RESUMO
Antiphospholipid syndrome (APS) is a clinical autoimmune disorder characterized by thrombosis/pregnancy morbidity associated with the persistence of lupus anticoagulant (LA) and/or anticardiolipin (aCL) antibodies. We assessed the contribution of antibodies to beta2-glycoprotein I (anti-beta2GPI) and prothrombin (anti-PT) to the thrombotic risk in a cohort of 194 consecutive patients with persistent LA and/or aCL. Median follow-up was 45 months. A total of 39 patients (20.1%) had one documented episode of thrombosis during follow-up. Eleven of these patients had no previous thrombosis before enrollment in the study and 28 had recurrences of thrombosis. There were 21 venous and 18 arterial thrombotic events and the overall incidence of thrombosis was 5.6% per patient-year. After multivariate analysis, the male sex (P = 0.025), a previous thrombosis (P < 0.01), the presence of anti-beta2GPI (P = 0.001), and the presence of anti-PT (P = 0.03) remained as independent risk factors for recurrent thrombosis. Only IgG anti-beta2GPI and anti-PT were associated with an increased risk of thrombosis (P < 0.01 and P = 0.005). Patients testing positive for anti-beta2GPI had a higher rate of thrombosis than did antiphospholipid patients without anti-beta2GPI (8.0% vs. 3.1% per patient-year). Similarly, a higher rate of thrombosis was found in patients with positive anti-PT compared with patients without anti-PT (8.6% vs. 3.5% per patient-year). Considering only the group of 142 LA positive patients, the highest incidence of thrombosis was found in LA patients positive for both anti-beta2GPI and anti-PT (8.4% per patient-year). In conclusion, the presence of IgG anti-beta2GPI and anti-PT in patients with LA and/or aCL and mainly in those with LA predicts a higher risk of thromboembolic events.
Assuntos
Autoanticorpos/sangue , Glicoproteínas/imunologia , Protrombina/imunologia , Trombose/etiologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Anticorpos Anticardiolipina/sangue , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Humanos , Imunoglobulina G , Incidência , Inibidor de Coagulação do Lúpus/sangue , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Recidiva , beta 2-Glicoproteína IRESUMO
The recently described monocytoid B-cell lymphoma is a low-grade lymphoma presenting most frequently in elderly women and commonly associated with autoimmune diseases. Leukaemic expression of this disease has been reported in advanced stages. A case of monocytoid lymphocytosis without lymph node enlargement is presented herein. A 60-year old woman complaining of easy bruises was found to have a 2-cm splenomegaly. Her laboratory data included the following: haemoglobin, 125 g/L; haematocrit, 0.35 L/L; white cell count, 29 x 10(9)/L with 32% PMN, 3% stabs, 2% myelocytes, 1% metamyelocytes, 30% lymphocytes and 32% atypical mononucleated cells showing wide, pale cytoplasm neatly contoured and oval nucleus with monocytoid features. The basal coagulation study showed prothrombin 50%, APTT 40 seconds, fibrinogen 68 mg/dL and FDP between 80 and 160 ng/dL. Splenomegaly without lymph-node enlargement was found on CT scan. The bone-marrow biopsy showed a 68% monocytoid lymphocytic infiltration, acid-phosphatase positive and tartrate-sensitive, without fibrosis. Bone-marrow and peripheral immunophenotype showed those cells to be CD22, CD 19 and CD11 positive, while T and CD25 markers were absent. The patient was treated with alpha-2b interferon at a dose of 3MU three times a week for 6 months, with general improvement and regression of the leukaemic expression. Eleven months after diagnosis she died of a central nervous system haemorrhage. The morphological, immunological and cytochemical features of the monocytoid lymphocytes in this case are commented, along with their variable behaviour. A review of the literature is also carried out, attention being laid on the onset and the response to therapy of B-cell monocytoid lymphomas as the singularity of this case lies on its exclusively leukaemic onset. It is concluded that an interrelationship between monocytoid B-lymphocytic leukaemia and B-cell monocytoid lymphoma might possibly exist, such as that between chronic lymphocytic leukaemia and diffuse lymphocytic lymphoma.
Assuntos
Leucemia de Células B , Medula Óssea/patologia , Feminino , Humanos , Fatores Imunológicos/uso terapêutico , Imunofenotipagem , Interferon alfa-2 , Interferon-alfa/uso terapêutico , Leucemia de Células B/complicações , Leucemia de Células B/patologia , Leucemia de Células B/terapia , Pessoa de Meia-Idade , Células-Tronco Neoplásicas/imunologia , Células-Tronco Neoplásicas/patologia , Púrpura/etiologia , Proteínas Recombinantes , Esplenomegalia/etiologia , Resultado do TratamentoRESUMO
The effectiveness of a commercial drug containing fibrinogen, thrombin and factor XIL (Tissucol, Immuno) was assessed in 127 patients receiving oral anticoagulant treatment with acenocoumarin who were subjected to 183 minor surgical procedures: 107 exodontia, 53 periodontal procedures, 17 combinations of the former, 4 liver biopsies and 2 skin biopsies. All but the liver biopsies were performed in the outpatient clinic. Mild haemorrhage appeared in 21 instances. None of the patients required systemic administration of coagulation factors, and the maneuvers did not take any longer than in patients with integrity of the coagulation mechanisms. The outstanding benefits of this technique are: less discomfort for patients, who can be subjected to a single procedure while otherwise requiring several sessions; anticoagulation needs not be discontinued, subcutaneous heparin being otherwise necessary; low risk of complications and avoidance of substitutive therapy; lesser economic burden, as no hospital admission is needed.