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Related Experiment Video

Updated: Jul 14, 2026

Isolating Human Peripheral Blood Mononuclear Cells and CD4+ T cells from Sézary Syndrome Patients for Transcriptomic Profiling

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Wiskott-Aldrich syndrome.

H A Harfi¹, S Al-Malik, A Tulba

¹Division of Allergy and Clinical Immunology, Departments of Pediatrics and Medicine, and Pathology and Laboratory Medicine, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.

Annals of Saudi Medicine

|July 1, 1992

Summary

Wiskott-Aldrich Syndrome (WAS) cases in Saudi Arabia showed successful bone marrow transplants and splenectomies, correcting hematologic and immunologic issues. This study details the first reported WAS cases from the region.

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Area of Science:

Immunology
Hematology
Pediatrics

Background:

Wiskott-Aldrich Syndrome (WAS) is a rare X-linked immunodeficiency characterized by eczema, thrombocytopenia, and recurrent infections.
Early diagnosis and management are crucial for improving outcomes in WAS patients.

Purpose of the Study:

To report the first detailed cases of Wiskott-Aldrich Syndrome diagnosed in Saudi Arabia.
To describe the clinical presentation, management, and outcomes of WAS patients in a tertiary care setting.

Main Methods:

Retrospective case series analysis of five Wiskott-Aldrich Syndrome patients diagnosed between 1981 and 1990.
Review of patient records, including clinical history, laboratory findings, and treatment interventions such as bone marrow transplantation and splenectomy.

Main Results:

Five cases of Wiskott-Aldrich Syndrome were identified, with a notable history of early-onset rectal bleeding in most patients.
Two patients with classical WAS underwent successful allogeneic bone marrow transplantation from their sisters, achieving complete hematologic and immunologic engraftment, normalization of IgE, and resolution of eczema.
One patient died from intracranial hemorrhage, while two others underwent splenectomy, resulting in normalized platelet counts and cessation of bleeding.

Conclusions:

Bone marrow transplantation is an effective curative treatment for Wiskott-Aldrich Syndrome, restoring immune function and hematologic parameters.
Splenectomy can be a viable option for managing thrombocytopenia and bleeding in WAS patients when transplantation is not feasible.
This report highlights the importance of recognizing and managing Wiskott-Aldrich Syndrome in Saudi Arabia, providing valuable insights into its presentation and treatment outcomes in the region.