Atraumatic splenic rupture is a serious intraabdominal emergency that requires emergent intervention. This can be due to a number of causes. In this case report, we introduce a rare cause of atraumatic splenic rupture, which is an otherwise benign asymptomatic disease that only manifests clinically upon rupture, namely splenic peliosis. There is limited existing knowledge concerning the disease\'s etiology and diagnosis; however, this study presents the possible etiological explanations, associated risk factors, and possible radiologic diagnostic modalities.

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.

BACKGROUND: Peliosis is a rare condition with anatomopathological characteristics that affect the liver. However, splenic peliosis is even more unique and rare. Patients with such abnormality usually exhibit no symptoms. Moreover, this is a lethal condition due to the high probability of splenic rupture accompanied by shock.
METHODS: We present a case of a 29-year-old Arab female who was admitted to the hospital with severe upper abdominal pain that started 1 week from the date of admission, associated with nausea, anorexia, low-grade fever, and vomiting, with no past medical history or comorbidities. A computerized tomography scan with contrast showed intraperitoneal free fluid along with multiple hypodense splenic cysts. Hence, an emergent exploratory laparotomy with splenectomy was performed. Splenic peliosis was confirmed by the histopathological examination.
CONCLUSIONS: Further investigations are warranted if peliosis is confirmed in one organ, for example, the liver, to detect its presence in any other potential organs that can be affected by peliosis. Splenic peliosis is extraordinarily rare. Furthermore, such a disease has no established management plan. Definitive treatment is surgical. Many aspects of splenic peliosis remain puzzling requiring more research in the near future.

We report findings from an autopsy case who died from massive bleeding because of splenic peliosis. The case subject was an 80-year-old man who had diabetes mellitus and who was receiving hemodialysis and anticoagulant therapy. Postmortem computed tomography demonstrated massive intra-abdominal hemorrhage especially seen around the spleen. At autopsy, we found abundant hemorrhagic ascites, including a large number of clots, in the abdominal cavity. The spleen had several distinct dark red areas ranging in size from 1.5 to 2.5 cm and showed spontaneous rupture along with hematoma formation on the outside of the splenic capsule on the anterior side. From these findings, we concluded that the cause of death in this case was massive hemorrhage owing to spontaneous rupture of splenic peliosis. Although peliosis itself rarely causes death, but when it is destroyed, massive bleeding leads to death. Thus, it is necessary to know the histopathological characteristics of peliosis, in forensics.

BACKGROUND: Atraumatic splenic rupture is a rare but life-threatening condition which may be associated with hematological malignancies.
METHODS: We present the case of a 63-year-old male patient with a history of chronic myelomonocytic leukemia and sarcoidosis under therapy with prednisone, who suffered an atraumatic splenic rupture with hemodynamic instability. He was managed with proximal splenic artery embolization and secondary open splenectomy. On pathology the diagnosis of peliosis lienalis was established.
CONCLUSIONS: Peliosis is a rare pathological entity, which presents with multiple blood-filled cavities within parenchymatous organs and is of unknown etiology and pathogenesis. In retrospect a rapid increase in splenomegaly and inhomogeneous parenchyma of the spleen on sonography was realized.
CONCLUSIONS: Sonographic changes in size and parenchyma of the spleen in patients with hematological malignancies might help suspecting peliosis lienalis with impending splenic rupture and could alter clinical management towards a prophylactic splenectomy.

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.