Spontaneous splenic rupture due to Peliosis and the association with malignancy: A case series and literature review

INTRODUCTION AND IMPORTANCE: 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. CASE PRESENTATION: 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. CLINICAL DISCUSSION: 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. CONCLUSION: Based on our clinical experience and focused literature review, it appears likely that there is a relationship between splenic peliosis and hematologic malignancy.