All of the following statement are true about wiskott Aldrich syndrome except
**Core Concept**
Wiskott-Aldrich Syndrome (WAS) is a rare X-linked recessive disorder characterized by eczema, thrombocytopenia, and immune deficiency, primarily affecting males. The syndrome is caused by mutations in the WAS gene, which encodes a protein called WASp involved in the regulation of actin cytoskeleton and platelet function.
**Why the Correct Answer is Right**
WAS is an X-linked recessive disorder, meaning the mutated gene responsible for the condition is located on the X chromosome. Since males have only one X chromosome, a single copy of the mutated gene is sufficient to cause the condition. Females, on the other hand, would need to inherit two copies of the mutated gene (one from each parent) to develop the condition, making it less likely. The other options are correct statements about WAS: eczema (D) is a common feature, thrombocytopenia (C) is a hallmark of the condition, and there is indeed a failure of platelet aggregation in response to agonists (B) due to the role of WASp in platelet function.
**Why Each Wrong Option is Incorrect**
**Option A:** This statement is incorrect because WAS is an X-linked recessive disorder, not autosomal recessive. Autosomal recessive disorders affect both males and females equally and require two copies of the mutated gene to manifest.
**Option B:** While platelet aggregation is affected in WAS, stating "failure of aggregation of platelets in response to agonists" is not entirely accurate. The actual issue is with platelet function and the cytoskeletal changes that impair platelet aggregation.
**Option D:** Eczema is a common feature of WAS, so this statement is true.
**Clinical Pearl / High-Yield Fact**
Wiskott-Aldrich Syndrome is a classic example of a condition where the genetic defect affects multiple cell types, including platelets, T cells, and B cells, highlighting the importance of understanding the complex interplay between genetic mutations and cellular function.
**β Correct Answer: A. It is an autosomal recessive disorder**