A Rare Presentation of Extramedullary Hematopoiesis in Post-polycythemic Myelofibrosis

被引：0
作者：
Ceyla Konca Degertekin
Zübeyde Nur Özkurt
Nalan Akyürek
Münci Yağcı
机构：
[1] Gazi University Faculty of Medicine,Department of Endocrinology and Metabolism
[2] Gazi University Faculty of Medicine,Department of Hematology, Department of Internal Medicine
[3] Gazi University Faculty of Medicine,Department of Pathology
来源：
Indian Journal of Hematology and Blood Transfusion | 2014年 / 30卷
关键词：
Polycythemia vera; Myelofibrosis; Extramedullary hematopoiesis; Skin; Splenectomy; Heart;
D O I：
暂无
中图分类号：
学科分类号：
摘要：
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.
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页码：12 / 16
页数：4
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