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Immunology

Description

All immune deficiencies except HIVb
ivenkat
Quiz by ivenkat, updated more than 1 year ago
ivenkat
Created by ivenkat over 9 years ago
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Resource summary

Question 1

Question
All of the following are correctly matched except?
Answer
  • 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

Question 2

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

Question 3

Question
All of the following are defects in phagocytic cells except?
Answer
  • LAD (Leukocyte Adhesion Deficiency)
  • CGD (chronic granulomatous disease)
  • G6PD deficiency
  • Myeloperoxidase deficiency
  • Chediak-Higashi syndrome
  • None of the above

Question 4

Question
All of the following are seen in LAD except?
Answer
  • 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

Question 5

Question
For CGD dihydrorhodamine test (DHR) is more accurate than NBT
Answer
  • True
  • False

Question 6

Question
All of the following are correctly matched except?
Answer
  • Chediak Higashi - no oxidative burst
  • Chronic granulomatous disease - no oxidative burst
  • Both of the above
  • None of the above

Question 7

Question
all of the following are correctly matched except?
Answer
  • Paroxysmal Nocturnal Hemoglobinuria - DAF deficiency (CD55/CD59)
  • Hereditary Angioedema - C1 esterase deficiency
  • CH50 tests - alternate pathway and A50 tests - classic pathway

Question 8

Question
All of the following about Bruton's agammaglobulinemia are correct except?
Answer
  • 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)

Question 9

Question
X linked hyper IgM (XHIGM) - all of the following are true except?
Answer
  • a B cell defect
  • CD 40 L is deficient
  • None of the above

Question 10

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

Question 11

Question
IN CVID the roles in B and plasma cell survival and isotype switch is common due to a defect in TACI
Answer
  • True
  • False

Question 12

Question
all of the following are true about Di George syndrome except
Answer
  • Autosomal dominant inheritance
  • Deletion of 22q11
  • Hypercalcemia
  • Low set ears, underdeveloped jaw, small mouth, abnormally increased distance between the eyes

Question 13

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

Question 14

Question
All the following statements are true except?
Answer
  • 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

Question 15

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

Question 16

Question
All of the following are true except?
Answer
  • JAK3 deficiency - Autosomal Dominant SCID
  • Omenn Syndrome - Cannot undergo somatic recombination

Question 17

Question
The following are correctly matched except?
Answer
  • 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

Question 18

Question
All of the following are true about Wiskott-ALdrich Syndrome except ?
Answer
  • X-linked recessive
  • Thrombocytosis (elevated platelet levels)
  • Severe eczema by one year of age - elevated IgE
  • recurrent pyogenic infections with encapsulated bacteria, bloody diarrhea

Question 19

Question
All of the following about Ataxia-Telangiectasia are true except?
Answer
  • Autosomal recessive
  • Selective IgM deficiency

Question 20

Question
X-linked lymphoproliferative syndrome - highly fatal infectious mononucleosis
Answer
  • True
  • False

Question 21

Question
In both multiple mylemoa and Waldenstrom's Macrogobulinemia have punched out lesions in the skull
Answer
  • True
  • False
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