How I manage patients with Wiskott Aldrich syndrome Article Swipe

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Elizabeth Rivers , Austen Worth , Adrian J. Thrasher , Siobhan O. Burns ·

YOU? · · 2019 · Open Access · · DOI: https://doi.org/10.1111/bjh.15831

Summary Wiskott Aldrich syndrome (WAS) is a primary immunodeficiency disease resulting in recurrent infections, eczema and microthrombocytopaenia. In its classical form, significant combined immune deficiency, autoimmune complications and risk of haematological malignancy necessitate early correction with stem cell transplantation or gene therapy. A milder form, X‐linked thrombocytopaenia (XLT), shares similar bleeding risk from thrombocytopaenia but is not associated with other significant clinical features and is generally managed conservatively. Here, we detail our approach to the diagnosis and treatment of classical WAS and XLT.

Related Topics

Wiskott–Aldrich Syndrome

Medicine

Malignancy

Disease

Hematopoietic Stem Cell Transplantation

Concepts

Wiskott–Aldrich syndrome Medicine Immunodeficiency Primary immunodeficiency Malignancy Wiskott–Aldrich syndrome protein Disease Hematopoietic stem cell transplantation Immunopathology Transplantation Immune system Immunology Dermatology Internal medicine Cell Gene Biology Actin cytoskeleton Cytoskeleton Genetics Biochemistry

Metadata

Type: review
Language: en
Landing Page: https://doi.org/10.1111/bjh.15831
PDF: https://onlinelibrary.wiley.com/doi/pdfdirect/10.1111/bjh.15831
OA Status: bronze
Cited By: 56
References: 48
Related Works: 10
OpenAlex ID: https://openalex.org/W2921913141

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OpenAlex ID: https://openalex.org/W2921913141

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DOI: https://doi.org/10.1111/bjh.15831

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Title: How I manage patients with Wiskott Aldrich syndrome

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Type: review

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Language: en

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Publication year: 2019

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Publication date: 2019-03-12

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Authors: Elizabeth Rivers, Austen Worth, Adrian J. Thrasher, Siobhan O. Burns

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Landing page: https://doi.org/10.1111/bjh.15831

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PDF URL: https://onlinelibrary.wiley.com/doi/pdfdirect/10.1111/bjh.15831

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OA status: bronze

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OA URL: https://onlinelibrary.wiley.com/doi/pdfdirect/10.1111/bjh.15831

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Concepts: Wiskott–Aldrich syndrome, Medicine, Immunodeficiency, Primary immunodeficiency, Malignancy, Wiskott–Aldrich syndrome protein, Disease, Hematopoietic stem cell transplantation, Immunopathology, Transplantation, Immune system, Immunology, Dermatology, Internal medicine, Cell, Gene, Biology, Actin cytoskeleton, Cytoskeleton, Genetics, Biochemistry

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Cited by: 56

Total citation count in OpenAlex
Citations by year (recent): 2025: 7, 2024: 9, 2023: 10, 2022: 10, 2021: 12

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References (count): 48

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Related works (count): 10

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title	How I manage patients with Wiskott Aldrich syndrome
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