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The Acute Lupus Hemophagocytic Syndrome

Kit-fai Wong, MBBS; Pak-kwan Hui, MD; John K. C. Chan, MBBS; Yuk-wah Chan, MMed; and Shau-yin Ha, MBBS
[+] Article, Author, and Disclosure Information

Requests for Reprints: Kit-fai Wong, MBBS; Institute of Pathology, Queen Elizabeth Hospital, Kowloon, Hong Kong.

Current Author Addresses: Drs. Wong and J. Chan: Institute of Pathology, Queen Elizabeth Hospital, Kowloon, Hong Kong.

Drs. Hui and Y. Chan: Clinical Pathology Unit and Medical Unit A, Kwong Wah Hospital, Kowloon, Hong Kong.

Dr. Ha: Department of Pathology, Queen Mary Hospital, Hong Kong.

© 1991 American College of PhysiciansAmerican College of Physicians

Ann Intern Med. 1991;114(5):387-390. doi:10.7326/0003-4819-114-5-387
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Objective: To characterize an unusual mode of presentation of systemic lupus erythematosus: acute and severe pancytopenia related to reactive hemophagocytosis.

Design: Retrospective case series.

Setting: Two general community hospitals in Hong Kong.

Patients: Six patients presenting with a reactive hemophagocytic syndrome, identified over a 3.5 year period, diagnosed with systemic lupus erythematosus according to the criteria of the American Rheumatism Association.

Results: In addition to severe pancytopenia and marrow hemophagocytosis, other characteristic features were fever, hypocomplementemia, high antinuclear antibody titer, and cutaneous and visceral vasculitis. There was no evidence of an underlying infection. The pancytopenia responded dramatically to treatment with steroids.

Conclusion: Recognition of the acute lupus hemophagocytic syndrome and distinction from an infection-associated hemophagocytic syndrome is important because it responds well to steroid therapy. The evaluation of patients presenting with a hemophagocytic syndrome should include serologic tests for systemic lupus erythematosus.





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