The acute lupus hemophagocytic syndrome.
OBJECTIVE
To characterize an unusual mode of presentation of systemic lupus erythematosus: acute and severe pancytopenia related to reactive hemophagocytosis.


METHODS
Retrospective case series.


METHODS
Two general community hospitals in Hong Kong.


METHODS
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.


CONCLUSIONS
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.
Wong
K F
KF
Institute of Pathology, Queen Elizabeth Hospital, Kowloon, Hong Kong.
Hui
P K
PK
Chan
J K
JK
Chan
Y W
YW
Ha
S Y
SY
D000208:Acute Disease; D000328:Adult; D001773:Blood Cells / Q000276:immunology; D001853:Bone Marrow / Q000473:pathology; D002648:Child; D005260:Female; D006644:Histiocytes / Q000276:immunology / Q000473:pathology; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000276:immunology / Q000473:pathology; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D008875:Middle Aged; D010198:Pancytopenia / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology; D010587:Phagocytosis / Q000276:immunology; D011241:Prednisone / Q000627:therapeutic use; D012189:Retrospective Studies
D011241:Prednisone; D008775:Methylprednisolone