Splenectomy resolved cytopenias and coagulopathy in a 53-year-old woman with primary splenic angiosarcoma presenting as disseminated intravascular coagulation and heart failure symptoms.
Case Report (n=1)
This case highlights that primary splenic angiosarcoma can present with DIC and cardiopulmonary symptoms mimicking heart failure, and splenectomy can serve as both a diagnostic and temporizing intervention.
Abstract Introduction Primary splenic angiosarcoma (PSA) is an exceptionally rare vascular malignancy originating from splenic sinusoidal endothelium.1 It carries a median survival under 12 months due to early metastasis and diagnostic.2 Clinical presentation is nonspecific often mimicking hematologic or infectious disorders and disseminated intravascular coagulation (DIC) as the initial manifestation is particularly uncommon.3 We present a diagnostically complex case of PSA manifesting as refractory cytopenias, DIC, and decompensated heart failure. Case Description A 53-year-old woman with HFpEF presented with one week of worsening exertional dyspnea. Initial evaluation revealed hemoglobin 6.8 g/dL, platelets 42 x 109/L, LDH 653 U/L, total bilirubin 1.7 mg/dL (indirect 1.1), and markedly elevated D-dimer 72,000 ng/mL. Coagulation profile met ISTH criteria for overt DIC (score 5). The direct antiglobulin test was negative, and peripheral smear showed no schistocytes. CT abdomen/pelvis demonstrated marked splenomegaly (16.8 cm) with heterogeneous enhancement but no focal lesion or lymphadenopathy. Extensive hematologic, infectious, and rheumatologic evaluations, including bone marrow biopsy, were unrevealing. Aggressive transfusion support temporarily alleviated the dyspnea, however, the patient’s cytopenias persisted. Given progressive splenomegaly and transfusion-refractory DIC, she was transferred to a tertiary center where splenectomy was performed. Histopathology revealed atypical endothelial proliferation with necrosis and numerous mitoses. Tumor cells expressed ERG and CD31, confirming endothelial differentiation, and lacked SOX10 and cytokeratin, supporting the diagnosis of primary splenic angiosarcoma. Post-splenectomy, her cytopenias and coagulopathy improved. She was readmitted 10 days later with a sterile exudative right pleural effusion, likely reactive, and remains clinically stable under oncologic follow-up. Discussion This case highlights the diagnostic complexity of PSA presenting with DIC and cardiopulmonary symptoms. The pathophysiology of DIC in PSA is linked to tumor-driven endothelial activation and release of procoagulant microparticles.3,4 Early imaging findings, which are limited to splenomegaly, may obscure recognition.5 Splenectomy remains both diagnostic and temporizing, often improving hematologic derangements, though long-term prognosis remains poor (median survival 1 year). Pulmonary manifestations, including dyspnea and effusions, underscore how PSA can mimic heart failure or pulmonary disease, emphasizing the need for multidisciplinary collaboration between hematology, pulmonology, and surgery. Early recognition in patients with unexplained cytopenias and DIC may facilitate curative intervention and improve outcomes. References: 1. Neuhauser TS et al. Am J Surg Pathol. 2000;24(6):882-895. 2. Falk S et al. Cancer. 1993;71(4):1425-1437. 3. Hu Q et al. Front Oncol. 2022;12:832102. 4. Kong Y et al. BMC Cancer. 2020;20:989. 5. Agaimy A et al. Virchows Arch. 2019;475(1):3-21. This abstract is funded by: None
Anand et al. (Fri,) conducted a case report in Primary splenic angiosarcoma with disseminated intravascular coagulation (n=1). Splenectomy was evaluated. Splenectomy resolved cytopenias and coagulopathy in a 53-year-old woman with primary splenic angiosarcoma presenting as disseminated intravascular coagulation and heart failure symptoms.