Instability of oral anticoagulant therapy was evaluated by the percentage of INRs and the time within the target range, and the variability between successive measurements. Compliance was assessed by means of electronic pill bottles. Eighty-six patients were included. Apart from the variability index, instability was similar between groups. Correlations between compliance and instability were observed only in the acenocoumarol group. No difference was found between the education groups. In patients starting oral anticoagulant therapy, dose determination may be the most important factor contributing to instability.
Subject(s)
Anticoagulants/administration & dosage , Anticoagulants/pharmacokinetics , Blood Coagulation/drug effects , Acenocoumarol/administration & dosage , Acenocoumarol/pharmacokinetics , Administration, Oral , Adult , Aged , Aged, 80 and over , Female , Half-Life , Humans , International Normalized Ratio , Male , Middle Aged , Patient Compliance , Patient Education as Topic , Warfarin/administration & dosage , Warfarin/pharmacokineticsABSTRACT
Excessive apoptosis is implicated in the pathogenesis of myelodysplastic syndromes (MDS). We assessed by flow cytometry the expression of several members of the Bcl-2 family in bone marrow mononuclear cells (BMMNC) of 168 MDS samples at diagnosis. The proteins studied were Bcl-2, Bcl-xL (anti-apoptotic), Bax, Bad, Bak, and Bcl-xS (pro-apoptotic). The percentage of BMMNC expressing Bcl-2 and Bcl-xL was higher in refractory anemia with excess of blasts (RAEB), RAEB in transformation (RAEB-T), and chronic myelomonocytic leukemia (CMML) than in refractory anemia (RA) and RA with ringed sideroblasts (RAS). Conversely pro-apoptotic proteins Bad, Bak, and Bcl-xS were detected in a higher percentage of cells in RA and RAS. RA and RAS were associated with an increased Bcl-xS/Bcl-xL ratio. The expression of anti-apoptotic proteins was also correlated with that of CD34 and P170 and with the percentage of blast cells. Two-color analyses demonstrated that CD34 and Bcl-2 were usually expressed in the same cells. No significant correlation was found with cytogenetic abnormalities. Higher expression of pro-apoptotic Bcl-2-family proteins (Bak, Bad, Bcl-xS) and higher Bcl-xS/Bcl-xL ratio were associated with longer survival and decreased risk of leukemic transformation in univariate analysis, whereas expression of anti-apoptotic proteins was associated with decreased survival. Consequently Bcl-2 proteins expression was well correlated with the International Prognostic Scoring System (IPSS). Our data confirm that the control of apoptosis is deregulated in MDS cells. Moreover, the study of markers such as CD34 (or Bcl-2), Bcl-xL, and Bcl-xS provides additional prognostic information.
