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Allergy/Immunology > Immunodeficiencies > Flashcards

Immunodeficiencies Flashcards

1
Q

Primary immunodeficiencies

A

Immunodeficiencies that are truly the underlying cause. not a secondary characteristic of another disease.

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2
Q

Normal Child immune system

A
  • 6-8 respiratory infections per year for first 10-12 years.

- 6 episodes of otitis and 2 episodes of GE per year for first 2-3 years.

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3
Q

A child with an immunodeficiency should NOT receive what?

A

Live virus vaccines.

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4
Q

XLA

A

-Brunton’s
-X-linked hypogammaglubulinemia
-B-cell deficiency
-Pyogenic infxns
Tx: IVIG, IGSC
-Reduces number and deficiency of infxns

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5
Q

sIgAD

A
  • Selective IgA deficiency
  • Decreased IgA with normal IgM and IgG
  • Should be made after age 4
  • Most common primary immunodeficiency in adults
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6
Q

sIgAD presentation

A
  • Incidental finding w/o clinical features in most persons.

- Increased incidence of sinopulmonary infxns

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7
Q

Patients with sIgAD should NOT get a____?

A

Transfusion, or gamma globulin because of possible anaplylaxis against IgA in donor blood.

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8
Q

B cells protect against?

A

Bacteria

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9
Q

T cells protect against?

A

Viruses, Fungus

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10
Q

CVID

A
  • B-cell deficiency
  • Common variable immunodeficiency
  • Defective immunoglobulin production
  • Markedly low levels of IgG, low levels of IgA and IgM.
  • A collection of diseases
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11
Q

CVID presentation

A
  • Recurrent sinopulmonary infxns
  • Malignant disease, inflammatory disorders, autoimmunity
  • Chronic intestinal disease
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12
Q

Thymic Aplasia

A 
T-cell deficiency
DiGeorge's Syndrome
-Defective development of pharyngeal pouch system, which gives rise to thymus, thyroid, parathyroid
-Hypothyroidism (cretinism)
-Hypoparathyroidism
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13
Q

Thymic aplasia presentation

A
  • Severe viral, fungal, protozoal infxns
  • Occurs early in life
  • Pneumonia and thrush are most common
  • Cardiac abnormalities, tetany
  • facial abnormalities, cleft pallet
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14
Q

Thymic aplasia management

A
  • Immediate tx of cardiac dysfxn.
  • Control metabolic abnormalities (hypocalcemia and tetany)
  • Give only irradiated blood
  • If severe, consider thymus transplant
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15
Q

SCID

A

Severe Combined Immunodeficiency (bubble boy)

  • Absence of normal thymic tissue and other lymph tissues and devoid of lymphocytes.
  • Both B and T cells are defective*
  • Levels may be very low, or normal but not functioning properly.
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16
Q

SCID Presentation

A
  • Failure to thrive: Usually die within first 1-2 years.
  • Pneumocystis, viral infxns
  • Chronic diarrhea, diaper rash
  • Thrush
17
Q

SCID Tx

A
  • Bone marrow or blood transplantation may restore immunity.
  • Transplants done in first 3 months of life have high success rates.
  • IVIG
  • Enzyme replacement drugs
18
Q

SCID Genetic Defect

A

Is from several different areas/genes
X-linked most common
Some are autosomal

19
Q

Wiscott Aldrich, Ataxia-Telangiectasia

A

VERY rare combo immunodeficiency diseases.

20
Q

Hereditary Angioedema

A
  • Uncommon autosomal dominant disease
  • Caused by a deficiency of C1 inhibitor.
  • Characterized by edema of axilla and airway.
  • Abd. pain, N/V
21
Q

Hereditary Angioedema Mgmt

A

Acute: May have to intubate if airway is affected.

  • Human plasma (C1 inhibitor concentrate)
  • FFP’s, androgens, C1 inhibitor for prophylaxis or chronic disease state.
22
Q

Malnutrition Immunodeficiency

A
  • Increased infxn rates
  • contributing factor in 60% of infxn related deaths in latin america.
  • T cells decline, specific AB responses impaired
  • Declined phagocytic fxns
  • Lymphoid organs depleted of cells
23
Q

Infections that result in immunodeficiency

A

HIV
Measles (T-cell lymphopenia)
Parasite infections (alteration of macrophage fxn)

24
Q

Cancer

A

Immune deficiency secondary to chemo, radiation, and cancer itself.

  • Chronic lymphocytic leukemia
  • Multiple myeloma
  • Solid tumors (inhibit fxn of lymphocytes)
25
Q

Renal disease

A
  • Nephrotic Syndrome: Cant fight infxn
  • Dialysis (reduced T cell fxn)
  • Glucocorticoids (suppress phagocytosis)
26
Q

Sarcoidosis

A
  • Autoimmune disease
  • Accumulation of T lymphocytes, phagocytes and pulmonary granulomas.
  • T-cell lymphopenia
  • B-cell hyperactivity
27
Q

2 meds used in transplants for immunosuppression

A

Cyclosporine

Tacrolimus

Allergy/Immunology (10 decks)

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