Diseases of Immunity Pt 3 Flashcards

1
Q

Histopath of B cell deficiencies

A

absent or reduced follicles and germinal centers in lymphoid organs, reduced Ig levels

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2
Q

Infectious consequences of B cell deficiencies

A

pyogenic bacterial infections, enteric bacterial and viral infections

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3
Q

Histopath of T cell deficiencies

A

reduced T cell zones in lymphoid organs, reduced DTH reactions to common ags. defective T cell proliferative responses

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4
Q

Infectious consequences of T cell deficiencies

A

viral and other intracellular microbial infections, some cancers

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5
Q

Histopath of innate immune deficiencies

A

dependent on which component is defective

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6
Q

Infectious consequences of innate immune deficiencies

A

pyogenic bacterial and viral infections

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7
Q

Chediak Higashi syndrome

A

autosomal recessive disorder, failure of phagolysosomal fusion resulting in increased bacterial infections; fatal without a stem cell transplant

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8
Q

Blood smear of Chediak Higashi

A

giant granules, accumulation of organisms

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9
Q

Presentation of Chediak Higashi pts

A

defects in melanocytes leading to albinism or grey streaks in hair

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10
Q

Chronic granulomatous disease

A

GROUP of genetic disorders, failure of superoxide anion production within phagocytes; accumulation of macrophages walls off the infection

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11
Q

Membrane Attack Complex defiiency

A

C5, 6, 7, 8, 9 can be affected; lysis of cell membrane cannot occur; Neisseria infections are common (meningitis)

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12
Q

Hereditary angioedema

A

autosomal dominant deficiency of C1 inhibitor

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13
Q

Presentation of hereditary angioedema

A

headache, dizziness, throat swelling, vomiting, abd pain, bladder and genital swelling, cutaneous edema, cork screw appearance of intestines

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14
Q

Severe Combined Immunodeficiency (SCID)

A

most common deficiency is in both B and T cell lineages due to a defect in T cell function

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15
Q

X-linked SCID

A

males, mutation in IL receptors, T cell reduced and B cells unable to make Ab without T cell help

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16
Q

Autosomal Recessive SCID

A

adenosine deaminase (ADA) deficiency, accumulation of toxic purine metabolites that blocks T cell formation (some B cell influence but lack of Th cells suppresses humoral)

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17
Q

SCID treatments

A

stem cell transplant, gene therapy

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18
Q

DiGeorge syndrome

A

primary T cell deficiency due to failure of pharyngeal pouches 3 and 4 (thymus, parathyroids, heart, great vessels)

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19
Q

DiGeorge manifestations

A

facial and palatal abnormalities, cardiac abn, tetany, immune deficiency (T cell, humoral deficiency)

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20
Q

X-linked agammaglobulinemia

A

Bruton tyrosine kinase gene defect on x-csome; inability of pre-B cells to mature, presents in early to late childhood

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21
Q

Presentation of agammaglobulenemia

A

infection by encapsulated bacteria (S. pneumoniae, H. influenzae B, S. pyogenes, S. aureus, P. aeruginosa), viruses, and protozoa (Giardia lamblia)

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22
Q

Hyper-IgM syndrome

A

encapsulated bacterial infections predominate due to lack of opsonizing antibodies; CD40:CD40L mutations (prevents class switching); High IgM

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23
Q

Hyper-IgM treatment

A

IVIg, stem cell transplant

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24
Q

Common Variable Immunodeficiency (CVID)

A

results in hypogammaglobulinemia; most common primary immunodeficiency; encapsulated bacterial infections

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25
Q

CVID presentation

A

recurrent sinopulmonary infections by pyogenic bacteria due to low IgA, granulomas, chronic diarrhea, autoimmune disease (anemia, thrombocytopenia)

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26
Q

IgA deficiency

A

familial or acquired, less defense of inhaled and ingested pathogens

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27
Q

Presentation of IgA deficiency

A

sinus and resp infections, urinary bladder infections, GI infection, autoimmune dz and allergies, anapylactic rxn against RBC transfusion

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28
Q

Transfusion related anaphylaxis

A

IgA present in transfusion may trigger anaphylactic reaction, red cell washing can prevent reactions

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29
Q

Wiskott Aldrich Syndrome triad

A

thrombocytopenia, eczema, recurrent infections due to T cell loss and hypogammaglobulinemia

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30
Q

Wiskott Aldrich mutation

A

WASP gene

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31
Q

Wiskott Aldrich treatment

A

stem cell transplant

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32
Q

Ataxia Telangiectasia

A

neurodegenerative disease, vascular malformation, immune deficiency (resp. infections, autoimmune dz, CA); autosomal recessive ATM gene mutation

33
Q

Examples of secondary immunodeficiency

A

CA, DM, metabolic dz, chronic illness, treatment-related chemo/rad/immunosupression, AIDS

34
Q

Acquired Immunodeficiency Syndrome

A

manifestation when HIV affects body to degree where immune dysfunction results in opportunistic infections, secondary neoplasms, neuro manifestations

35
Q

Beginnings of AIDS

A

started in 1980s and was mostly seen in homosexual men, blood transfusion recipients, infants/female; presented as infections ,tumors, neuro impairment

36
Q

Who is at risk for HIV?

A

homosexual/bisexual men, IV drug abusers, hemophiliacs, other recipients of blood/blood components, heterosexual contacts, newborns in areas of high female prevalence

37
Q

Most common modes of HIV transmission

A

Male->male, Male->female

38
Q

Parenteral routes of virus transmission

A

IV drugs, transfusions

39
Q

Mother to child virus transmission

A

placental transfer, during delivery with contact of secretions in birth canal, after birth with ingestion of breast milk

40
Q

HIV virus classification

A

Retrovirus, lentivirus family

41
Q

HIV capsid protein

A

p24

42
Q

HIV glycoproteins

A

gp120, gp41

43
Q

HIV viral enzymes

A

protease, reverse transcriptase, integrase

44
Q

HIV LTR gene

A

initiates transcription, binds TFs

45
Q

HIV gag gene

A

encodes for proteins inside the virus (capsid, matrix, nucleocapsid)

46
Q

HIV env gene

A

encodes for surface glycoproteins

47
Q

HIV pol enzymes

A

encodes for viral enzymes (protease, reverse transcriptase, etc)

48
Q

Entry of HIV

A

attaches to CD4 receptor on T cells via gp120, gp41 allows for membrane fusion and inserts viral genome

49
Q

HIV replication

A

Reverse transcriptase synthesizes proviral DS DNA from original RNA; integrase inserts proviral DNA into host genome; when host cell activates, LTR is triggered to transcribe viral RNA

50
Q

Antigenic stimulation of HIV infected cell causes release of what molecule

A

NF-kB, initiating viral transcription (when it should be upregualating T cell response

51
Q

HIV release

A

host cell dies, pyroptosis may occur (inflammasome-mediated)

52
Q

HIV infection of non-T cells

A

macrophages, dendritic cells, microglia

53
Q

B lymphocytes in HIV infection

A

proliferative response (due to secondary infection)m may become clonal (develop into lymphoma), non-specific hypergammaglobulinemia, impaired humoral immunity

54
Q

Pathogenesis of HIV

A

infection of mucosal tissues, death of mucosa memory T cells, infection establishment in lymphoid tissues, spread of infection throughout the body, immune response (triggering partial control of replication), clinical latency

55
Q

First detectable sign of HIV

A

viral RNA (use NAT)

56
Q

Second detectable sign of HIV

A

p24

57
Q

Third detectable sign of HIV

A

antibody to HIV

58
Q

Window period

A

time between infection and seroconversion

59
Q

Acute retroviral syndrome

A

fever, sore throat, muscle aches, self-limited

60
Q

Viral set point

A

end of initial viremic spike, may predict CD4 cell loss and reflects clinical latency

61
Q

Clinical latency

A

period of “silent massacre” of CD4 cells, may last years without symptoms

62
Q

AIDS case definition

A

AIDS-defining conditions (P. jiroveci pneumonia, chronic cryptosporidiosis, invasive cervical cancer, CD4<200 cell/mm3

63
Q

HIV treatment course when detected early

A

HAART initiated, CD4 counts monitored, prophylactic treatment against infections

64
Q

Manifestations of AIDS

A

opportunistic infection (Pneumocystis pneumonia, TB, toxoplasma, C. mucositis) and neoplasia (Kaposi Sarcoma and lymphoma)

65
Q

Pneumocystis jiroveci

A

opportunistic fungal infection, AIDS defining illness, diffuse or focal radiographically

66
Q

Kaposi sarcoma

A

vascular tumor, Human herpesvirus 8, primary effusion lymphoma

67
Q

AIDS related lymphomas

A

opportunistic viral infections may lead to EBV lymphoma, decrease in cellular immunity leads to B cell hyperplasia (burkitt lymphoma)

68
Q

AIDS-defining cancer

A

cervical and anal cancer–viral driven

69
Q

Amyloidosis

A

misfolding of proteins leading to beta-pleated sheets

70
Q

Types of amyloid

A

local, systemic, acquired or hereditary, different chemical properties

71
Q

Where does amyloid tend to manifest?

A

kidney, brain, heart, liver, fat tissue

72
Q

Initial presentation of amyloid in kidney

A

proteinuria and edema

73
Q

Initial presentation of amyloid in heart

A

dysrhythmias

74
Q

Major fibril protein in multiple myeloma (primary amyloidosis)

A

AL

75
Q

Major fibril protein in chronic inflammatory conditions

A

AA

76
Q

Major fibril protein in familial mediterranean fever

A

AA

77
Q

Major fibril protein in familial amyloidotic neuropathies and systemic senile amyloidosis

A

AATR

78
Q

Major fibril protein in medullary carcinoma of thyroid

A

A Cal