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Successful treatment of early secondary myelofibrosis in SLE with IVIG

Department of Medicine 'B', Research Unit of Autoimmune Diseases, Tel Aviv University

Y. Levy

Department of Medicine 'B', Research Unit of Autoimmune Diseases, Tel Aviv University

Y. Bar-Dayan

Department of Medicine 'B', Research Unit of Autoimmune Diseases, Tel Aviv University

A. Afek

Pathology Institute, Sheba Medical Center, Tel Hashomer and the Sackler School of Medicine, Tel Aviv University

G. Zandman-Goddard

Department of Medicine 'B', Research Unit of Autoimmune Diseases, Tel Aviv University

Y. Skurnik

Department of Medicine 'B', Research Unit of Autoimmune Diseases, Tel Aviv University

F. Fabrrizzi

Clinical Trial Consultant, Lucca, Italy

Y. Shoenfeld

Department of Medicine 'B', Research Unit of Autoimmune Diseases, Tel Aviv University

Myelofibrosis has been reported as a rare cause of pancytopenia in patients with autoimmune diseases. We describe a 54 y old female patient who was admitted with severe anemia subsequently found to be due to marrow fibrosis. During the course of her hospitalization, relying both on her clinical symptoms as well as the results of a wide range of laboratory tests and diagnostic procedures, the diagnosis of systemic lupus erythematosus was established. The patient was treated with high dose steroids, but improvement of her clinical symptoms as well as normalization of her peripheral blood count were achieved only after high dose intravenous therapy with gamma globulin (IVIG) was instituted. Along with the improvement in the peripheral blood parameters normalization of the bone marrow architecture was recorded on a repeated bone marrow biopsy. IVIG therapy should be considered in extreme cases of bone marrow suppression in SLE.

Key Words: systemic lupus erythematosus • secondary myelofibrosis • intravenous immunoglobulin

Lupus, Vol. 6, No. 4, 408-411 (1997)
DOI: 10.1177/096120339700600412


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