Adult-onset Still's disease complicated with hemophagocytic syndrome.
Hemophagocytic syndrome (HS) that occurs in the course of adult-onset Stills disease (AOSD) has been reported only rarely in the literature. HS and AOSD share overlapping clinical and laboratory features, therefore, it is difficult to recognize HS as a complication of AOSD. Here, we report the case of a 46-year old woman with classical features of AOSD. Severe pancytopenia and jaundice associated with extreme hyperferritinemia occurred during high-dose steroid treatment. Bone marrow biopsy showed typical pathological features of hemophagocytosis, which confirmed the coexistence of HS with AOSD. The patient was treated with methylprednisolone pulse therapy of 500 mg/day for 3 days, as recommended in cases of HS complicating AOSD, and her condition improved gradually. During the disease course, extensive studies could not identify any viral infection or other known underlying etiology for the reactive hemophagocytosis. Currently, the patient is in remission on low-dose prednisolone and azathioprine.
Yeh
Horng-Ming
HM
Department of Internal Medicine, Tainan Municipal Hospital, Tainan, Taiwan.
Liu
Ming-Fei
MF
Chang
Kuo-Kuan
KK
Liu
Shian-Min
SM
Chen
Chang-Hung
CH
D001379:Azathioprine / Q000627:therapeutic use; D001853:Bone Marrow / Q000473:pathology; D004359:Drug Therapy, Combination; D005260:Female; D005334:Fever / Q000209:etiology; D005938:Glucocorticoids / Q000008:administration & dosage; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications* / Q000473:pathology; D008775:Methylprednisolone / Q000008:administration & dosage; D008875:Middle Aged; D020551:Pulse Therapy, Drug; D016706:Still's Disease, Adult-Onset / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D016896:Treatment Outcome
D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D001379:Azathioprine; D008775:Methylprednisolone