Common T-cell immune-def. syndromes

Question 1

What are common T-cell immune-def. syndromes?

Answer 1

• Thymic aplasia
• Chronic mucocutaneous candidiasis
• Hyper IgE

Question 2

What is Thymic aplasia?

Answer 2

Thymic aplasia: absent thymus and absent parathyroid glands due to microdeletion of chromosome 22; decreased T-cells (increased viral and fungal infections), decreased PTH, and decreased Ca2+, no thymic shadow, due to:

• –DiGeorge syndrome/velocardiofacial syndrome:
• —–Cardiac defects: (tetralolgy of fallot or —truncus arteriosus)
• ——Abnormal facies (sometimes)
• ——Thymic hypoplasia
• ——Cleft palate (sometimes)
• ——Parathryoid aplasia

Question 3

What is Chronic mucocutaneous candidiasis?

Answer 3

Chronic mucocutaneous candidiasis is a T-cell dysfunction with impaired immunity against Candida

Question 4

What is Hyper IgE syndrome?

Answer 4

It is the def of Th17 cells; impaired neutrophil recruitment