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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.

Arthritis and Rheumatism
|December 1, 1991
PubMed

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.

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