Results: 16 patients were eligible to analysis. The median age at diagnosis was 34 years (15-48); 10 males and 6 females. The referral motive was: respiratory complaints -37.5%; bone changes -37.5%; dental complaints -25%; constitutional symptoms -19%; mucocutaneous lesions -6% and one patient (6%) was accidentally diagnosed after a thyroidectomy. The tissue of histological diagnosis was: bone -50%; pulmonary tissue -37.5%; liver, genital mucosa and thyroid -6%, respectively. Staging was: single organ involvement (uni/multifocal) -69% and multisystem disease in 31%. Clinical re-evaluation of these cases is being done at the moment. The median follow up was 5 years (1 month -11 years) and the overall survival was 92%. Currently 19% are alive without signs of disease; 44% are alive with disease; 25% are under treatment and 12% died. Discussion: These results agree with published literature. Considering the actual guidelines 56% patients were incompletely staged, which probably lead to suboptimal treatment. There is heterogeneity of clinical procedures aiming at staging and treatment of these patients. Conclusion:The diagnosis of adulthood Langerhans cell histiocytosis is difficult considering the diversity of clinical behavior. Frequently this also leads to diagnosis delay. Prospective international clinical trials enrolling adult patients are important.
We present the case of a male patient not vaccinated against hepatitis B virus (HBV) and with reactivity to a surface antibody who, after immunosuppression for a multiple myeloma, had HBV reactivation. Pharmacological HBV suppression was tried, but viremia could not be suppressed. Production-detection core mutations or immunity issues can explain this clinical phenomenon. CASE REPORT
SummaryAfter the injection of DDAVP in 39 non-anaesthezised dogs (0.4 μg/kg) factor VIII: C activity rose to 145% of baseline values (p <0.0001) and the fibrinolytic potential of euglobulin precipitate rose to 196% (p <0.0001). The injection of DDAVP was repeated three times in each dog of a group of good responder animals at weekly intervals, but after: A) Pentobarbital anesthesia (30 mg/kg) - the increase of factor VIII: C was reduced from 164% to 116% (n = 11; p <0.0005) and the increase in fibrinolytic activity was reduced from 270% to 192% (n = 11; p <0.05). B) Injection of propranolol (1 mg/kg) - the increase of factor VIII :C was reduced from 167% to 110% (n = 13; p <0.0005) and there was no significant decrease of fibrinolytic activity (n = 13; n.s.). C) Splenectomy - the increase of factor VIII: C was reduced from 166% to 122% (n = 10; p <0.0005) and fibrinolytic activity was not significantly changed from 196% to 256% (n = 9; n.s.). There were no statistically significant differences in the increases of factor VIII :C and fibrinolytic potential of euglobulin precipitate after repeating only the injection of DDAVP three times in the same animal at weekly intervals (n = 5; n.s.). We conclude that the increases in factor VIII: C and fibrinolytic activities observed after DDAVP infusion in the dog are due to different mechanisms of action. Pentobarbital anesthesia reduced the increase of factor VIII: C and fibrinolytic activity, but on the other hand, beta-blockade and splenectomy influenced differently the behaviour of both biological activities.Injection of the synthetic analogue of vasopressin 1-deamino-8-d-arginine vasopressin (DDAVP) increases the plasma levels of factor VIII procoagulant activity (factor VIII: C) and plasminogen activator both in man and dog (1, 2, 3, 4).The mechanism of action of DDAVP in determining these responses is not well known. In this work, after having confirmed that DDAVP increases factor VIII: C and fibrinolytic activity in the blood of the unanaesthezised dog, and in order to clarify the mechanisms involved in these pharmacological effects, we repeated the DDAVP injection in animals previously submitted to propranolol, pentobarbital anesthesia and splenectomy.
CONTEXT: Primary amyloidosis, also known as AL amyloidosis, is commonly caused by clonal expansion of plasma cells in the bone marrow, thereby segregating light chains of clonal immunoglobulin that settle in tissues in the form of insoluble amyloid fibrils. The aim of this study was to report a case of primary amyloidosis with renal failure, diagnosed in Hospital São João, Porto, Portugal, focusing on the diagnostic difficulties and presenting a literature review. CASE REPORT:A 68-year-old Caucasian man was admitted to the Internal Medicine Department of the hospital with a condition of anasarca and nephrotic syndrome. After performing a renal biopsy that tested positive using Congo red and immunohistochemistry, lambda light chain amyloidosis was diagnosed. This evolved into terminal renal disease, which led to hemodialysis and several episodes of urinary and catheter infections. He was started on chemotherapy, consisting of bortezomib 0.7 mg/m 2 and dexamethasone 40 mg in six cycles. This led to clinical improvement, stabilization of the illness and good tolerance of the treatment.CONCLUSION: Amyloidosis is a rare entity that is difficult to diagnose. This is because of the unspecific early clinical manifestations of the disease.The hypothesis of amyloidosis is only considered when specific organ failure occurs. This case consisted of primary amyloidosis with involvement of the kidneys as an initial presentation of the disease and its difficulties were shown, going from the clinical approach to the final diagnosis. RESUMOCONTEXTO: A amiloidose primária, também conhecida como amiloidose AL, é geralmente causada pela expansão clonal de plasmócitos na medula óssea que segregam cadeias leves de imunoglobulina clonal, as quais se depositam nos tecidos na forma de fibrilas amiloides insolúveis. O objetivo deste estudo é relatar um caso de amiloidose primária com acometimento renal diagnosticado no Hospital São João, Porto, Portugal, enfatizando as dificuldades do diagnóstico e apresentando uma revisão da literatura.RELATO DO CASO: Homem de 68 anos, branco, foi admitido no Serviço de Medicina Interna do hospital com quadro de anasarca e síndrome nefrótica. Após realizar biópsia renal, que foi positiva para o vermelho congo e imunoistoquímica, foi diagnosticada amiloidose de cadeia leve lambda. Evoluiu para doença renal terminal, o que levou a hemodiálise e tendo vários episódios de infecções urinárias e do cateter. Iniciou a quimioterapia com bortezimib, 0,7 mg/m 2 , e dexametasona, 40 mg em seis ciclos, levando a uma melhoria clínica, a estabilização da doença e boa tolerância ao tratamento.CONCLUSÃO: Amiloidose consiste em uma entidade rara e de difícil diagnóstico. Isso ocorre devido a manifestações clínicas da doença pouco específicas, e esta hipótese só é considerada quando do acometimento de um órgão em particular. O caso em questão refere-se a uma apresentação da amiloidose primária com envolvimento renal, como apresentação clínica inicial da doença, e as dificuldades desde a abordagem clínica até o dia...
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