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Pathology Other Autoimmune Disorders 8abf867a

All of the following statements are true about Wiskott Aldrich syndrome except:

A
It is an autosomal recessive disorder
B
There is failure of aggregation of platelets in response to agonists
C
Thrombocytopenia is seen
D
Patient presents with eczema
High-Yield Explanation
Wiskott-Aldrich syndrome X-linked disease characterized by thrombocytopenia, eczema, and a marked vulnerability to recurrent infection, resulting in early death. There is progressive loss of T-lymphocytes in the peripheral blood and in the T-cell zones (paracoical areas) of the lymph nodes, with variable defects in cellular immunity. Patients do not make antibodies to polysaccharide antigens, and the response to protein antigens is poor. IgM levels in the serum are low, but levels of IgG are usually normal. Paradoxically the levels of IgA and IgE are often elevated. Rx:- Allogeneic hematopoietic stem cell transplant

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