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-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 30
  • Basım Tarihi: 2014
  • Doi Numarası: 10.1007/s12288-012-0218-z
  • Dergi Adı: INDIAN JOURNAL OF HEMATOLOGY AND BLOOD TRANSFUSION
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Anahtar Kelimeler: Polycythemia vera, Myelofibrosis, Extramedullary hematopoiesis, Skin, Splenectomy, Heart, AGNOGENIC MYELOID METAPLASIA, EXTRA-MEDULLARY HEMATOPOIESIS, STEM-CELL TRANSPLANTATION, MYELOPROLIFERATIVE DISORDERS, CYTOGENETIC ABNORMALITIES, IDIOPATHIC MYELOFIBROSIS, SECONDARY MYELOFIBROSIS, MARROW TRANSPLANTATION, PULMONARY-HYPERTENSION, VERA
  • Gazi Üniversitesi Adresli: Evet

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.