真性红细胞增多症后骨髓纤维化中髓外造血的罕见表现
A Rare Presentation of Extramedullary Hematopoiesis in Post-polycythemic Myelofibrosis.
作者信息
Konca Degertekin Ceyla, Ozkurt Zübeyde Nur, Akyürek Nalan, Yağcı Münci
机构信息
Department of Endocrinology and Metabolism, Gazi University Faculty of Medicine, Gazi Üniversitesi Hastanesi, 06100 Beşevler, Ankara, Turkey.
Department of Hematology, Department of Internal Medicine, Gazi University Faculty of Medicine, Ankara, Turkey.
出版信息
Indian J Hematol Blood Transfus. 2014 Sep;30(Suppl 1):12-6. doi: 10.1007/s12288-012-0218-z. Epub 2012 Dec 7.
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.
真性红细胞增多症是一种骨髓克隆性增殖性疾病，在其自然病程中可能演变为骨髓纤维化。进展为骨髓纤维化通常是晚期并发症，临床上表现为难治性血细胞减少和髓外造血（EMH）。在真性红细胞增多症后骨髓纤维化过程中，EMH可发生于任何组织。然而，皮肤和心脏受累似乎非常罕见。我们报告一名56岁女性，患有难治性真性红细胞增多症后骨髓纤维化，脾切除术后在多个器官，尤其是皮肤和心脏发生了EMH。结合该病例，根据文献讨论了临床表现、治疗选择、EMH的预后意义及细胞遗传学的作用。
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