Wiskott‐Aldrich syndrome: a comprehensive review

MJ Massaad, N Ramesh… - Annals of the New York …, 2013 - Wiley Online Library
Wiskott‐Aldrich syndrome (WAS) is a rare X‐linked primary immunodeficiency characterized
by microthrombocytopenia, eczema, recurrent infections, and an increased incidence of …

Asplenic-hyposplenic overwhelming sepsis: postsplenectomy sepsis revisited

K Hansen, DB Singer - Pediatric and Developmental Pathology, 2001 - Springer
Absence of the spleen or splenic function predisposes individuals to risk of overwhelming
infection. These infections are most often due to encapsulated organisms, especially …

High-dose intravenous gammaglobulin for idiopathic thrombocytopenic purpura in childhood

P Imbach, V d'Apuzzo, A Hirt, E Rossi, M Vest… - The lancet, 1981 - Elsevier
Seven children with chronic or intermittent and six with acute idiopathic thrombocytopenic
purpura (ITP) were treated with large intravenous doses of polyvalent, intact immunoglobulin …

A multiinstitutional survey of the Wiskott-Aldrich syndrome

KE Sullivan, CA Mullen, RM Blaese… - The Journal of …, 1994 - Elsevier
Abstract The Wiskott-Aldrich syndrome is an X-linked primary immunodeficiency originally
characterized by the clinical triad of thrombocytopenia, eczema, and immunodeficiency. We …

X-linked thrombocytopenia (XLT) due to WAS mutations: clinical characteristics, long-term outcome, and treatment options

MH Albert, TC Bittner, S Nonoyama… - Blood, The Journal …, 2010 - ashpublications.org
A large proportion of patients with mutations in the Wiskott-Aldrich syndrome (WAS) protein
gene exhibit the milder phenotype termed X-linked thrombocytopenia (XLT). Whereas stem …

The Wiskott-Aldrich syndrome protein (WASP): roles in signaling and cytoskeletal organization

SB Snapper, FS Rosen - Annual review of immunology, 1999 - annualreviews.org
▪ Abstract The Wiskott-Aldrich Syndrome (WAS) is a rare X-linked primary immunodeficiency
that is characterized by recurrent infections, hematopoietic malignancies, eczema, and …

Actin cytoskeletal defects in immunodeficiency

DA Moulding, J Record, D Malinova… - Immunological …, 2013 - Wiley Online Library
The importance of the cytoskeleton in mounting a successful immune response is evident
from the wide range of defects that occur in actin‐related primary immunodeficiencies (PID …

Splenectomy and/or bone marrow transplantation in the management of the Wiskott-Aldrich syndrome: long-term follow-up of 62 cases

CA Mullen, KD Anderson, RM Blaese - 1993 - ashpublications.org
This study describes the effects of two major treatment options, splenectomy and/or bone
marrow transplantation, on the natural history of the Wiskott-Aldrich (WAS) syndrome. The …

The role of the spleen in resistance to infection.

JF Bohnsack, EJ Brown - Annual review of medicine, 1986 - europepmc.org
The spleen serves two major critical functions in protecting against bloodstream bacterial
infections--it is a phagocytic filter that removes bacteria from the bloodstream and it is an …

The Wiskott-Aldrich syndrome

HD Ochs - Springer seminars in immunopathology, 1998 - Springer
Wiskott, in 1937, described three brothers who presented shortly after birth with
thrombocytopenia, bloody diarrhea, eczema, and recurrent ear infections [104] …