共 59 条
Clinical spectrum, pathophysiology and treatment of the Wiskott-Aldrich syndrome
被引:63
作者:

Albert, Michael H.
论文数: 0 引用数: 0
h-index: 0
机构:
Univ Munich, Dept Pediat Hematol Oncol, Dr von Haunersches Kinderspital, Munich, Germany Univ Munich, Dept Pediat Hematol Oncol, Dr von Haunersches Kinderspital, Munich, Germany

Notarangelo, Luigi D.
论文数: 0 引用数: 0
h-index: 0
机构:
Harvard Univ, Sch Med, Manton Ctr Orphan Dis Res, Boston, MA USA
Harvard Univ, Childrens Hosp, Sch Med, Div Immunol, Boston, MA 02115 USA Univ Munich, Dept Pediat Hematol Oncol, Dr von Haunersches Kinderspital, Munich, Germany

Ochs, Hans D.
论文数: 0 引用数: 0
h-index: 0
机构:
Univ Washington, Dept Pediat, Seattle, WA 98195 USA
Ctr Immun & Immunotherapy, Seattle Childrens Res Inst, Seattle, WA USA Univ Munich, Dept Pediat Hematol Oncol, Dr von Haunersches Kinderspital, Munich, Germany
机构:
[1] Univ Munich, Dept Pediat Hematol Oncol, Dr von Haunersches Kinderspital, Munich, Germany
[2] Harvard Univ, Sch Med, Manton Ctr Orphan Dis Res, Boston, MA USA
[3] Harvard Univ, Childrens Hosp, Sch Med, Div Immunol, Boston, MA 02115 USA
[4] Univ Washington, Dept Pediat, Seattle, WA 98195 USA
[5] Ctr Immun & Immunotherapy, Seattle Childrens Res Inst, Seattle, WA USA
基金:
美国国家卫生研究院;
关键词:
gene therapy;
hematopoietic stem cell transplantation;
WAS gene;
Wiskott-Aldrich syndrome;
X-linked neutropenia;
X-linked thrombocytopenia;
STEM-CELL TRANSPLANTATION;
BONE-MARROW-TRANSPLANTATION;
SYNDROME PROTEIN-DEFICIENCY;
X-LINKED THROMBOCYTOPENIA;
ACTIN POLYMERIZATION;
GENE-THERAPY;
T-CELLS;
B-CELLS;
WASP;
MUTATIONS;
D O I:
10.1097/MOH.0b013e32834114bc
中图分类号:
R5 [内科学];
学科分类号:
1002 ;
100201 ;
摘要:
Purpose of review The Wiskott-Aldrich syndrome (WAS), caused by mutations in the WAS gene, is a complex and diverse disorder with X-linked inheritance. This review focuses on recent developments in the understanding of its basic pathophysiology, diverse clinical phenotypes and optimal patient management including novel therapies. Recent findings The protein encoded by the WAS gene is a multifunctional signaling element expressed in immune and hematopoietic cells that plays a critical role in cytoskeletal reorganization, immune synapse formation and intracellular signaling. The type of specific mutation, its location within the gene and its effect on protein expression play a major role in determining an individual patient's clinical phenotype. Recent clinical observations and molecular studies have created a sophisticated picture of the disease spectrum. The improved outcome of stem cell transplantation from related and unrelated matched donors and promising early results from the first clinical gene therapy trial have added new therapeutic options for these patients. Summary Classic WAS, X-linked thrombocytopenia and X-linked neutropenia are caused by WAS gene mutations, each having a distinct pattern of clinical symptoms and disease severity. New developments in the understanding of these syndromes and novel therapeutic options will have a major impact on the treatment of individuals with WAS mutations.
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页码:42 / 48
页数:7
相关论文
共 59 条
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LMU, Dr Von Haunerschen Kinderspital, D-80337 Munich, Germany LMU, Dr Von Haunerschen Kinderspital, D-80337 Munich, Germany

Nonoyama, Shigeaki
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Natl Def Med Coll, Tokorozawa, Saitama, Japan LMU, Dr Von Haunerschen Kinderspital, D-80337 Munich, Germany

Notarangelo, Lucia Dora
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Univ Brescia, Brescia, Italy LMU, Dr Von Haunerschen Kinderspital, D-80337 Munich, Germany

Burns, Siobhan
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UCL, Inst Child Hlth, London, England LMU, Dr Von Haunerschen Kinderspital, D-80337 Munich, Germany

Imai, Kohsuke
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Natl Def Med Coll, Tokorozawa, Saitama, Japan LMU, Dr Von Haunerschen Kinderspital, D-80337 Munich, Germany

Espanol, Teresa
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Vall Hebron Hosp, Barcelona, Spain LMU, Dr Von Haunerschen Kinderspital, D-80337 Munich, Germany

Fasth, Anders
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Queen Silvia Childrens Hosp, Gothenburg, Sweden LMU, Dr Von Haunerschen Kinderspital, D-80337 Munich, Germany

Pellier, Isabelle
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CHU Angers, Angers, France LMU, Dr Von Haunerschen Kinderspital, D-80337 Munich, Germany

Strauss, Gabriele
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Otto Heubner Zentrum Kinder & Jugendmed, Charite Campus Virchow Klinikum, Berlin, Germany LMU, Dr Von Haunerschen Kinderspital, D-80337 Munich, Germany

Morio, Tomohiro
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Tokyo Med & Dent Univ, Tokyo, Japan LMU, Dr Von Haunerschen Kinderspital, D-80337 Munich, Germany

Gathmann, Benjamin
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Univ Freiburg Klinikum, Freiburg, Germany LMU, Dr Von Haunerschen Kinderspital, D-80337 Munich, Germany

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St Radboud Univ Nijmegen, Med Ctr, Nijmegen, Netherlands LMU, Dr Von Haunerschen Kinderspital, D-80337 Munich, Germany

Fillat, Cristina
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Ctr Invest Biomed Red Enfermedades Raras, Ctr Regulacio Genom, Barcelona, Spain LMU, Dr Von Haunerschen Kinderspital, D-80337 Munich, Germany

Hoenig, Manfred
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Univ Klin Kinder & Jugendmed Ulm, Ulm, Germany LMU, Dr Von Haunerschen Kinderspital, D-80337 Munich, Germany

Nathrath, Michaela
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Tech Univ Munich, Univ Childrens Hosp, Munich, Germany LMU, Dr Von Haunerschen Kinderspital, D-80337 Munich, Germany

Meindl, Alfons
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Tech Univ Munich, Frauenklin, Klinikum Rechts Isar, Munich, Germany LMU, Dr Von Haunerschen Kinderspital, D-80337 Munich, Germany

Pagel, Philipp
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Tech Univ, Lehrstuhl Genomorientierte Bioinformat, Wissensch Zentrum Weihenstephan, Freising Weihenstephan, Germany LMU, Dr Von Haunerschen Kinderspital, D-80337 Munich, Germany

Wintergerst, Uwe
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Hop Necker Enfants Malad, Paris, France LMU, Dr Von Haunerschen Kinderspital, D-80337 Munich, Germany

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UCL, Inst Child Hlth, London, England LMU, Dr Von Haunerschen Kinderspital, D-80337 Munich, Germany

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LMU, Dr Von Haunerschen Kinderspital, D-80337 Munich, Germany LMU, Dr Von Haunerschen Kinderspital, D-80337 Munich, Germany

Ochs, Hans D.
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Univ Washington, Seattle Childrens Hosp, Seattle, WA 98195 USA LMU, Dr Von Haunerschen Kinderspital, D-80337 Munich, Germany
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Univ Hosp Leuven, Ctr Human Genet, Dept Haematol, B-3000 Louvain, Belgium
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Bertone, Marko
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机构: Childrens Hosp, Div Immunol, Boston, MA 02215 USA

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机构: Childrens Hosp, Div Immunol, Boston, MA 02215 USA

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机构: STANFORD UNIV, MED CTR, DEPT GENET, STANFORD, CA 94305 USA

OCHS, HD
论文数: 0 引用数: 0
h-index: 0
机构: STANFORD UNIV, MED CTR, DEPT GENET, STANFORD, CA 94305 USA

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机构: STANFORD UNIV, MED CTR, DEPT GENET, STANFORD, CA 94305 USA
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Dupré, L
论文数: 0 引用数: 0
h-index: 0
机构: San Raffaele Telethon Inst Gene Therapy, HSR TIGET, I-20132 Milan, Italy

Aiuti, A
论文数: 0 引用数: 0
h-index: 0
机构: San Raffaele Telethon Inst Gene Therapy, HSR TIGET, I-20132 Milan, Italy

Trifari, S
论文数: 0 引用数: 0
h-index: 0
机构: San Raffaele Telethon Inst Gene Therapy, HSR TIGET, I-20132 Milan, Italy

Martino, S
论文数: 0 引用数: 0
h-index: 0
机构: San Raffaele Telethon Inst Gene Therapy, HSR TIGET, I-20132 Milan, Italy

Saracco, P
论文数: 0 引用数: 0
h-index: 0
机构: San Raffaele Telethon Inst Gene Therapy, HSR TIGET, I-20132 Milan, Italy

Bordignon, C
论文数: 0 引用数: 0
h-index: 0
机构: San Raffaele Telethon Inst Gene Therapy, HSR TIGET, I-20132 Milan, Italy

Roncarolo, MG
论文数: 0 引用数: 0
h-index: 0
机构: San Raffaele Telethon Inst Gene Therapy, HSR TIGET, I-20132 Milan, Italy