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Previous Volume 342:1255-1265 April 27, 2000 Number 17 Next

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Myelofibrosis with myeloid metaplasia is classified broadly as a chronic myeloid disorder and, more specifically, as a chronic myeloproliferative disease¹ (Figure 1). The primary pathogenetic mechanism is a clonal stem-cell disorder that leads to ineffective erythropoiesis, dysplastic-megakaryocyte hyperplasia, and an increase in the ratio of immature granulocytes to total granulocytes. This clonal myeloproliferation is characteristically accompanied by reactive myelofibrosis (bone marrow fibrosis) and by extramedullary hematopoiesis in the spleen or in multiple organs. The diagnosis is often suspected when teardrop-shaped red cells and myeloid precursors are detected in the peripheral blood. The typical clinical features include marked splenomegaly, . . . [Full Text of this Article]

Terminology and Classification

Pathogenetic Mechanisms

Diagnosis

Differential Diagnosis

Clinical Features

Epidemiology

Clinical Presentation

Complications

Prognosis

Management

Drug Therapy

Splenectomy

Splenic Irradiation

Allogeneic Stem-Cell Transplantation

Autologous Stem-Cell Transplantation

Antifibrotic and Antiangiogenic Therapy

Conclusions

Source Information

From the Division of Hematology and Internal Medicine, Mayo Clinic and Mayo Foundation, Rochester, Minn.

Address reprint requests to Dr. Tefferi at the Mayo Clinic, 200 First St. SW, Rochester, MN 55905, or at tefferi.ayalew@mayo.edu.

References

Related Letters:

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