Isolated splenic peliosis is an extremely rare condition characterized by the presence of multiple blood-filled cavities, occasionally resulting in non-traumatic splenic rupture with fatal bleeding. In our case, a 64-year-old man was brought by ambulance due to weakness and abdominal pain without nausea or febrility. On clinical examination, the patient was sensitive to palpation with significant tenderness over the abdomen but no associated features of peritonitis. He collapsed during the imaging examination and became unconscious and asystolic. Cardiopulmonary resuscitation was not successful. The patient died approximately within 2 hours of admission to the hospital. Postmortal examination showed 2800 ml of intraperitoneal blood with clots and a laceration of the lower pole of the spleen. Macroscopic examination of the spleen revealed huge nodular splenomegaly, measuring 21 cm x 19 cm x 5 cm, weighing 755 g. On the cut surfaces, multiple randomly distributed blood-filled cavities ranging from 0,5 to 2 cm in diameter were seen. At microscopic examination, the specimens showed multiple irregular haemorrhagic cyst-like lesions that were not lined by any epithelium or sinusoidal endothelium, consistent with the diagnosis of peliosis lienis. Although the condition is often clinically silent, the forensic pathological significance arises from the differential diagnosis of resultant intraperitoneal haemorrhage and sudden death, mimicking a violent death.

UNASSIGNED: Isolated splenic peliosis is an extremely rare condition. The associations of splenic peliosis with various infections, medications, and conditions have unclear significance. We present three patients from the past twenty years with spontaneous splenic rupture due to peliosis, two of whom had hematologic malignancy, to draw attention to a possible correlation.
METHODS: A 31-year-old male with essential thrombocytopenia and antiphospholipid-antibody syndrome presented with worsening abdominal pain and hypotension. The patient denied any trauma. Computed-tomography demonstrated hemoperitoneum and splenic rupture with innumerable blood-filled splenic cysts. An uncomplicated emergency open splenectomy was performed with shed-blood reinfusion. The patient was discharged on postoperative day five. The patient developed acute myelogenous leukemia and died six years later. A 44-year-old otherwise healthy male presented with left upper-quadrant and shoulder pain without reported trauma. Computed-tomography (CT) imaging revealed splenomegaly, multiple splenic cystic lesions, and free intraperitoneal blood. A laparoscopic splenectomy, complicated by a pancreatic leak that was managed with a drain, was performed. The patient was discharged on postoperative day three and was well at 37 months follow-up. A 78-year-old male with splenomegaly and chronic anemia on warfarin for atrial fibrillation presented in shock with a distended abdomen after falling from a standing height. The patient was resuscitated with two units of packed red blood cells and underwent emergent abdominal exploration. The spleen was ruptured. An open splenectomy was performed and four liters of intraperitoneal blood were evacuated. Pathology confirmed splenic peliosis and historic diffuse large B-cell lymphoma. The patient had an excellent response to chemotherapy but died 12 years later.
UNASSIGNED: Splenic peliosis is a rare vascular phenomenon of unclear etiology. Several toxic and pharmaceutical agents have been associated with spontaneous splenic rupture in patients with peliosis. There are also a number of reported patients who were noted to have hematologic disorders, suggestive of a potential association to the pathophysiology of peliosis.
CONCLUSIONS: Based on our clinical experience and focused literature review, it appears likely that there is a relationship between splenic peliosis and hematologic malignancy.

Peliosis is a rare entity that is characterized by cystic or blood-filled spaces in the parenchyma of solid organs. It is most commonly seen in the spleen in patients who also have peliosis hepatis. Isolated splenic peliosis is very rare and spontaneous rupture due to splenic peliosis is even more uncommon. This diagnosis has high importance in forensic pathology as it is a rare cause of atraumatic splenic rupture. We present a case in which the diagnosis of isolated splenic peliosis with massive hemoperitoneum was made at autopsy.