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19. Integrative > 5. Defensive Breakdown > Flashcards

5. Defensive Breakdown Flashcards

1
Q

What type of inheritance does B cell congenital immunodeficiency have?

A

X linked recessive.

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2
Q

What is the presentation of B cell congenital immunodeficiency?

A

Early presentation, no tonsils. Get recurrent infections.

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3
Q

How are immunoglobulin levels impacted by B cell congenital immunodeficiency?

A

Low IgA and IgM.

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4
Q

What is B cell insufficiency?

A

Failure of B cells to mature.

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5
Q

What are the infections patients with B cell insufficiency get?

A

Recurrent bacterial, usually respiratory tract infections.

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6
Q

How is B cell insufficiency treated?

A

Ig transplantation.

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7
Q

What immunodeficiency is seen in DiGeorge syndrome?

A

T cell.

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8
Q

How does DiGeorge syndrome cause immunodeficiency?

A

Absent thymus and other congenital defects so T cell deficiency.

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9
Q

Which infections are T cell deficient patients prone to?

A

Fungal and viral infections primarily.

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10
Q

What is severe combined immunodeficiency?

A

Recessive condition causing low B and T cells.

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11
Q

How does SCID present?

A

Chronic thrush, extensive diaper rush, failure to thrive, recurrent fungal, bacterial and viral infections.

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12
Q

How is SCID managed?

A

Bone marrow transplant.

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13
Q

What is ataxia telangiectasia?

A

Recessive immunodeficiency with thymic hypoplasia and low B cells which is fatal by aged 20.

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14
Q

How is ataxia telangiectasia treated?

A

With bone marrow transplantation.

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15
Q

What is chronic granulomatous disease?

A

X linked recessive disorder leading to persistent infections of the skin, respiratory and GI tract due to defective bacterial killing by neutrophils.

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16
Q

What are the acquired B cell immunodeficiencies?

A

Hypogammaglobulinaemia, chronic lymphatic leukaemia, lymphoproliferative disorders, myeloma, nephrotic syndrome.

17
Q

What are the acquired T cell immunodeficiencies?

A

HIV, chemotherapy, Hodgkin’s disease, immunosuppression.

18
Q

What are the acquired B and T cell immunodeficiencies?

A

Radiotherapy, chronic lymphatic leukaemia, malnutrition.

19
Q

What are the acquired neutrophil immunodeficiencies?

A

Neutropenia (not enough), myelodysplasia (not functioning).

20
Q

What does the HIV virus infect?

A

T helper cells via CD4 molecules, this affects T and B cell responses.

21
Q

What is the result of the immunodeficiency of HIV?

A

Opportunistic infections.

22
Q

What are the fungal infections seen in HIV?

A

Pneumocystis jiroveci (pneumonia), candida albicans (thrush), aspergillus fumigatus (pneumonia), histoplasma capsulatum (disseminated), cryptococcus neoformans (meningoencephalitis, pneumonia).

23
Q

What are the mycobacterial infections in AIDS?

A

TB, MAI (mycobacterium avium-intracellular).

24
Q

What are the parasitic infections seen in AIDS?

A

Cryptosporidia (GIT), isospora (colon), toxoplasma gondii (CNS, eyes, lymph nodes).

25
Q

What are the viral infections seen in AIDS?

A

Cytomegalovirus (GIT, CNS), herpes zoster (shingles), herpes simplex (muco-cutaneous).

26
Q

How is pneumocystis jiroveci investigated?

A

Direct microscopy of bronchoalveolar lavage.

27
Q

How is pneumocystic jiroveci treated empirically?

A

Cotrimoxazole.

28
Q

What is the appearance of pneumocystic jiroveci on CXR?

A

Fine, reticular, interstitial pattern. Fine and patchy.

29
Q

How is candida albicans diagnosed?

A

Clinically - white plaques.

30
Q

What is the treatment of candida albicans?

A

Fluconazole or related drugs.

31
Q

How does CMV normally arise?

A

Reactivation of old infection.

32
Q

What can CMV cause?

A

Pneumonitis, oesophagitis, colitis, hepatitis.

33
Q

What is the treatment of CMV?

A

Acyclovir, gancyclovir.

34
Q

How is herpes simplex treated?

A

Acyclovir, gancyclovir.

35
Q

What is primary and secondary TB?

A

Primary - first exposure.

Secondary - reactivation of past infection that didn’t cause illness.

36
Q

What is post-transplant lymphoproliferative disorder?

A

B cell proliferation driven by EBV that may progress to lymphoma.

37
Q

How is post-transplant lymphoproliferative disorder managed?

A

Reduced immunosuppression.

19. Integrative (7 decks)

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