Successful treatment of early secondary myelofibrosis in SLE with IVIG

Author:

Aharon A.1,Levy Y.1,Bar-Dayan Y.1,Afek A.2,Zandman-Goddard G.1,Skurnik Y.1,Fabrrizzi F.3,Shoenfeld Y.1

Affiliation:

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

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

3. Clinical Trial Consultant, Lucca, Italy

Abstract

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.

Publisher

SAGE Publications

Subject

Rheumatology

Reference27 articles.

1. Shoenfeld Y., Schwartz RS Hematologic manifestations in SLE. In: Schur (ed). The Management of Systemic Lupus Erythematosus. Grune & Stratton: N.Y., 1983 , pp 123-127.

2. Autoimmune Myelofibrosis

3. Idiopathic myelofibrosis: A clinical study of 80 patients

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