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Malignant histiocytosis as a fatal complication of systemic lupus erythematosus
J M Grau1, J L Aguilar, M C Cid
1Department of Internal Medicine, Hospital Clínic i Provincial, Barcelona, Spain.
Insights
Systemic lupus erythematosus (SLE) can lead to rare complications. This case highlights malignant histiocytosis as a previously unreported SLE complication, identified post-mortem.
Area of Science:
- Rheumatology
- Hematology
- Pathology
Background:
- Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with diverse clinical manifestations.
- Renal involvement is common in SLE, significantly impacting patient prognosis.
- The patient had a 9-year history of SLE, primarily affecting the kidneys.
Observation:
- The patient experienced a complex clinical course over the past year, marked by infectious and noninfectious complications.
- Development of pancytopenia, fever, and markedly elevated lactate dehydrogenase levels preceded the patient's demise.
- Autopsy revealed findings consistent with malignant histiocytosis.
Findings:
- Bone marrow examination via light and electron microscopy, alongside immunophenotyping, confirmed malignant histiocytosis.
- Malignant histiocytosis was identified as a complication of systemic lupus erythematosus.
- This represents the first reported instance of malignant histiocytosis occurring in the context of SLE.
Implications:
- This case expands the spectrum of known SLE complications.
- Highlights the importance of considering rare hematologic malignancies in SLE patients with severe constitutional symptoms.
- Suggests potential need for increased vigilance and diagnostic exploration for histiocytic disorders in SLE management.
Abstract:
We present the case of a 26-year-old Caucasian woman with systemic lupus erythematosus (SLE) of 9 years duration, mainly involving the kidney. Her clinical course was complicated in the last year by several infectious and noninfectious events, and after pancytopenia, fever, and extremely high levels of lactate dehydrogenase developed, the woman died. Light and electron microscopy of bone marrow, together with immunophenotyping, disclosed malignant histiocytosis, a complication of SLE that has not been reported.