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.

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Hemophilia A (HA) is a rare bleeding disorder characterized by reduced factor VIII (FVIII) activity and consequently spontaneous bleeding. Since the introduction of prophylaxis with safer FVIII concentrates, people with HA are ageing. Interestingly, they are developing cardiovascular diseases as their non‐hemophilia counterparts. We describe a 48‐year‐old patient with severe HA who presented a third‐degree atrioventricular block. A DDDR pacemaker was implanted under supervision of the Hematology Clinics. There were no adverse events during the procedure. The procedure was safe, and it should be performed under the supervision of a hemophilia expert.

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This case gives us the lesson that we need to include ASVC in the differential diagnosis of SVCS. The importance of the V-Leiden factor as a joint risk with this congenital defect for venous thromboembolism episodes was also highlighted.