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Kaplan: Pathology > Immunopathology > Flashcards

Immunopathology Flashcards

1
Q

Type 1 HSR characteristics.

A

anaphylctic type are characterized by IgE-dependent release of chemical mediators from mast cells and basophils.

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2
Q

Type 2 HSR characteristics?

A

antibody mediated are mediated by IgG or IgM ab directed against a specific target cell or tissue

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3
Q

Type 3 HSR characteristics?

A

immune complex disease characterized by formation of in situ or circulating ab-ag immune complexes, which deposit in tissue resulting in inflammation and tissue injury

Examples: serum sickness, SLE, and glomerulonephritis

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4
Q

Type IV HSR characteristics?

A

cell mediated type are mediated by sensitized T lymphocytes in. in delayed type hypersensitivity, CD4+ TH1 lymphocytes mediate granuloma formation examples include PPD skin test and TB

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5
Q

Differentiate the HSR of SLE in hematologic rxns vs skin manifestations.

A

hematologic type II HSR
Skin manifestations Type III HSR

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6
Q

What are some autoabtiboies that may be detected in sera from those with lupus?

A

antinuclear ab (ANA)
anti-dsDNA
anti-Sm
antihistone
nonhistone nuclear RNA proteins
blood cells

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7
Q

Lupus treatment?

A

steroids and immunsuppressive agents

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8
Q

What is Sjogren syndrome?

A

sicca syndrome an autoimmune disease characterized by destruction of the lacrimal and salivary glands resulting in the inability to produce saliva and tears

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9
Q

What are some ab implicated in Sjogren syndrome?

A

SS-A (Ro)
SS-B (La

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10
Q

What is scleroderma?

A

progressive systemic sclerosis and is an autoimmune edisease characterized by fibroblast stimulation and deposition of collagen in the skin and internal organs

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11
Q

Pathogensis of scleroderma?

A

Activation of fibroblasts by cytokines interleukin 1 (IL-1), platelet derived growth factor (PDGF), and/or fibroblast growth factor (FGF) with resulting activated fibroblasts causing fibrosis

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12
Q

What is diffuse scleroderma?

A

has anti-DNA topoisomerase I ab (Scl-70) widespread skin involvement, and early involvement of the visceral organs

organs that can be affected inclue the esophagus (dysphagia), Gi tract (malabsoprtion), lungs (pulmonary fibrosis), heart, and kidney issues

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13
Q

What is localized scleroderma?

A

CREST syndrome
calcinosis
raynaud phenomenon
esophageal dysmotility
sclerodacyly
telangiectasia

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14
Q

What is mixed connective tissue disease?

A

overlap condition with features of SLE, systemic sclerosis, and polymyositis

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15
Q

What ab are nearly aloways positive in mixed connective tissue disease?

A

antiribonucleoprotein ab

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16
Q

What ab can be found in localized scleroderma?

A

anti-centromere ab

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17
Q

X linked agammaglobulinemia of Bruton?

A

immunodeficiency characterized by a developmental failure to produce mature B cells and plasma cells resulting in agammaglobulinemia

occurs because of loss of function mutations of B cell bruton tyrosine kinsase

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18
Q

C/F of X linked agammaglobulinemia? Common infections?

A

affects male infants at 6 months of life due to loss passive maternal immunity

common infections include pharyngitis, otitis media, bronchitis, and pneumonia

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19
Q

What is common variable immunodeficiency?

A

Group of disorders characterized by defects in B cell maturation that can lead to defective IgA or IgG prodution

20
Q

C/F of common variable immunodeficiency?

A

recurrent bacterial infections and increased susceptibility to giardia lamblia

complications include increase frequency of developing autoimmune disease, Non-Hodgkin lymphoma, and gastric cancer

21
Q

What is DiGeorge syndrome?

A

an embryologic failure to develop the 3rd and 4th pharyngeal pouch, resulting in the absence of the PTH glands and thymus

22
Q

C/F of DiGeorge syndrome?

A

neonatal hypocalcemia and tetany,
T-cell deficiency,
recurrent infections with viral and fungal organisms

23
Q

What is SCID?

A

combined deficiency of cell-mediated and humoral immunity that is often caused by a progenitor cell defect

Modes of inheritance are variable and can include X-linked (mutation of common (gamma) chain or the IL receptors IL-2, IL-4, IL-7, IL-9, IL-15, and IL-21 and AR (deficiency of adenosine deaminase

24
Q

Clinical features of SCID?

A

recurrent infections with bacteria, fungi, viruses and protozoa; susceptibility to Candida, cytomegalovirus (CMV) and pneumocystitis jiroveci infections and adverse rxns to live virus immunizations.

25
Q

What is Wiskott-Aldrich Syndrome?

A

an X linked recessive disease with mutation in gene for WASP wiskott-aldrich syndrome protein

26
Q

Clinical signs of Wiskott Aldrich syndrome? Complications?

A

clinical triad of recurrent infections, severe thrombocytopenia, and eczema (chronic spongiform dermatitis)

increased risk of non-Hodgkin lymphoma and death due to infection or hemorrhage

27
Q

Treatment for Wiskott Aldrich syndrome?

A

hematopoietic stem cell transplantation

28
Q

What are complement system disorders?

A

involve a variety of factors, with deficiencies of different factors producing different clinical patterns

29
Q

In both classical and alternate pathways, C3 deficiency leads the patient to be susceptible to what disease/infections?

A

recurrent bacdterial infections and immune complex disease

30
Q

C5, C6, C7, and C8 deficiencies cause a patient to become susceptible to what conditions?

A

recurrent meningococcal and gonococcal infections

31
Q

In the classical pathway only, C1q, C1r, C1s, C2, and C4 deficiencies leave a person susceptible to what?

A

marked increases in immune complex diseases, including infections with pyogenic

32
Q

In the alternate pathway, Factor B and properdin deficiencies cause a person to become susceptible to what?

A

increased neisserial infections

33
Q

MHC class II deficiency can be characterized by what?

A

defects in positive selection of thymocytes

34
Q

How are CD4+ T cells and CD8+ lymphocytes affected in patients with MHC class II deficiency?

A

few CD4+ with normal CD8+ T cells

35
Q

What mutations are associated with MHC II defienciency?

A

mutations in genes CIITA that encode proteins that regulate MHC class II gene expression

36
Q

What is hyper IgM syndrome characterized by?

A

normal B and T lymphocyte numbers and normal to elevate Ig levels but significantly decreased IgA, IgG and IgE levels

37
Q

What are the mutations associated with hyper IgM syndrome?

A

mutations in gene for CD40 ligand result in most common form of X-linked hyper IgM syndrome

38
Q

Selective IgA deficiency is characterized by a strong susceptibility to what conditions?

A

sinopulmonary infections, diarrhea, and adverse reactions to transfusions can occur

39
Q

Be able to draw out the diagram on primary immune deficiency syndromes and where each condition is implicated in affecting

A
40
Q

How do you diagnose HIV?

A

CDC recommends initial testing with an ag/ab combination immunoassay, followed by a confirmatory HIV-1/HIV-2 ab differentiation immunoassay

If confirmatory test is negative, testing with an HIV-1 nucleic acid test is done

41
Q

What is the acute phase of HIV infection characterized by?

A

viremia with a reduction in CD4 count, mononucleosis-like viral symptoms and lymphadenopathy and seroconversion

42
Q

What is the latent phase of HIV infection characterized by?

A

asymptomatic or persistent generalized lymphadenopathy with continued viral replication in the lymph nodes and spleen,

low level of virus in the blood,

and minor opportunistic infections including oral thrush (candidiasis) and herpes zoster

43
Q

What is transplant rejection largely caused by?

A

differences in HLA alleles between donor and recipient

44
Q

Hyperacute rejection.

A

occurs within minutes to hours due to preformed ab in the recipient

lymphocyte cross-matching has almost eliminated this problem

45
Q

Acute rejection.

A

occurs in first 6 months and may be cellular (CD8+ T lymphocytes kill graft cells) or ab-mediated

46
Q

Chronic rejection.

A

occurs after months or years and may be cell-mediated or ab mediated. Vasculature components are targeted, and histopathologic changes depend on the organ involved

Kaplan: Pathology (10 decks)

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