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BMS150 Midterm 2 > Immunology - Immunodeficiencies > Flashcards

Immunology - Immunodeficiencies Flashcards

1
Q

Immunodeficiency that is mostly inborn (genetic) and often detected in infancy or childhood (sometimes adulthood)

A

Primary Immunodeficiency

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2
Q

Immunodeficiency that is acquired due to external factors

A

Secondary Immunodeficiency

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3
Q

Causes the inability of Pro-B cells to differentiate into Pre-B cells

A

X-linked agammaglobulinemia

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4
Q

In XLA, they can make a ______ chain variable region but not a ______ chain, so they are unable to make antibodies

A

heavy; light

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5
Q

Enzyme lacking in XLA

A

Tyr kinase

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6
Q

Clinical features of XLA

A
  • Usually only affects males (X-linked)
  • Recurrent respiratory infections call attention to the disease
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7
Q

T/F: Most bacteria causing recurring respiratory infections in patients with XLA are gram (+)

A

True

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8
Q

Diagnostic findings for XLA (3)

A
  • B-cell absence or very decreased
  • All immunoglobulins are depressed
  • B-cell areas of lymphatic tissues are underdeveloped
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9
Q

XLA treatment

A

IVIG therapy

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10
Q

Immunological issues present with common variable immunodeficiency (3)

A
  • defects in most classes of Ab secretion
  • inability of Th cells to amplify Ab production
  • reduced CT cell activity
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11
Q

A common feature of CVI is __________, usually involving all AB classes but sometimes only IgG

A

hypogammaglobulinemia

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12
Q

Clinical features of CVI

A

Resembles XLA - recurrent infections; also can have recurrent severe herpes infection

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13
Q

Diagnostic findings of CVI

A
  • no other B-cell abnormality
  • reduced immunoglobulins
  • B-cell areas of lymphatic tissues are hyperplastic
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14
Q

Treatment of CVI

A

IVIG therapy

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15
Q

Most common primary immune deficiency

A

Isolated IgA deficiency - 1/600

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16
Q

Symptoms of patients with Isolated IgA deficiency?

A

Most asymptomatic

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17
Q

Patients with Isolated IgA deficiency have a Hx of recurrent…?

A

otitis media, sinusitis, bronchitis, pneumonia, GIT infections

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18
Q

Potentially deadly complication of Isolated IgA deficiency?

A

Anaphylaxis post-blood transfusion since transfused IgA antibodies recognized as foreign

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19
Q

Clinical pearl of IgA deficiency?

A

Serology for detecting celiac disease is based on IgA detection => IgA deficiency can lead to false negatives in celiac patients

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20
Q

Immunodeficiency where patients make IgM but have difficulty producing IgG, IgA, IgE?

A

Hyper-IgM Syndrome

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21
Q

What chromosome is Hyper-IgM Syndrome gene on?

A

X chromosome

22
Q

In Hyper-IgM Syndrome, Th-cells can’t activate ______ and _______

A

B-cells and macrophages

23
Q

Clinical features of hyper-IgM syndrome

A
  • Recurrent purulent infection (CNS, resp tract, GIT)
  • Many viral infections
  • decreased neutrophil counts
24
Q

Treatment for hyper-IgM syndrome?

A

IVIG and intense antibiotic prophylaxis

25
Q

T-cell deficiency in which the 3rd and 4th pharyngeal pouches don’t develop

A

22q11 deletion (DiGeorge) Syndrome

26
Q

With DiGeorges syndrome, you’re short on…?

A

A thymus parathyroid gland, some thyroid tissue - also heart and great vessel defects

27
Q

Hypothesized that the _____ family of transcription factors is absent/doesn’t function, which is important for the transcription of _________.

A

T-box; brachial/pharyngeal arch development and large vessels

28
Q

Because of thymic hypoplasia resulting in variable loss of T-cells, what is increased?

A

Increased fungal and viral infections, and autoimmunity

29
Q

Clinical features of DiGeorge syndrome

A
  • Cardiac abnormalities
  • Craniofacial abnormalities
  • Developmental delay
  • Hypoparathyroidism
30
Q

What electrolyte abnormality is associated with hypoparathyroidism?

A

Ca and P

31
Q

Blood products can result in what for patients with DiGeorge syndrome?

A

graft-vs-host disease

32
Q

What is graft-vs-host disease?

A

Mothers T-cells are transferred across the placenta occasionally, causing the mom’s lymphocytes to attack the baby’s tissue

33
Q

A multitude of etiologies defined by recurrent, severe infections by a wide range of pathogens

A

Severe Combined Immunodeficiency

34
Q

What is required in SCID patients to ensure death doesn’t occur at a young age?

A

Bone marrow transplant or stem cell therapies

35
Q

In SCID, there are defects in both ______ and ______ immunity.

A

cell-mediated and humoral

36
Q

What is X-linked SCID due to?

A

mutation in the gamma-chain of a variety of cytokine receptors

37
Q

Signal transducing component of what receptors for which IL’s are affected?

A

IL-2, IL-4, IL-7, IL-9, IL-11, IL-15, IL-21

38
Q

What is autosomal SCID due to?

A

mutation in adenosine deaminase and RAG mutations

39
Q

What does adenosine deaminase mutation do?

A

causes accumulation of deoxyadenosine and its derivatives = toxic to rapidly dividing lymphocytes

40
Q

What does a RAG mutation do?

A

Blocks B-cell and T-cell development

41
Q

Pathological findings of SCID?

A
  • small thymus with few lymphocytes
  • depletion in T-cell areas of other lymphatic tissue
42
Q

Most common complement defect?

A

C2 deficiency

43
Q

Why do many people with C2 deficiency have no increased incidence of infection?

A

Alternative pathway is adequate for most cases

44
Q

Autosomal dominant disorder less common than the complement deficiency disorders, that causes a deficit in C1 inhibitor?

A

Hereditary angioedema

45
Q

Results of a deficit in C1 inhibitor?

A
  • unchecked activation of classical pathway
  • Increased bradykinin production
  • increased activation of certain components of clotting cascade
46
Q

Clinical features of hereditary angioedema

A
  • episodic attacks that usually progressively become worse
  • severe abdominal pain
  • swelling of face, hands, legs, groin
47
Q

Hereditary angioedema treatment

A

C1 inhibitor from blood products

48
Q

Fungal infection of the lung

A

Pneumocytis jiroveci pneumonia

49
Q

Reactivation of varicella zoster virus => painful rash

A

Herpes Zoster (shingles)

50
Q

Conversion of latent TB to active form that usually affects the lungs

A

Tuberculosis

51
Q

A chronic parasitic infection usually acquired through direct contact with contaminated soil

A

Stringyloidiasis

BMS150 Midterm 2 (14 decks)

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