Successful treatment with cyclosporine in a case of hemophagocytic syndrome manifesting as severe liver dysfunction.
Forms of hemophagocytic syndrome, which affects mainly children, vary from mild to very severe and often fatal. We describe an adult patient with hemophagocytic syndrome in whom severe liver dysfunction developed. The condition continued to deteriorate despite treatment with plasma exchange, high-dose gamma globulin, and corticosteroid therapy. Treatment with cyclosporine (2.3 mg/kg/day) dramatically improved the condition and normalized liver function. Cyclosporine reduced the serum levels of ferritin, interferon-tau, interleukin-6, and soluble interleukin-2 receptor. These findings suggest that hemophagocytic syndrome accompanied with severe liver dysfunction results from hypercytokinemia, and cyclosporine is useful in preventing a fatal outcome during the acute phase.
Omagari
K
K
Second Department of Internal Medicine, Nagasaki University School of Medicine, Japan.
Ashida
R
R
Oh-I
H
H
Minamino
Y
Y
Sasaki
O
O
Ozono
Y
Y
Maeda
T
T
Sadamori
N
N
Tomonaga
M
M
Kohno
S
S
D000328:Adult; D000410:Alanine Transaminase / Q000097:blood; D001663:Bilirubin / Q000097:blood; D001854:Bone Marrow Cells / Q000473:pathology; D016572:Cyclosporine / Q000627:therapeutic use*; D005260:Female; D005293:Ferritins / Q000097:blood; D015616:Histiocytosis, Non-Langerhans-Cell / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007371:Interferon-gamma / Q000097:blood; D015850:Interleukin-6 / Q000097:blood; D008099:Liver / Q000473:pathology; D008107:Liver Diseases / Q000188:drug therapy* / Q000473:pathology; D010976:Platelet Count; D015375:Receptors, Interleukin-2 / Q000097:blood
D007166:Immunosuppressive Agents; D015850:Interleukin-6; D015375:Receptors, Interleukin-2; D007371:Interferon-gamma; D016572:Cyclosporine; D005293:Ferritins; D000410:Alanine Transaminase; D001663:Bilirubin