Journal List > J Korean Rheum Assoc > v.14(2) > 1003555

Kim, Park, Kim, Kim, Park, Shin, Kim, and Choe: Two Cases of Adult Onset Still's Disease with Concomitant Hemophagocytic Syndrome

Abstract

Adult onset Still's disease is an rare inflammatory disease with the characteristic of fever, skin rash, arthralgia or arthritis, lymphadenopathy, leukocytosis and multiple systemic organ involvement. Its accurate pathogenesis has not been elucidated yet. Its clinical manifestation is also very diverse, from relatively mild symptoms to severe complications such as concomitant infection, liver failure, disseminated intravascular coagulation, myocarditis, adult respiratory distress syndrome, which may lead to death in some cases. Particularly, concomitant hemophagocytic syndrome is rare complication that could induce a fatal outcome. Thus it is important to diagnose early and start treatments. Until now, it has been reported in only one case of adult onset Still's disease in Korea. Here, we report two female cases of adult onset Still's disease with concomitant hemophagocytic syndrome.

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Fig. 1.
Faint salmon-colored rashes on the neck, trunk (A), and face (B).
jkra-14-160f1.tif
Fig. 2.
Bone marrow aspiration reveals histiocytes phagocytizing red blood cells and neutrophils (χ 400).
jkra-14-160f2.tif
Fig. 3.
Atypical lymphocytes in peripheral blood smear (∗400) (A) and phagocytizing histiocyte (arrow) in bone marrow aspirates (x400) (B).
jkra-14-160f3.tif
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