Primary autoimmune myelofibrosis: definition of a distinct clinicopathologic syndrome.

Published

Journal Article

Myelofibrosis is characterized by reticulin fibrosis of the bone marrow with resulting features of myelophthisis. Besides hematopoietic malignancies and other neoplasms involving the bone marrow, myelofibrosis has been described in association with autoimmune disorders, especially systemic lupus erythematosus. We describe the clinicopathologic features of a primary form of autoimmune myelofibrosis (AIMF) in patients who do not have systemic lupus erythematosus or another well-defined autoimmune syndrome. Absence of marked splenomegaly, peripheral blood cytopenias with mild teardrop poikilocytosis and leukoerythroblastosis, bone marrow lymphoid aggregates, and presence of autoantibodies are some of the salient features of primary AIMF. AIMF should especially be differentiated from chronic idiopathic myelofibrosis, a neoplastic myeloproliferative disease. Primary AIMF appears to have an excellent prognosis, with all patients reported in this series responding to a short course of corticosteroid therapy.

Full Text

Cited Authors

  • Pullarkat, V; Bass, RD; Gong, JZ; Feinstein, DI; Brynes, RK

Published Date

  • January 2003

Published In

Volume / Issue

  • 72 / 1

Start / End Page

  • 8 - 12

PubMed ID

  • 12508261

Pubmed Central ID

  • 12508261

Electronic International Standard Serial Number (EISSN)

  • 1096-8652

International Standard Serial Number (ISSN)

  • 0361-8609

Digital Object Identifier (DOI)

  • 10.1002/ajh.10258

Language

  • eng