How is WAS severity scored and what does it determine?

Zhu score: 1 (thrombocytopenia only) to 5 (malignancy). Scores 3–5 include significant infections, autoimmune disease, or malignancy. Score ≥3 indicates definitive HSCT recommendation. WASP expression by flow cytometry and WAS gene sequencing confirm diagnosis and inform genotype-phenotype correlation.

What is the prognosis for WAS with and without HSCT?

Without HSCT, median survival historically was 15 years (causes: bleeding, infection, autoimmunity, lymphoma). HLA-matched sibling HSCT achieves 90%+ overall survival. Matched unrelated donor HSCT has 80% OS. Gene therapy (lentiviral WAS) has achieved sustained correction in early-phase trials.

Wiskott-Aldrich Syndrome Severity Score

Apply the Zhu severity score for Wiskott-Aldrich syndrome grading thrombocytopenia, eczema, infections, autoimmunity, and malignancy to stratify patients for hematopoietic stem cell transplantation.

Use the full interactive calculator below, or

Related Calculators

PID Suspicion Score

Medical Long-tail

Wiskott-Aldrich Syndrome Severity Score

Related Calculators

Frequently Asked Questions

How is WAS severity scored and what does it determine?

What is the prognosis for WAS with and without HSCT?