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Systemic lupus erythematosus and myelofibrosis
The American Journal of Medicine
|November 1, 1986
Summary
Systemic lupus erythematosus (SLE) can cause acute thrombocytopenia and myelofibrosis. Corticosteroid treatment improved thrombocytopenia but not myelofibrosis in one patient with SLE.
Area of Science:
- Rheumatology
- Hematology
Background:
- Systemic lupus erythematosus (SLE) is a chronic autoimmune disease.
- Hematologic abnormalities are common in SLE patients.
Observation:
- A 27-year-old woman with SLE developed acute thrombocytopenia.
- She also had myelofibrosis, indicated by difficult bone marrow aspiration due to increased reticulin.
- Serologic findings included antibodies to red cells and granulocytes, increased platelet-associated IgG, low complement levels, circulating immune complexes, and high titers of antinuclear antibodies and anti-native DNA antibodies.
Findings:
- Corticosteroid therapy successfully reversed the thrombocytopenia.
- However, the myelofibrosis and serologic abnormalities persisted.
- Literature review identified four additional SLE patients with myelofibrosis, two of whom experienced regression of myelofibrosis with corticosteroid treatment.
Implications:
- This case highlights a rare association between SLE and myelofibrosis.
- It suggests that while corticosteroids can manage thrombocytopenia in SLE, their efficacy for myelofibrosis may be variable.
- Further research is needed to understand the pathogenesis and optimal treatment strategies for myelofibrosis in SLE patients.