title: A Rare Presentation of Extramedullary Hematopoiesis in Post-polycythemic Myelofibrosis.
abstract: Polycythemia vera is a clonal proliferative disorder of the bone marrow that could possibly evolve into myelofibrosis in its natural course. Progression to myelofibrosis is usually a late stage complication and presents clinically with refractory cytopenias and extramedullary hematopoiesis (EMH). EMH can occur in any tissue during the course of post-polycythemic myelofibrosis. However, skin and cardiac involvements seems to be very rare. We present a 56-year-old woman with post-polycythemic myelofibrosis refractory to treatment, developing EMH after splenectomy in various organs, exceptionally the skin and the heart. Along with the case, the clinical presentations, treatment options, prognostic significance of EMH and the role of cytogenetics is discussed in the light of the literature.
authors.affiliation: Department of Endocrinology and Metabolism, Gazi University Faculty of Medicine, Gazi Üniversitesi Hastanesi, 06100 Beşevler, Ankara, Turkey.
authors.forename: Ceyla
authors.initials: C
authors.lastname: Konca Degertekin
authors.affiliation: Department of Hematology, Department of Internal Medicine, Gazi University Faculty of Medicine, Ankara, Turkey.
authors.forename: Zübeyde Nur
authors.initials: ZN
authors.lastname: Ozkurt
authors.affiliation: Department of Pathology, Gazi University Faculty of Medicine, Ankara, Turkey.
authors.forename: Nalan
authors.initials: N
authors.lastname: Akyürek
authors.affiliation: Department of Hematology, Department of Internal Medicine, Gazi University Faculty of Medicine, Ankara, Turkey.
authors.forename: Münci
authors.initials: M
authors.lastname: Yağcı
keywords: Extramedullary hematopoiesis; Heart; Myelofibrosis; Polycythemia vera; Skin; Splenectomy