T cell deficiency

1.

1 point

The following characteristics describes which T cell deficiency disease:
-Absent or low level of IgA, Normal or low level of IgG, Elevated or normal level of IgM

Ataxia telangiectasia

DiGeorge Syndrome

XL hyper-IgM syndrome

Wiskott Aldrich syndrome (WASP)

Nezelof Syndrome

2.

1 point

The lifetime risk of cancer is about 100x higher in patients with Ataxia Telangiectasia than the normal population.

True

False

3.

1 point

Which of the following are caused by T cell deficiency due to T cell
differentiation?
I - Ataxia telangiectasia
II - Wiskott Aldrich syndrome (WASP)
III - Di Goerge Syndrome
IV - Nezelof syndrome

I, II, III & IV

I, II & III

III & IV

II & IV

I

II

4.

1 point

Which of the following is a defect in Nezelof's syndrome?

Defective Isotypic switching

All T cells in the peripheral blood have gamma/delta TCR

All of the Above

Abnormal migration and motility of immune cells.

Inactive purine nucloside phosphorylase

Inhibition of the Ras Kinase Pathway preventing the synthesis of AP-1

5.

1 point

Which of the following is (are) defects in Wiskott-Aldrich Syndrome?
I - Abnormal migration and motility of immune cells
II - Inability to translocate NFkB in the nucleus
III - Defective T cell proliferation

II & III

I & III

III

I & II

II

I

6.

1 point

The following characteristics describes which T Cell deficiency disease?
X-linked
Normal IgG levels
Increased IgA and IgE levels
decreased IgM
Can't produce Antibodies to carbohydrate antigens.

DiGeorge Syndrome

XL hyper-IgM syndrome

Wiskott Aldrich syndrome (WASP)

Nezelof Syndrome

Ataxia telangiectasia

7.

1 point

Which of the following are X-linked diseases?

Wiskott Aldrich Syndrome (WASP

Nezelof Syndrome

XL Hyper-IgM Syndrome

Ataxia telangiectasia

DiGoerge Syndrome

8.

1 point

10) Which of the following are caused by inactive Purine Nucleoside Phosphorylase?
I - Muscle Tetni
II - Accumulation of deoxy-GTP
III - Inhibition of ribonucleotide reductase
IV- Inhibition of DNA synthesis
V - Inhibition of Lymphocyte Proliferation

II, III, IV, V

I & II

I

II

I, II, III, IV, V

II & III

III & IV

9.

1 point

Which of the following is a congenital hypoplasia of the thymus with normal parathyroid function?

Nezelof Syndrome

None of the above

Wiskott Aldrich syndrome (WASP)

DiGeorge Syndrome

Ataxia telangiectasia

XL hyper-IgM syndrome

10.

1 point

Which of the following is caused by DiGeorge Syndrome?
I Pulmonary Stenosis - small aparture as opposed to a normal vein.
II Inhibition of DNA synthesis, preventing lymphocytes from proliferating
III Muscle Tetni

I & III

II & III

I

II

III

11.

1 point

T cells in patients with Wiskott-Aldrich Syndrome are able to translocate AP1 and NFkB in the nucleus but not NF-AT.

True

False

12.

1 point

Which of the following is a T-cell deficiency caused by the failure of development of the third and fourth pharyngeal pouches.

DiGeorge syndrome

Nezelof syndrome

Ataxia telangiectasia

XL hyper-IgM syndrome

13.

1 point

Which of the following maybe the defect(s) in X-linked Hyper IgM syndrome (XHM)?
I - B cells can't switch isotype
II - The cytokines that tell the B cells to switch are defective
III - B cells are defective, they can only produce IgM antibodies.

II & III

I & II

II

I, II, III

I & III

I

14.

1 point

Which of the following is (are) caused by T cell deficiency due to a Dysfunctional T cell?
I - Ataxia telangiectasia
II - Wiskott Aldrich syndrome (WASP)
III - Di Goerge Syndrome
IV - Nezelof syndrome

III & IV

I

I, II, III & IV

II

II & III

I & II

15.

1 point

Which of the following is NOT a characteristic of DiGeorge Syndrome?

Low set ears

Wide set eyes

Lack of muscular coordination

Defects usually on chromosome 22

Affects primarily the thymus

Shortened structure of upper lip

16.

1 point

Which of the following is used to treat Wiskott-Aldrich Syndrome?

hydroxychloroquine

IV immunoglobulin injections

FK506

Stem Cell reconstitution

Daclixumab

17.

1 point

Patients with Nezelof syndrome have normal parathyroid function. Patients with DiGeorge syndrome have no parathyroid.

True

False

18.

1 point

Which of the following is (are) major causes of T cell deficiencies?

I - T Cell differentiation
II - Dysfunctional T cells
III - IL-2 receptor defects

I

II

II & III

III

I & II

I & III

19.

1 point

Which of the following are characteristics of patients with Ataxia Telangiectasia
(AT)?
I - lack of muscle coordination
II - X-ray Hypersensitivity
III - Red veins in the corners of the eyes

I, II, III

II & III

I & III

I & II

III

I

II

20.

1 point

Why do patients with Ataxia Telangiectasia have a higher risk of developing cancer than the normal population?

They have an abnormal p53 that the ATM protein cannot bind to in order to stop cell division when DNA damage is detected.

They have a malfunctioning ATM protein that cannot activate p53 when DNA damage is detected to stop cell division.

They have a malfunctioning ATM protein that is unable to inactivated p53.p53 stay active and cell division continue uncotrolled.

Lorant have no idea what he talking about.

They have a malfunctioning p53 that is unable to activate the ATM proteinthat stop cell division when DNA is damage.

21.

1 point

Which of the following is (are) defects in Ataxia Telengiectasia?
I - Absence of the parthyroid
II - Defective Isotypic Switching
III - All peripheral blood have gamma/delta TCRs.

I & III

III

I & II

I

II

I, II, III

II & III

22.

1 point

Which of the following T cell deficiency diseases are caused by a defect in isotypic switching? (Choose all that apply)

Wiskott Aldrich Syndrome (WASP)

Nezelof Syndrome

Ataxia Telangiectasia

XL hyper-IgM syndrome

DiGeorge Syndrome

23.

1 point

Which of the following is (are) used to treat X-linked Hyper IgM syndrome (XHM)?
I - injection of IV immunoglobulin (IVIG)
II - Granulocyte colony-stimulating factor (GSF)
III Antibiotics

I

I & II

I, II, III

II & III

I & III

II