Background: Primary antiphospholipid syndrome (PAPS) is characterized by arterial and venous thrombosis, pregnancy loss, often recurrent, in the presence and persistence on antiphospholipid antibodies (aPL). The issue of early atherosclerosis, as evaluated by measuring carotid intima media thickness (IMT), associated with aPL, has been limitedly explored in PAPS.
Methods: In an age- and sex-matched case-double-control study, intima media thickeness of carotid arteries was measured using high-resolution B-mode ultrasound in 49 thrombotic PAPS patients (18 M, 31 F, mean age 37+/-11), in 49 patients who suffered thrombosis for inherited thrombophilia and 49 healthy subjects.
Rheumatology (Oxford)
December 2008
Objective: To test the inflammation and immune activation hypothesis in primary thrombotic APS (PAPS) and to identify clinical and laboratory factors related to inflammation and immune activation.
Methods: PAPS (n = 41) patients were compared with patients with inherited thrombophilia (IT, n = 44) and controls (CTR, n = 39). IgG aCL, IgG anti-beta2-glycoprotein I (beta(2)GPI), high-sensitivity CRP (hs-CRP), serum amyloid A (SAA), CRP bound to oxidized low-density lipoprotein-beta(2)GPI complex (CRP-oxLDL-beta(2)GPI) (as inflammatory markers) neopterin (NPT), soluble CD14 (sCD14) (as immune activation markers) were measured by ELISA.
Antiphospholipid syndrome (APS) is the most common cause of acquired thrombophilia, but experimental and clinical evidence accumulated over the years suggest that the clinical manifestations of APS go beyond those of a simple hypercoagulable state. Although still a controversial topic, the elevated risk of atherosclerosis in systemic lupus erythematosus seems little accounted for by the presence of antiphospholipid antibodies, whereas premature atherosclerosis has been addressed in few series of patients with primary APS. The available data in primary APS suggest that traditional risk factors for atherosclerosis are less involved in arterial disease, rather antiphospholipid antibodies appear as major players.
View Article and Find Full Text PDFBudd-Chiari syndrome is a rare disease due to occlusion of the hepatic veins often presenting with acute liver failure. Common causes of Budd-Chiari syndrome are chronic myeloproliferative disorders, while acute leukemia has been associated with hepatic vein thrombosis in only two cases in the literature to date. We report a case of Budd-Chiari syndrome complicating a non-promyelocytic acute myelogenous leukemia leading to fulminant hepatic failure.
View Article and Find Full Text PDFThe contribution of pro-thrombotic factors towards the development of arterial disease (AD) and splanchnic vein thrombosis (SVT) was retrospectively evaluated in 79 patients (39M, 40F, mean age 55 +/- 16 years) with myeloproliferative disorders (MPD) (essential thrombocythemia [n = 26], primary proliferative polycythemia [n = 27], and idiopathic myelofibrosis [n = 26]). Of these, 18 had AD and 17 SVT, the remaining 44 were non-thrombotic (NT). Plasma concentrations of natural anticoagulants, plasma homocysteine (HC), IgG anticardiolipin antibodies (aCL), and thrombophilic genotypes (methylenetetrahydrofolate reductase C(677)T, factor V Leiden, prothrombin G(20210)-->A) were determined.
View Article and Find Full Text PDFForty-five patients suffering from advanced B-CLL were randomized to receive interferon-alpha (IFN alpha) or no treatment after achieving complete remission or partial response, following a chemotherapy protocol called MiNa. The two groups were fully comparable in terms of clinical characteristics and level of response obtained by chemotherapy. IFN alpha was given at a dose of 3 megaunits three times a week intramuscularly for 1 year.
View Article and Find Full Text PDFIn a prospective study on 44 cases of T-cell origin acute lymphoblastic leukemia, 20 patients were found to display an immature immunophenotype (CD7+, CD4-, CD8-, CD1-) and were classified as T-stem cell leukemia (T-SCL). Twenty-four patients expressed CD4 and/or CD8 antigens on their blast cells, designated T acute lymphoblastic leukemia (T-ALL). The T-SCL subset showed a significantly higher median age, a more frequent incidence of extramedullary leukemia, a morphology L1 in most cases, and a poor response to treatment in terms of either complete remission rate or median survival duration.
View Article and Find Full Text PDFA case of a 55 years old woman suffering from multiple myeloma with strong bone marrow proplasmocytic infiltration, several osteolytic and osteoporotic lesions and high seric M-component level and hypertensive heart failure is described. After 32 months of partial remission obtained with cyclic chemotherapy, large cutaneous tumors arose. Despite of a new therapeutic trial, in the last 8 months, an increase of bone marrow and seric signs was observed without involvement of the lungs or kidneys or expression of plasma-cell leukemia.
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