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All immune deficiencies except HIVb

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Immunology

Immunology

Question 1 of 21

1

All of the following are correctly matched except?

Select one of the following:

  • APCED - AIRE gene is affected - T cells react to self antigens (Central tolarence)

  • IPEX - FOX P3 is affected - Lack of Tregs - autoimmune - Peripheral tolarence

  • Chronic granulomatous Disease - gp91 gene - NADPH oxidase deficiency

  • Leucocyte adhesion deficiency - CD20 deficiency

Explanation

Question 2 of 21

1

All of the following lead to suspicion of an immunodeficiency in infants or children except

Select one of the following:

  • Needs for IV antibiotics and or hospitalization

  • 2 or more serious sinus infections or pneumonias within a year

  • Recurrent or resistant candidasis

  • Infection with opportunistic organism

  • Resistant to viral infections

Explanation

Question 3 of 21

1

All of the following are defects in phagocytic cells except?

Select one of the following:

  • LAD (Leukocyte Adhesion Deficiency)

  • CGD (chronic granulomatous disease)

  • G6PD deficiency

  • Myeloperoxidase deficiency

  • Chediak-Higashi syndrome

  • None of the above

Explanation

Question 4 of 21

1

All of the following are seen in LAD except?

Select one of the following:

  • Recurrent viral infections

  • Elevated white blood count

  • autosomal recessive

  • Rebuck Skin window test and flow cytometric analyses show absence of CD18 are diagnostic

  • Absence of pus and delayed wound healing

Explanation

Question 5 of 21

1

For CGD dihydrorhodamine test (DHR) is more accurate than NBT

Select one of the following:

  • True
  • False

Explanation

Question 6 of 21

1

All of the following are correctly matched except?

Select one of the following:

  • Chediak Higashi - no oxidative burst

  • Chronic granulomatous disease - no oxidative burst

  • Both of the above

  • None of the above

Explanation

Question 7 of 21

1

all of the following are correctly matched except?

Select one of the following:

  • Paroxysmal Nocturnal Hemoglobinuria - DAF deficiency (CD55/CD59)

  • Hereditary Angioedema - C1 esterase deficiency

  • CH50 tests - alternate pathway and A50 tests - classic pathway

Explanation

Question 8 of 21

1

All of the following about Bruton's agammaglobulinemia are correct except?

Select one of the following:

  • X linked recessive transmission

  • Serum titers of IgM is not detectable, Iga and IgG are low

  • cannot handle measles infection

  • none of the above

  • Caused by mutation in btk gene (Bruton's tyrosine kinase)

Explanation

Question 9 of 21

1

X linked hyper IgM (XHIGM) - all of the following are true except?

Select one of the following:

  • a B cell defect

  • CD 40 L is deficient

  • None of the above

Explanation

Question 10 of 21

1

All of the following for selective IgA deficiency are true except?

Select one of the following:

  • Most common immunodeficiency in European descent

  • Only mucosal IgA is absent and serum is normal

  • none of the above

  • Some patients when transfused with blood containing normal IgA develop severe even fatal anaphylactic reaction because they have made IgE anti-IgA antibodies

Explanation

Question 11 of 21

1

IN CVID the roles in B and plasma cell survival and isotype switch is common due to a defect in TACI

Select one of the following:

  • True
  • False

Explanation

Question 12 of 21

1

all of the following are true about Di George syndrome except

Select one of the following:

  • Autosomal dominant inheritance

  • Deletion of 22q11

  • Hypercalcemia

  • Low set ears, underdeveloped jaw, small mouth, abnormally increased distance between the eyes

Explanation

Question 13 of 21

1

All of the following are true about Bare Lymphocytes syndrome (BLS) except?

Select one of the following:

  • BLS type I - MHCII deficiency, BLS type II - MHC I deficiency

  • Mutation in TAP 1 or TAP 2 genes

  • All of the above

  • None of the above

Explanation

Question 14 of 21

1

All the following statements are true except?

Select one of the following:

  • ADA deficiency - has NO T,B, NK cell

  • PNP deficiency - No T cells

  • X linked SCID - No T cells

  • Autosomal SCID - NO T or B cells

Explanation

Question 15 of 21

1

All of the following about X- linked SCID are true except?

Select one of the following:

  • LEAST common form of SCID

  • Mutation of the gamma-c chain of IL-2 receptor (CD132)

  • Normal B cell levels

  • lack of delayed hypersensitivity, atrophy of thymus, susceptible to viral fungal and bacterial infections, lymphocytopenia

Explanation

Question 16 of 21

1

All of the following are true except?

Select one of the following:

  • JAK3 deficiency - Autosomal Dominant SCID

  • Omenn Syndrome - Cannot undergo somatic recombination

Explanation

Question 17 of 21

1

The following are correctly matched except?

Select one of the following:

  • X linked SCID - absent T cells, B cells present - Jak 3 deficiency and CD45 deficiency

  • IL-7R alpha chain deficiency - CD3 delta chain deficiency - absent T cell, B cell absent

  • ADA deficiency - absent T and B cells

  • RAG1 or RAG2 deficiency - absent T and B cells

Explanation

Question 18 of 21

1

All of the following are true about Wiskott-ALdrich Syndrome except ?

Select one of the following:

  • X-linked recessive

  • Thrombocytosis (elevated platelet levels)

  • Severe eczema by one year of age - elevated IgE

  • recurrent pyogenic infections with encapsulated bacteria, bloody diarrhea

Explanation

Question 19 of 21

1

All of the following about Ataxia-Telangiectasia are true except?

Select one of the following:

  • Autosomal recessive

  • Selective IgM deficiency

Explanation

Question 20 of 21

1

X-linked lymphoproliferative syndrome - highly fatal infectious mononucleosis

Select one of the following:

  • True
  • False

Explanation

Question 21 of 21

1

In both multiple mylemoa and Waldenstrom's Macrogobulinemia have punched out lesions in the skull

Select one of the following:

  • True
  • False

Explanation

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