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Flashcards in 7. Immunopathology Deck (46):
1

What is type I hypersensitivity and what is another name for it?

AKA Anaphylactic type; it?s hypersensitivity reaction that are characterized by IgE-related release of chemical mediators from mast cells and basophils. The release is triggered by exposure to an antigen but requieres prior sensitization to the antigen. Can be systemic (drugs or bee stings), or localized (food allergies, atopy or asthma).

2

What is type II hypersensitivity and what is another name for it?

AKA Cytotoxic type; it's hypersensitivity reaction that are characterized by production of an IgG or IgM antibody directed against a specific targe cell or tissue.

3

What is type III hypersensitivity and what is another name for it?

AKA Immune complex disease; it's hypersensitivity reaction characterized by the formation of in situ or circulating antibody antigen immune complexes, which deposit in tissue resulting in inflammation and tissue injury.

4

What is type IV hypersensitivity and what is another name for it?

AKA Cell-mediated type, it's hypersensitivity reaction mediated by sensitized T lymphocytes.

5

What is complement-dependent cytotoxicity and give an example.

A type of type II hypersensitivity; it is the fixation of complements that result in osmotic lysis or opsonization of antibody coated cells/ e.i., autoimmune hemolytic anemia.

6

What is antibody-dependent cell mediated cytotoxicity (ADCC) and give an example.

A type of type II hypersensitivity; it is cytotoxic killing of an antibody-coated cell, e.i., pernicious anemia.

7

What is anti-receptor antibody reaction and give an example.

A type of type II hypersensitivity; it is when antibodies activate or interfere with receptors, e.i., Graves disease.

8

What are three examples of type III hypersensitivity?

I. Serum sickness. II. Systemic lupus erythematosus (SLE). III. Glomerulonephritis.

9

What is delayed type hypersensitivity?

A type of type IV hypersensitivity, it is when CD4+ T-cell lymphocyte mediate granuloma formation seen in tuberculosis and PPD skin test.

10

What is cytotoxic T-cell mediated reaction?

A type of type IV hypersensitivity, it is when CD8+ T-cell lymphocytes destroy antigen-containing cells seen in viral infections, immune reaction to tumors, contact dermatitis, and graft rejection.

11

What is systemic lupus erythematosis (SLE)?

Chronic systemic autoimmune disease characterized by loss of self-tolerance and production of autoantibodies.

12

What mechanism of injury does systemic lupus erythematosis inflict on the body?

Type II and III hypersensitivity.

13

What are the clinical signs of systemic lupus erythematosis?

Hematologic (hemolytic anemia, thrombocytopenia, neutropenia, lymphopenia), arthritis (polyarthralgia and synovitis without joint deformity), Skin (malar "butterfly" rash, maculopapuplar rash, ulcerations and bullae formation), Heart (Libman-Sacks endocarditis (nonbacteral verrucous endocarditis), Serosal surface (pericarditis, pleuritis, and pleural effusions), and CNS (focal neurologic symptoms, seizures, and psychosis).

14

According to WHO classification of kidney manifestation of lupus, what is Class I?

Normal.

15

According to WHO classification of kidney manifestation of lupus, what is Class II?

Mesangial lupus nephritis.

16

According to WHO classification of kidney manifestation of lupus, what is Class III?

Focal proliferative glomerulonephritis.

17

According to WHO classification of kidney manifestation of lupus, what is Class IV?

Diffuse proliferative glomerulonephritis (most common and severe).

18

According to WHO classification of kidney manifestation of lupus, what is Class V?

Membranous glomerulonephritis.

19

What is Sjogren syndrome and what is another name for it?

AKA Sicca syndrome, it is an autoimmune disease characterized by destruction of the lacrimal and salivary glands resulting in the inability to produce saliva and tears.

20

What are the clinical signs of Sjogren syndrome?

Keratoconjuctivitis sicca (dry eyes) and corneal ulcers, xerostomia (dry mouth), and Mikulicz syndrome (enlargement of the salivary and lagrimal glands).

21

What other diseases are associated with Sjogren syndrome?

Rheumatoid arthritis and other autoimmune diseases.

22

What antibodies are associated with systemic lupus erythematosis?

Antinuclear antibody (>95%), Anti-dsDNA(40-60%), Anti-Sm (20-30%).

23

What complication is assocaited with Sjogren syndrome?

Increased risk of developing lymphoma.

24

What is scleroderma and what is another name for it?

AKA Progressive systemic sclerosis, it is an autoimmune disease characterized by fibroblasts stumilation and deposition of collagen in the skin and internal organs. Activation of fibroblast by cytokines interleukin 1 (IL-1), platelet-derived growth factor (PDGF), and/or fibroblast growth factor (FGF) leads to fibrosis.

25

What are the clinical signs of scleroderma?

Can be diffuse (widespread skin involvement and early involvement of the visceral organs) or localized (CREST syndrome).

26

What is CREST syndrome?

Localized presentation of scleroderma; Calcinosis, Raynoud phenomenon, Esophageal dysmotility, Sclerodactyly, Telangiectasia.

27

What is X-linked agammaglobulinemia of Brunton and causes it?

Inherited primary immunodeficiency syndrome characterized by a developmental failure to produce mature B cells and plasma cells, resulting in agammablobulinemia. Caused by mutation of B-cell Brunton tyrosine kinase (btk).

28

What are the clinical symptoms of X-linked agammaglobulinemia of Brunton?

Male infants who suffer repeated bouts of infection starting at 6 months of age. They have repeated episodes of pharyngitis, otitis media, bronchitis, and pneumonia. Organisms for these repeated infections are most commonly H. Influenza, S. pneumococcus, and S. aureus.

29

What is Common variable immunodeficiency?

A primary immune deficiency syndrome, characterized by a group of disorders characterzied by a B-cell maturation defect and hypogammaglobulinemia.

30

What are the clinical signs of common variable immunodeficiency?

Both sexes are affected, onset is in childhood, recurrent bacterial infections and increased susceptibility to Giardia lamblia.

31

What is DiGeorge syndrome?

A primary immune deficiency syndrome, characterized by embryological failure to develop the 3rd and 4th pharyngeal pouches, resulting in the absence of the parathyroid glands and thymus.

32

What are the clinical signs of DiGeorge syndrome?

Hypocalcimia and tetany, T-cell deficiency, and recurrent infections with viral and fungal organisms.

33

What is Severe combined immunodeficiency (SCID)?

A primary immune deficiency syndrome, it is characterized by a combined deficiency of cell-mediated and humoral immunity often caused by a stem-cell defect.

34

What are the clinical symptoms of Severe combined immunodeficiency (SCID)?

Adverse reactions to live virus immunizations, recurrent infections with bacteria, fungi, viruses, and protozoa. Susceptible to candida, cytomegalovirus (CMV), and Pneumocystis carinii infections.

35

What is Wiskott-Aldrich syndrome and what causes it?

A primary immune deficiency syndrome, it is characterized by recurrent infections, severe thrombocytopenia, and eczema. It is an X-linked recessive inheritance; mutation in the gene for Wiskott-Aldrich syndrome protein (WASP).

36

What is the treatment for Wiskott-Aldrich?

Bone morrow transplant.

37

What is the major complication in long-term cardiac transplant patients?

Accelerated graft arteriosclerosis.

38

What is hyperacute rejection?

Occurs immediately after transplantation, mediated by preformed antibodies. Micro: neutrophilic vasculitis with thrombosis.

39

What is acute rejection?

Occurs weeks or months after organ transplantation, characterized by abrupt onset of oliguria and azotemia. Micro: neutrophilic vasculitis and interstitial lymphocytes. Treated with increased doses of immunosuppressive drugs.

40

What is chronic rejection?

Occurs months or years after organ transplant, characterized by gradual onset of oliguria, hypertension (HTN), and azotemia. Micro: intimal fibrosis of vessels and interstitial lymphocytes. Poor response to treatment.

41

What is Acquired immunodeficiency syndrome (AIDS)?

HIV positive and CD4 count

42

Describe the human immunodeficiency virus (HIV).

Enveloped RNA retrovirus with reverse transcriptase, it infects CD4+ cells (macrophages, lymph node follicular dendritic cells, Langerhans cells and CD4+ T-cell lymphocytes). They bind to CD4 by gp120; entry into the cell by fusion requires gp41 and coreceptors CCR5 (beta-chemokine receptor 5), and CXCR4 (alpha-chemokine receptor).

43

What is the treatment for AIDS?

Reverse transcriptase inhibitors, protease inhibitors, prophilaxis for opportunistic infections based on CD4 count.

44

What are the reservoirs for HIV in the human body?

Macrophages and follicurla dendritic cells.

45

What is antiribonucleoprotein antibodies SS-A and SS-B associated with?

Sjogren syndrome.

46

What is anti-DNA topoisomerase I antibodies (Scl-70) associated with?

Scleroderma.