A Rare Presentation of Extramedullary Hematopoiesis in Post-polycythemic Myelofibrosis


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Degertekin C. K. , ÖZKURT Z. N. , AKYÜREK N. , Yagci M.

INDIAN JOURNAL OF HEMATOLOGY AND BLOOD TRANSFUSION, cilt.30, 2014 (SCI İndekslerine Giren Dergi) identifier identifier identifier

  • Cilt numarası: 30
  • Basım Tarihi: 2014
  • Doi Numarası: 10.1007/s12288-012-0218-z
  • Dergi Adı: INDIAN JOURNAL OF HEMATOLOGY AND BLOOD TRANSFUSION

Özet

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.