|
|
 Previous Article | Table of Contents | Next Article 
Elevation of the Serum Fas Ligand in Patients With Hemophagocytic
Syndrome and Diamond-Blackfan Anemia
Daiichiro Hasegawa,
Seiji Kojima,
Eiji Tatsumi,
Akira Hayakawa,
Yoshiyuki Kosaka,
Hajime Nakamura,
Masahiro Sako,
Yuko Osugi,
Shigekazu Nagata, and
Kimihiko Sano
From the Departments of Pediatrics and of Tumor Genetics, Kobe
University School of Medicine, Kobe, Japan; the Division of Hematology
and Oncology, Children's Medical Center, Japanese Red Cross Nagoya
First Hospital, Nagoya, Japan; the Department of Pediatrics, Osaka City
General Hospital, Osaka, Japan; the Department of Pediatrics, Osaka
University Hospital, Osaka, Japan; and the Department of Genetics,
Osaka University Medical School, Osaka, Japan.
Fas ligand (FasL) is a membrane protein that is expressed in
activated T cells and natural killer cells. FasL binds to Fas on target
cells and induces apoptosis. There exists a soluble form of FasL
(sFasL), and sFasL also induces apoptosis of Fas-bearing cells. The
serum sFasL concentrations were reported to be elevated in patients
with large granular lymphocytic leukemia and natural killer cell
lymphoma. In this study, we have measured serum sFasL concentrations in
other hematological disorders, including severe aplastic anemia (SAA),
hemophagocytic lymphohistiocytosis (HLH), and Diamond-Blackfan anemia
(DBA). The serum sFasL concentration of age-matched healthy controls
was 0.16 ± 0.11 ng/mL (mean ± SD, n = 22). The serum sFasL levels
in the patients with HLH and DBA were 3.75 ± 3.82 (n = 19;
P < .0001, HLH v control) and 2.76 ± 2.43 ng/mL (n
= 6; P = .012, DBA v control), respectively. Serum interferon- concentration was elevated in the patients with HLH (1.61 ± 2.62 ng/mL) but not in those with DBA (below the detectable level). These results suggest that the Fas-FasL system plays a role, at
least in part, in the pathophysiology of HLH and DBA.
Blood, Vol. 91 No. 8 (April 15), 1998:
pp. 2793-2799
© 1998 by The American Society of Hematology.
 CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati What's this?
This article has been cited by other articles:
|
|
|
|
 
A. D. Billiau, T. Roskams, R. Van Damme-Lombaerts, P. Matthys, and C. Wouters
Macrophage activation syndrome: characteristic findings on liver biopsy illustrating the key role of activated, IFN-{gamma}-producing lymphocytes and IL-6- and TNF-{alpha}-producing macrophages
Blood,
February 15, 2005;
105(4):
1648 - 1651.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
L. M. Blanco-Colio, J. L. Martin-Ventura, J. M. Sol, C. Diaz, G. Hernandez, and J. Egido
Decreased circulating Fas ligand in patients with familial combined hyperlipidemia or carotid atherosclerosis: Normalization by atorvastatin
J. Am. Coll. Cardiol.,
April 7, 2004;
43(7):
1188 - 1194.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
E. M. Schneider, I. Lorenz, M. Muller-Rosenberger, G. Steinbach, M. Kron, and G. E. Janka-Schaub
Hemophagocytic lymphohistiocytosis is associated with deficiencies of cellular cytolysis but normal expression of transcripts relevant to killer-cell-induced apoptosis
Blood,
September 26, 2002;
100(8):
2891 - 2898.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
Y. Dror and M. H. Freedman
Shwachman-Diamond syndrome marrow cells show abnormally increased apoptosis mediated through the Fas pathway
Blood,
May 15, 2001;
97(10):
3011 - 3016.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
G. Matute-Bello, R. K. Winn, M. Jonas, E. Y. Chi, T. R. Martin, and W. C. Liles
Fas (CD95) Induces Alveolar Epithelial Cell Apoptosis in Vivo : Implications for Acute Pulmonary Inflammation
Am. J. Pathol.,
January 1, 2001;
158(1):
153 - 161.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. Jiang, M.-W. H. Wu, P. Sternberg, and D. P. Jones
Fas Mediates Apoptosis and Oxidant-Induced Cell Death in Cultured hRPE Cells
Invest. Ophthalmol. Vis. Sci.,
March 1, 2000;
41(3):
645 - 655.
[Abstract]
[Full Text]
|
|
|
|
|
|
|
K.-i. Seino, K. Iwabuchi, N. Kayagaki, R. Miyata, I. Nagaoka, A. Matsuzawa, K. Fukao, H. Yagita, and K. Okumura
Cutting Edge: Chemotactic Activity of Soluble Fas Ligand Against Phagocytes
J. Immunol.,
November 1, 1998;
161(9):
4484 - 4488.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
