Myelofibrosis with myeloid metaplasia

Giovanni Barosi

Research output: Contribution to journalArticle

25 Citations (Scopus)

Abstract

MMM is a chronic myeloproliferative disorder characterized by bone marrow fibrosis and neoangiogenesis, constitutive release of a high number of CD34+ stem cells from the bone marrow, and extramedullary hematopoiesis. It presents with heterogeneous clinical features in which anemia and progression to symptomatic splenomegaly dominate. The pathogenesis is undefined, but the dual action of deregulation of the bFGF pathway may influence myeloproliferation, myelofibrosis, and neoangiogenesis. Animal models suggest that chronic exposure to high doses of thrombopoietin or impairment of the capacity of megakaryocytes to differentiate into platelets, as occurs in the GATA-Ilow mice, is a necessary event for myelofibrosis. Allogeneic stem cell transplantation offers a chance of cure, and low conditioning regimens may extend the age of transplantable patients with lower mortality. Autologus stem cell transplantation and splenectomy are risky procedures that may be considered in patients with advanced disease when conventional therapies for correcting anemia (danazol, recombinant human erythropoietin, or cyclosporine) or chemotherapy for splenomegaly and myeloproliferation (hydroxyurea or interferon alfa) have failed. Thalidomide has been tested in numerous series, and its capacity to improve anemia and thrombocytopenia while reducing splenomegaly has been documented.

Original languageEnglish
Pages (from-to)1211-1226
Number of pages16
JournalHematology/Oncology Clinics of North America
Volume17
Issue number5
DOIs
Publication statusPublished - Oct 2003

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Primary Myelofibrosis
Splenomegaly
Anemia
Stem Cell Transplantation
Extramedullary Hematopoiesis
Danazol
Thrombopoietin
Myeloproliferative Disorders
Thalidomide
Megakaryocytes
Hydroxyurea
Splenectomy
Erythropoietin
Interferon-alpha
Thrombocytopenia
Cyclosporine
Stem Cells
Blood Platelets
Animal Models
Bone Marrow

ASJC Scopus subject areas

  • Hematology
  • Oncology

Cite this

Myelofibrosis with myeloid metaplasia. / Barosi, Giovanni.

In: Hematology/Oncology Clinics of North America, Vol. 17, No. 5, 10.2003, p. 1211-1226.

Research output: Contribution to journalArticle

Barosi, Giovanni. / Myelofibrosis with myeloid metaplasia. In: Hematology/Oncology Clinics of North America. 2003 ; Vol. 17, No. 5. pp. 1211-1226.
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