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Konca Degertekin C, Ozkurt ZN, Akyürek N, et al. A Rare Presentation of Extramedullary Hematopoiesis in Post-polycythemic Myelofibrosis. Indian J Hematol Blood Transfus. 2012;30:12-6doi: 10.1007/s12288-012-0218-z.
Konca Degertekin, C., Ozkurt, Z. N., Akyürek, N., & Yağcı, M. (2014). A Rare Presentation of Extramedullary Hematopoiesis in Post-polycythemic Myelofibrosis. Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion, 3012-6. https://doi.org/10.1007/s12288-012-0218-z
Konca Degertekin, Ceyla, et al. "A Rare Presentation of Extramedullary Hematopoiesis in Post-polycythemic Myelofibrosis." Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion vol. 30 (2014): 12-6. doi: https://doi.org/10.1007/s12288-012-0218-z
Konca Degertekin C, Ozkurt ZN, Akyürek N, Yağcı M. A Rare Presentation of Extramedullary Hematopoiesis in Post-polycythemic Myelofibrosis. Indian J Hematol Blood Transfus. 2014 Sep;30:12-6. doi: 10.1007/s12288-012-0218-z. Epub 2012 Dec 07. PMID: 25332522; PMCID: PMC4192256.
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Indian J Hematol Blood Transfus. 2014 Sep;30:12-6. doi: 10.1007/s12288-012-0218-z. Epub 2012 Dec 07.

A Rare Presentation of Extramedullary Hematopoiesis in Post-polycythemic Myelofibrosis.

Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion

Ceyla Konca Degertekin, Zübeyde Nur Ozkurt, Nalan Akyürek, Münci Yağcı

Affiliations

  1. Department of Endocrinology and Metabolism, Gazi University Faculty of Medicine, Gazi Üniversitesi Hastanesi, 06100 Be?evler, Ankara, Turkey.
  2. Department of Hematology, Department of Internal Medicine, Gazi University Faculty of Medicine, Ankara, Turkey.
  3. Department of Pathology, Gazi University Faculty of Medicine, Ankara, Turkey.

PMID: 25332522 PMCID: PMC4192256 DOI: 10.1007/s12288-012-0218-z

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.

Keywords: Extramedullary hematopoiesis; Heart; Myelofibrosis; Polycythemia vera; Skin; Splenectomy

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