Biopsy/methods , Immunoglobulin G4-Related Disease/diagnosis , Immunoglobulin G4-Related Disease/immunology , Spermatic Cord , Splenic Vein , Tomography, X-Ray Computed/methods , Anti-Inflammatory Agents/administration & dosage , Diagnosis, Differential , Humans , Immunoglobulin G4-Related Disease/drug therapy , Immunoglobulin G4-Related Disease/physiopathology , Male , Middle Aged , Mycophenolic Acid/administration & dosage , Phlebitis/diagnosis , Phlebitis/etiology , Phlebitis/immunology , Prednisone/administration & dosage , Radiographic Image Enhancement/methods , Radiography, Abdominal/methods , Spermatic Cord/diagnostic imaging , Spermatic Cord/pathology , Splenic Vein/diagnostic imaging , Splenic Vein/pathology , Treatment Outcome
Lupus Erythematosus, Systemic/diagnosis , Lupus Nephritis/pathology , Vasculitis/diagnostic imaging , Vasculitis/etiology , Cyclophosphamide/therapeutic use , Female , Humans , Intestines/diagnostic imaging , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/drug therapy , Middle Aged
Primary splenic angiosarcoma is an extremely agressive and rare neoplasm. Manifestations as bone marrow invasion and coagulation disorders have been reported isolatedly. A 26 years-old woman presented with abdominal pain; several anemia and thrombocytopenia associated to leukoerythroblastic reaction were found in the laboratory. Consumpion coagulopathy signs and microangiopathy as schistocytes, prolonged prothrombine time, decreased fibrinogen and increased D dimer were also present. Imaging findings included a lobulated, enlarged spleen, with spontanously hyperdense areas, and heterogeneous nodules with intense, irregular enhancement after contrast administration. There were hepatic and pulmonary metastases, as well as bone lesions with conspicuous vessels. Clinical features of Kasabach-Merrit syndrome and imaging vascular neoplasm characteristics suggest a primary splenic angiosarcoma. Splenectomy and bone marrow biopsy confirmed the diagnosis of primary splenic angiosarcoma in metastatic stage.
Anemia, Myelophthisic/etiology , Hemangiosarcoma/complications , Kasabach-Merritt Syndrome/etiology , Splenic Neoplasms/complications , Adult , Anemia, Myelophthisic/diagnosis , Biopsy , Female , Hemangiosarcoma/diagnosis , Hemangiosarcoma/surgery , Humans , Kasabach-Merritt Syndrome/diagnosis , Splenectomy , Splenic Neoplasms/diagnosis , Splenic Neoplasms/surgery