“…In this regard, we published a case of splenic lymphangiomatosis in 2011, which in subsequent review was confirmed to correspond to KLA. This case involved a 22-year-old patient with no previous symptoms, who died of hemorrhagic shock and consumptive coagulopathy, with extensive peripancreatic and perisplenic hemorrhage 2. In this case, the spleen weighed 106 g and had a normal external appearance, with minimal microcysts that were difficult to visualize on cut section.…”