[Acute pulmonary thromboembolism in polysplenia syndrome]

We present a case of azygos continuation of the inferior vena cava, combined with polysplenia syndrome in a 72-year-old woman who presented with circulatory collapse due to acute pulmonary thromboembolism. Patients with polysplenia have multiple spleens, and their splenic function is usually normal, but this case was not. In this case, defective splenic function was associated with a high risk of fulminant bacterial infections, especially with encapsulated bacteria. The clinical features and prognosis of this entity are discussed.