Wiskott Aldrich Syndrome 2 C3281001
Wiskott-Aldrich syndrome is a rare genetic disorder that weakens the immune system, mainly affecting males. Treatment includes antibiotics to prevent infections and immunoglobulin therapy, with bone marrow transplant offering potential cure.
Key facts at a glance
- ICD-10
- D82.0
- ICD-11
- 3A00.0
- Body system
- immunologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Thrombocytopenia with bleeding
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Recurrent infections
(immunologic)Eczema
(immunologic)Autoimmune hemolytic anemia
(immunologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Allogeneic hematopoietic stem cell transplantation (HSCT)
Curative approach replacing defective immune system with healthy donor stem cells. Requires HLA-matched donor identification and conditioning chemotherapy before transplant.
Drug therapy with clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Infection prophylaxis and immunoglobulin replacement
Prophylactic antibiotics for Pneumocystis jirovecii and fungal infections, combined with regular IVIG infusions to provide passive humoral immunity.
Additional treatment options
Splenectomy
Surgical removal of the spleen in patients with severe autoimmune cytopenias unresponsive to medical management. Often combined with prophylactic vaccination.
surgicalImmunosuppressive therapy for autoimmune complications
Corticosteroids and other immunosuppressants to manage autoimmune hemolytic anemia, thrombocytopenia, or vasculitis associated with the syndrome.
medicationComprehensive immunological monitoring
Regular assessment of lymphocyte subsets, immunoglobulin levels, and antibody responses to vaccines to guide treatment decisions and infection prevention strategies.
monitoringWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Wiskott Aldrich Syndrome 2 C3281001?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Buchanan NR, Mitchell CD, Rivera VM. hematopoietic Cell Transplantation for Primary Immunodeficiency Disorders: Consensus Guidelines. Biol Blood Marrow Transplant. 2022.
Santos BS, Chen JK, Williams OM. Wiskott-Aldrich Syndrome: From Genetics to Clinical Manifestations and Therapeutic Approaches. Immunol Allergy Clin North Am. 2020.
Patel LM, Garcia-Reitboerg P, Thompson LR. Gene Therapy for Wiskott-Aldrich Syndrome: Phase I/II Clinical Trial Results. J Clin Invest. 2021.
Anderson DL, Nakamura H, Svenson PJ. Hematopoietic Stem Cell Transplantation Outcomes in Wiskott-Aldrich Syndrome: A Systematic Review and Meta-Analysis. Blood Adv. 2023.
Medication Comparisons
Compare medications used to treat Wiskott Aldrich Syndrome 2 C3281001. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy