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Systemic lupus erythematosus presenting as myelofibrosis
K Konstantopoulos1, E Terpos, H Prinolakis
1First Department of Internal Medicine, University of Athens School of Medicine, Greece.
Haematologia
|September 5, 1998
Summary
Systemic lupus erythematosus (SLE) rarely co-occurs with myelofibrosis. This case study shows steroids effectively treated both SLE-related thrombocytopenia and bone marrow fibrosis.
Area of Science:
- Rheumatology
- Hematology
- Pathology
Background:
- Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with diverse clinical manifestations.
- Myelofibrosis, a rare bone marrow disorder, is infrequently associated with SLE.
- Literature review indicates a scarcity of reported cases linking SLE and myelofibrosis.
Observation:
- A female patient presented with thrombocytopenia, a low platelet count.
- Diagnostic workup confirmed systemic lupus erythematosus (SLE).
- Bone marrow examination revealed increased reticulin, indicative of myelofibrosis.
Findings:
- The co-incidence of SLE and myelofibrosis was confirmed in this patient.
- Treatment with corticosteroids led to the reversal of thrombocytopenia.
- Steroid therapy also resolved the bone marrow fibrosis.
Implications:
- This case highlights a rare but treatable association between SLE and myelofibrosis.
- Corticosteroid therapy may be an effective treatment for myelofibrosis in SLE patients.
- Further research is warranted to understand the pathogenesis and optimal management of this rare co-occurrence.