Primary autoimmune myelofibrosis: Definition of a distinct clinicopathologic syndrome
Open Access
- 30 December 2002
- journal article
- research article
- Published by Wiley in American Journal of Hematology
- Vol. 72 (1), 8-12
- https://doi.org/10.1002/ajh.10258
Abstract
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. Am. J. Hematol. 72:8–12, 2003.This publication has 23 references indexed in Scilit:
- Immune disorders in agnogenic myeloid metaplasia: relations to myelofibrosisBritish Journal of Haematology, 2008
- Pathology of Autoimmune MyelofibrosisAmerican Journal of Clinical Pathology, 2001
- Myelofibrosis with Myeloid MetaplasiaThe New England Journal of Medicine, 2000
- Evans’ syndrome, myelofibrosis and systemic lupus erythematosus: role of procollagens in myelofibrosisPathology, 1995
- Autoimmune MyelofibrosisMedicine, 1994
- Myelodysplastic syndrome with increased marrow fibrosis: a distinct clinico‐pathological entityBritish Journal of Haematology, 1991
- Systemic tapes erythematosus and myelofibrosisClinical Rheumatology, 1989
- A distinct subtype of idiopathic myelofibrosis with bone marrow features mimicking hairy cell leukemia: Evidence of an autoimmune pathogenesisAmerican Journal of Hematology, 1987
- The Syndrome of Idiopathic MyelofibrosisMedicine, 1983
- Effect of splenectomy on teardrop-shaped erythrocytes in agnogenic myeloid metaplasiaArchives of Internal Medicine, 1977