J. Sibilia et al., PANCYTOPENIA SECONDARY TO HEMOPHAGOCYTIC SYNDROME IN RHEUMATOID-ARTHRITIS TREATED WITH METHOTREXATE AND SULFASALAZINE, Journal of rheumatology, 25(6), 1998, pp. 1218-1220
Hemophagocytic syndrome is an exceptional cause of pancytopenia, Its e
tiologies are most commonly viral or bacterial infections, lymphoproli
ferative syndromes, acquired or congenital immunodeficiencies. systemi
c diseases, or immunomodulatory treatment. We describe a patient with
rheumatoid arthritis (RA) treated with methotrexate (MTX), sulfasalazi
ne, and low dose corticosteroids, whose case was seriously complicated
by the occurrence of acute febrile pancytopenia. The pancytopenia app
eared secondary to hemophagocytic syndrome triggered by Escherichia co
li septicemia. The evolution was marked by severe aggravation of RA, p
robably due to release of cytokines from macrophages (tumor necrosis f
actor-alpha, interleukin 6). Reintroduction of MTX (without sulfasalaz
ine) resulted in partial remission and there was no reappearance of ne
w hematological anomalies after 16 month followup. A knowledge of this
syndrome is particularly important, since it mimics drug toxicity and
other complications such as lymphoproliferative diseases.