Acute pulmonary embolism revealing Ivemark syndrome in an adult

Ivemark syndrome is a polymalformative syndrome, the cardinal element of which is asple- nia, described for the first time by Bjorn Ivemark in 1955. This condition is rare in adults, as the majority of children affected do not survive beyond their first year. We report the case of a 21-year-old, hitherto asymptomatic, male patient, in whom this syndrome was diagnosed.