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. 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.

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.

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.

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 lesion characterized by the presence of blood-filled cysts, with unknown true incidence and etiology. It has been most frequently reported to the liver (peliosis hepatis) and to other organs of the mononuclear phagocytic system, such as spleen, bone marrow and lymph nodes. However, other organs may also be affected. Its occurrence has been linked to wasting conditions such as tuberculosis, cancer, immunosuppression and the use of androgenic-anabolic steroids. Herein, we report a case of pulmonary peliosis, in a 29-year-old man who was abusing testosterone as it was proved by toxicological analysis. To our knowledge this is the third reported case of pulmonary peliosis and the first one that is not associated with peliosis of any other organ.

Isolated peliosis of the spleen, characterized by the gross appearance of multiple cystlike, blood-filled cavities on cut surfaces of the organ, is a very rare pathologic entity that has been reported in 33 cases to date. We present 2 additional cases of isolated peliosis of the spleen observed at autopsy and confirmed by histology. In both cases investigated, cirrhosis of the liver was present. We hypothesize that local microcirculatory disturbances manifesting under altered local intravascular pressure conditions in the spleen may, at least to a certain degree, be responsible for the peliosis-associated vascular lesions. For the forensic pathologist, isolated peliosis of the spleen represents more than just another morphologic curiosity. The significance of peliosis lienalis lies in the potential of (1) overlooking the correct diagnosis as the cause of a spontaneous splenic rupture with intraabdominal hemorrhage, thus leading to the false conclusion of a violent death; and (2) misinterpreting the macromorphological appearance of this rare disease, also in the absence of splenic rupture, as a result of blunt force trauma.