BackgroundThrombotic thrombocytopenic purpura is a very rare hereditary blood deficiency disorder of ADAMTS13 (von Willebrand factor-cleaving protease) and a life-threatening thrombotic microangiopathy characterized by thrombocytopenia and microangiopathic hemolytic anemia. The deficiency in ADAMTS13 metalloprotease, which cleaves the von Willebrand factor, may be congenital or acquired. The congenital form is caused by inherited mutations in the ADAMTS13 gene. The diagnosis is challenging due to the nonspecific signs and symptoms, as well as the rarity of the disease.Case presentationWe present an unusual case of a 20-year-old feoderm woman from northeast region of Brazil who manifested thrombocytopenia during her pregnancy which was believed to be immune thrombocytopenic purpura.ConclusionsConsidering the importance of a differential diagnosis of thrombotic microangiopathic disorders, congenital thrombotic thrombocytopenic purpura may mimic the signs and symptoms of pre-eclampsia/eclampsia, hemolysis with elevated liver enzymes and low platelet count syndrome, and atypical hemolytic-uremic syndrome. It should be considered in suspect cases in patients with an ADAMTS13 activity at 5% without ADAMTS13 antibodies.

O prognóstico de gastrosquise permanece adverso nos países em desenvolvimento e os fatores associados a óbito não são conhecidos. O estudo objetivou avaliar os fatores associados com morte neonatal em casos de gastrosquise atendidos no IMIP. Foram incluídos 49 casos de gastrosquise atendidos no IMIP, Recife-Brasil, entre 1995 e 2001. A variável dependente foi morte neonatal e as independentes foram: diagnóstico pré-natal, local e tipo de parto, idade gestacional, peso ao nascer, intervalo entre parto e admissão e parto-cirurgia, correção cirúrgica primária ou em estágios, necessidade de ventilação mecânica e infecção pós-operatória. Calculou-se a razão de prevalência (RP) de morte neonatal com intervalo de confiança a 95%, realizando-se análise de regressão logística para determinar o risco ajustado de óbito. A mortalidade foi de 53% (26 casos), sendo infecção a principal causa de óbito (92%). O diagnóstico pré-natal associou-se com significante redução (74%) do risco de morte. Esse risco foi significantemente aumentado ( > 2 vezes) para RNs, com peso menor que 2,5Kg e provenientes de outros hospitais. Também verificou-se aumento significante do risco para o intervalo entre parto-admissão e parto-cirurgia maior que duas e quatro horas (respectivamente, 2,5 e 3,4). O risco de morte foi 2,6 vezes maior nos casos com ventilação mecânica. Na análise multivariada, persistiram associadas ao óbito a prematuridade e o intervalo entre parto e cirurgia maior que 4h. Observou-se uma elevada mortalidade entre recém-nascidos com gastrosquise, que pode ser explicada por fatores como ausência de pré-natal, prematuridade, baixo peso, parto fora dos centros terciários, longos intervalos entre parto e cirurgia e necessidade de ventilação mecânica.

Introduction: Acquired hemophilia A is very rare autoimmune disorder caused by an autoantibody to factor VIII that interferes with its coagulant function. It may be associated with a number of conditions such as lymphoproliferative disorders, drugs and solid malignancies. Hemarthrosis in Acquired hemophilia A is very rare clinical presentation and was never reported in cases induced by penicillin. Case presentation: We report a 65-year-old male, born and living in Brazil, who presented hemorrhagic episodes complicated with joint hemarthrosis due to acquired hemophilia A caused by a commonly used drug, Benzathine penicillin.Disease management was focused on controlling bleeding, primarily with the use of factor VIII inhibition bypassing activity and eradicate the autoantibody using various immunosuppressants. The challenges in manage this case included delayed diagnosis, durable remissions, difficulty achieving hemostasis. Clinical and laboratory coagulation response has improved eight weeks after only with the administration of double immunosuppression medication: corticosteroid and cyclophosphamide. Conclusion: This case gives us the lesson that prompt diagnosis and treatment achieved by hematologists may improve the prognosis and prevent severe bleeding. Early detection is vital to improving outcomes.