ABSTRACT
Primary splenic angiosarcoma is a rare abnormality and has a bad prognosis. It has unknown pathogenesis. This abnormality is usually presented by splenic rupture. Surgery is the most promising treatm Surgery is the most accepted and accurate method for diagnosis and treatment. Surgery before rupture increases the life expectancy. A 65-year-old woman who presented to the emergency room with abdominal pain, abdominal distension, and anemia was found to have a splenic mass and massive ascites. After getting a hemorrhagic sample from the abdomen, the patient was operated with splenic rupture prediagnosis. The spleen material was reported as splenic angiosarcoma. The staging 18F-FDG-Positron Emission Tomography-Computed Tomography did not show any metastasis. Five months later, paclitaxel treatment was initiated upon liver and bone metastasis, and the treatment still continues. Splenic angiosarcoma has a place among splenic parenchymal lesions. The splenectomy material names the diagnosis. Pathologic examination of splenectomy material is revealed certain diagnosis.
ABSTRACT
BACKGROUND: The prognosis of hemophagocytic syndrome (HPS) in kidney transplant recipients is reported to be poor, however the optimal therapeutic approach is still unclear. PATIENTS AND METHODS: The clinical and follow-up data of the 4 patients with HPS (3 male, 1 female; age 39.7 +/- 11.3 years) among 368 kidney transplant recipients during a 5-year period were retrospectively analyzed. RESULTS: HPS developed 35-61 days in the post-transplant period. All 4 patients presented with fever. Hepatosplenomegaly and lymphadenopathy were observed only in the first patient. Laboratory tests revealed pancytopenia and hyperferritinemia in all patients, but elevated liver enzymes were observed in 3. Two patients had cytomegalovirus infection, and 1 had Epstein-Barr virus infection. Three patients died despite aggressive supportive therapy, however the fourth case survived after graft nephrectomy. CONCLUSION: HPS pathogenesis in kidney transplants appears to be related with the graft itself. Graft nephrectomy may be the preferable therapeutic approach for kidney transplant recipients with HPS resistant to standard supportive therapy.
