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Sarcoidosis presenting as massive splenomegaly and severe epistaxis, case report

Sarcoidosis is a multisystem disorder of unknown etiology. Extrapulmonary sarcoidosis can involve any organ, but isolated spleen involvement is rare. Diagnosis can be challenging as other etiologies may have similar presentations. A 58-year-old African American female presented with life threatening...

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Detalles Bibliográficos
Autores principales: Stoelting, Austen, Esperti, Shawn, Balanchivadze, Nino, Piacentino, Valentino, Mangano, Andrew
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7160383/
https://www.ncbi.nlm.nih.gov/pubmed/32322388
http://dx.doi.org/10.1016/j.amsu.2020.03.007
Descripción
Sumario:Sarcoidosis is a multisystem disorder of unknown etiology. Extrapulmonary sarcoidosis can involve any organ, but isolated spleen involvement is rare. Diagnosis can be challenging as other etiologies may have similar presentations. A 58-year-old African American female presented with life threatening epistaxis, anemia, refractory thrombocytopenia, and massive splenomegaly. Lymphoproliferative, infectious, and autoimmune etiologies were eliminated with laboratory testing and bone marrow biopsy. The patient had multiple splenic artery aneurysms precluding an open diagnostic splenectomy. Partial splenic artery embolization was performed, which normalized the platelet count and resolved the spontaneous bleeding. This allowed diagnostic splenectomy and splenic artery repair to be safely performed. Surgical pathology demonstrated extensive non-caseating granulomas consistent with sarcoidosis. We present this case to demonstrate the omnipotent nature of sarcoidosis and a complex multi-disciplinary approach for successful diagnosis and treatment.