93. 關於Wiscott-Aldrich症候群之敘述下列何者為非？
(A)屬於性聯隱性遺傳(X-linkel recessive)。
(B)血液抹片可見微小血小板(Microplatelet)。
(C)有濕疹及瘀青。
(D)對多醣體抗原之抗體免疫反應低下。
(E)治療首選是定期注射免疫球蛋白(IVIG)。

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統計： A(7), B(7), C(2), D(2), E(17) #1125800

Yen-Ju Chu

B1 · 2016/06/06

#1371070

Wiskott-Aldrich syndrome:

Genetic:

X-linked recessive disease

mutation on Xp11.22-11.23

Manifestation

Atopic dermatitis

Thrombocytopenic purpura with normal appearing megakaryocytes but small defective platelets

Undue susceptibility to infection.

Onset

Prolonged bleeding from the circumcision site or bloody diarrhea during infancy.

Atopic dermatitis and recurrent infections usually develop during the 1st yr of life.

Immunodeficiency:

Uniformly impaired humoral immune response to polysccharide antigens.

Reduced T cell percentage, depressed lymphocyte response to mitogens.

Variable Ig level, ususally low IgM, elevated IgA and IgE, normal or slightly low IgG

Treatment:

Monthly IVIG should be given regardless of serum Ig levels.

Bone marrow or cord blood transplantation is the treatment of choice and is usually curative.