Immunodeficiency Flashcards

1
Q

Primary immunodeficiency diseases:

A

Conditions characterized by intrinsic deficits within the immune system and are caused by inherited or de novo genetic defects.

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2
Q

Antibody deficiency pattern of infections:

A
  • Bacteria: pneumococcus, H. flu, moraxella, staph aureus, meningococcus, pseudomonas, campylobacter, mycoplasma, ureaplasma.
  • Virus: common respiratory especially enteroviruses, rotavirus.
  • Protozoa: giardia, cryptosporidium.
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3
Q

Combined immunodeficiency pattern of infections:

A
  • Bacteria: listeria, enteric flora.
  • Virus: herpesviruses, RSV, influenza, parainfluenza, measles.
  • Fungi: pneumocystis, candida, cryptococcus, histoplasma.
  • Protozoa: toxoplamsa, cryptosporidium.
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4
Q

Cellular immunodeficiency pattern of infections:

A
  • Mycobacteria, especially atypical and including BCG.
  • Salmonella.
  • Candida.
  • Herpes viruses.
  • Pneumocystis.
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5
Q

Phagocyte defects pattern of infections:

A
  • Bacteria: Catalase positive; mycobacteria including BCG.

- Fungi: candida, aspergillus, paecilomyces.

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6
Q

X-linked agammaglobulinemia:

A
  • Susceptibility: pygoenic infections, viral meningo-encephalitis, vaccine strain poliomyelitis, mycoplasma arthritis.
  • Features: infancy/childhood with recurrent sinopulmonary pygenic infections, 25% with neutropenia.
  • Inheritance: X-linked.
  • Diagnosis: Absent B cells, family history, IgG < 100 mg/dl, B cells < 2% of lymphocytes, normal T cell number and function.
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7
Q

Common variable immunodeficiency:

A
  • Susceptibility: bacteria, common respiratory and enteroviruses, rotavirus, giardia, cryptosproidium.
  • Features: recurrent sinopulmonary infections, bronchiectasis, diarrhea, arthritis, giardia, autoimunity, asthma, lymphoproliferative disease, gastric CA and lymphoma.
  • Inheritance: sporadic, autosomal recessive.
  • Diagnosis: hypogammaglobulinemia, B cells present. Impaired antibody response.
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8
Q

Severe Combined Immunodeficiency (SCID):

A
  • Susceptibility: all infectious organisms including vaccine strains and opportunistic infections.
  • Features: failure to thrive, chronic diarrhea, erythroderma or other skin eruption. Specific gene defects may have associated features.
  • Inheritance: X-linked or autosomal recessive.
  • Diagnosis: lymphopenia in most, diminished or absent T cells in most, poor/absent in vitro mitogen-induced T cell proliferation in all.
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9
Q

Treatment of SCID:

A

Stem cell transplantation.

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10
Q

Chronic granulomatous disease:

A
  • Susceptibility: catalase positive organisms, indolent fungal infections.
  • Features: recurrent infections. Granulomas of skin, liver, lungs, lymph nodes, viscera, bones, joints. GI/GU obstruction secondary to granulomas.
  • Diagnosis: neutrophil oxidative burst assay by flow cytometry using DHR or nitroblue tetrazolium.
  • Tx: prophylactic antibiotics, gamma interferon, bone marrow transplantation.
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11
Q

Complement component deficiency:

A
  • Susceptibility/Features: recurrent pyogenic infection and also connective tissue disease. Late component deficiency (C5-9) recurrent neisseria species infection., Deficiency of regulatory protein C1 esterase inhibitor is associated with angioedema.
  • Diagnosis: CH50 for classical pathway, AH50 for alternative pathway, and/or individual complement levels.
  • Tx: prophylactic antibiotics, immunizations with bacterial polysaccharide vaccines.
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