RESUMO
There has been an exponential increase in incidence of severe aortic stenosis partially due to the lengthening of average lifespan. Among the most disabling symptoms of aortic stenosis are chest pain, fatigue, and dyspnea up to heart failure and pulmonary edema. In some cases, to worsen this symptomatology, there are coagulation disorders linked to an alteration of functional von Willebrand factor, responsible for progressive anemia. In elderly patients with severe aortic stenosis, the simultaneous presence of an angiodysplasia of the colon can favor blood dripping, which may cause iron deficiency anemia. The coexistence of colonic angiodysplasia and acquired von Willebrand disease in patients with aortic stenosis was identified as Heyde's syndrome. In the long term, Heyde's syndrome can contribute to worsen the clinical manifestations of severe aortic stenosis leading to heart failure. Here, we describe the case of a patient suffering of severe calcific aortic stenosis who developed Heyde's syndrome achieving a condition of heart failure with mildly reduced ejection fraction. Learning objectives: Severe aortic stenosis can alter the conformation of the circulating von Willebrand glycoprotein, causing an alteration of the hemostatic balance. When angiodysplasia of the colon coexists with aortic stenosis, a gastrointestinal blood drip can occur inducing an iron deficiency anemia that worsens the symptoms of aortic valvulopathy. This condition often remains undiagnosed. We discuss the pathophysiologic and hemodynamic mechanisms responsible for acquired von Willebrand syndrome in patients with severe aortic stenosis focusing on the clinical elements useful to raise the diagnostic suspicion and analyzing different alternative tools to recognize it promptly.
RESUMO
Background: Waldenstrom's disease is characterized by the presence of pathological changes in the B lymphocytes that are in the last stages of maturation. One characteristic of WM is the production of an abnormal high amount of IgM and hyper viscosity syndrome. The MW gets worse, symptoms such as fatigue, weight loss, night sweats, fever, recurrent infections and swollen lymph nodes develop in patients who have a known history of MGUS. In this clinical case, our patient without history of MGUS, presents for the first time for medical observation only for ascites and the presence of an interportocaval lymph node package. An atypical presentation of the disease that makes us reflect on the difficulty of making a diagnosis in the elderly patient and on pathogenetic hypotheses of ascites not yet explored. Case Presentation: Seventy-three-year-old patient, hospitalized for the onset of ascites with sloping edema, diffuse left pulmonary opacification. At the ultrasound check, cava and portal vessels patent and of regular caliber, however with inversion of flow in correspondence with the right branch and of the door to the hilum, with a subdiaphragmatic retrocaval focus with a maximum diameter of about 3 cm, which cannot be better viewed. CT scan of the abdomen with confirmation of the presence of an interportocaval lymph node package. After evidence of the electrophoretic protein picture of a double component, probably monoclonal with positive urinary immunofixation for free K chains. IgM dosage equal to 2190 mg. Serum immunofixation practice that confirms the diagnosis of type B lymphoproliferative syndrome as per Waldenstrom's disease, confirmed by bone marrow aspiration with morphological and flow cytometric study. Immediately begin chemotherapy with Bendamustine 120 mg. After 4 weeks of therapy with the reduction of IgM values, the patient no longer presented ascites. Conclusion: This case has an unusual presentation of this disease and we could shed a new light on the possible pathogenesis of portal hypertension in Waldenstrom'disease.