Diseases Flashcards

1
Q

Type 2 Hypersensitivty Cytotoxic diseases

A

Autoimmune Hemolytic Anemia

Acute Rheumatic Fever

Goodpasture Syndrome

Transfusion Reaction

Autoimmune Purpura

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2
Q

Type 2 Hypersensitivty noncytotoxic diseases

A

Myasthenia gravis

graves disease

type 2 diabetes

pernicious anemia

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3
Q

Transfusion Reactions

A

Type 2 Hypersensitivty cytotoxic

Ab attaches to RBC and initiates the complement system to lyse RBC

hemoglobin detected in plasma starts to filter through kidneys and found in urine- hemoglobinuria

hemoglobin-> bilirubin-> toxic levels-> fever, chills, blood clotting

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4
Q

Erythroblastosis fetalis

A

type 2 hypersens cytotoxic

Rh+ fetus, Rh- mother

IgG antibodies cross placenta- may be anti-rh

RhoGAM shot prevents B cell activation and memory cell formation

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5
Q

Grave’s disease

A

type 2 hypersen noncyto

stimulating abs are directed against the TSH receptor

over reactive thyroid followed by hypothyroid

HLA B8

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6
Q

Myasthenia Gravis

A

type 2 hypersens noncyto

ab inhibits ach binding, downmodulates receptors- against Ach receptor

muscle weakness, paralysis

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7
Q

Autoimmunie hemolytic anemia

A

type 2 hypersens cytotox

opsonization, phagocytosis and complement mediated destruction of RBCs- against RBC membrane proteins

hemolysis, anemia

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8
Q

Goodpasture Syndrome

A

type 2 hypersens cytotox

complement and Fc receptor mediated inflammation against type 4 collagen in basement membranes of kidney glomeruli and lung aveoli

nephritic, lung hemorrhage, linear Ab deposits

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9
Q

Acute rheumatic fever

A

type 2 hypersens cytotox

inflammation and macrophage activation against streptococcal cell wall ag- ab cross reacts with myocardial ag

myocarditis, arthritis

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10
Q

type 2 diabetes

A

type 2 hypersen noncyto

ab inhibits binding of insulin against insulin receptor

hyperglycemia

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11
Q

Pernicious anemia

A

type 2 hypersend noncyto

neutralization of IF, decreased absorption of vit B12- against intrinsic factor of gastric parietal cells

abnormal erythropoiesis, anemia

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12
Q

Serum sickness

A

Type 3 hypersen generalized

response to foreign protein in serum-> deposition of immune complexes systemically-> fever, vasculitis, arthritis, nephritis

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13
Q

Type 3 hypersensitivty diseases

A

systemic lupus erythematosus

polyarteritis nodosa

poststreptococcal glomerulonephrtis

serum sickness

arthus reaction

Rheumatoid arthritis

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14
Q

systemic lupus erythematosus

A

typer 3 hypersen generalized autoimmune disease

Ab- DNA, nucleoproteins

nephritis, arthritis, vasculitis

HLA DR2/DR3

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15
Q

polyarteritis nodosa

A

type 3 hypersens gen

ab- microbial antigens (hep B)

nephritis

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16
Q

Poststreptococcal glomerulonephritis

A

Type 3 hypersens

ab- streptococcal cell wall antigen

nephritis

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17
Q

contact dermatitis

A

type 4 hypersens

Tcells against modified skin proteins

DTH reaction in skin, rash

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18
Q

Type 4 hypersensitivity diseases

A

MS

rheumatoid arthritis

type 1 diabetes

crohns disease

contact sensitivity

chronic infections

viral hepatitis (B and C)

superantigen mediated diseases- TSS

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19
Q

X linked Hyper M syndrome

A

Th cells dont express CD40L- defective class switching-> only produce IgM

no memory cell population nor germinal centers, high IgM

recurrent bacterial infections with mostly encapsulated bacteria

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20
Q

Wiskott Aldrich Syndrome

A

x linked mutation in WAS gene- assoc with cellular cytoskeleton

necessary for T cell cytockeleton maintenance, T cells can’t properly react to APCs, platelets and leukocytes are small and fail to migrate; elevated IgE and IgA

eczema, reduced platelets, immunodefic

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21
Q

Ataxia- telangiectasia

A

mutation in DNA repair genes (ATM gene, repeairs ds DNA breaks during nonhomologous end joining) during recombination and defective lymphocyte maturation

gait abnormalities, vascular malformations, immunodefic

22
Q

Autoimmune lymphoproliferative Syndrome

A

inherited genetic defect of FAS pathway- signaling protein for lymphocyte apoptosis

leads to expansion of lymphocytes and autoimmunity; has a role in suppression of malignant transformation-> lymphoma

Cytopenia, eosinophilia, monocytosis, increased IL10, B12 and soluble FAS ligand

23
Q

Autoimmune polyendocrine syndrom type 1

A

mutation in AIRE gene, essential in self tolerance/ autoimmune regulator

classic triad- mucocutaneous candidiasis, hypoparathyroidism and adrenal failure

24
Q

Guillain Barre syndrome

A

inflammatory neuropathy due to cross reactivity between neural antigens and abs that is induced by specific infections- C. jejuni, influenza, cytomealovirus, EBV, mycoplasma pneumonia and HIV- express lipooligosaccharides in bacterial wall similar to gangliosides-> antiganglioside antibodies that attack nerves

increased protein level with normal WBC count in CSF and slowed nerve conduction

25
Q

Corticosteroids- 2 immune defic

A

depletes CD4 cells,

decreases eosinophils and basophils

inhibit T and B cell maturation and cytokine synthesis

26
Q

cycolosporin immune defic

A

inhibits IL2 dependent signaling

27
Q

HIV

A

depletion of CD4 helper T cells

28
Q

irradiation and chemotherapy treatments

A

acquired immunodefi

decreased bone marrow precursors for all leukocytes

29
Q

immunosuppresion for graft rejection and inflamm disease

A

acquired immunodefic

depletion or functional impairment of lymphocytes

30
Q

Removal of spleen

A

acquired immunodefic

decreased phagocytosis of microbes (encapsulated)

31
Q

B cell deficiencies

A

absent or reduced follicles and germinal centers in lymphoic organs

reduced serum Ig levels

common pyogenic bacterial infections., enteric bacterial and viral infections

32
Q

T cell deficiencies

A

may be reduced T cell zones in lymphoid organs

reduced DTH reactions to common antigens

defective T cell proliferative responses to mitogens in vitro

common viral and other intracellular microbial infections- pneumocystis jiroveci and virus associated malignancies (EBV)

33
Q

X linked SCID

A

markedly decreased T cells, normal/increased B cells, reduced serum Ig

caused by mutation in the common gamma chain subunit of several cytokine receptors

decreased IL7

34
Q

ADA/RD SCID

A

T-B-NK-

ADA deficiency leads to accumulation of toxic metabolities in lymphocytes-> progressive decrease in T and B cells

35
Q

X linked agammaglobulinemia (Brutons)

A

block in maturation beyond pre-B cells because of BTK mutation-> decrease in all serum Ig isotypes, reduced mature B cell numbers and underdeveloped germinal centers of lymph nodes

36
Q

Ig heavy chain deficiencies

A

chromosomal deletion involving Ig heavy chain locus and 14q32-> deficiency of IgG subclasses sometiems associated with absent IgA or IgE

37
Q

DiGeorge Syndrome

A

anamolous development of 3rd and 4th brachial pouches leading to thymic hypoplasia-> decreased T cells, normal B cells; normal or decreased serum Ig

failure to respond to intracellular pathogens, facial abnormalities, congenital heart disease, repeated infections with intracellular bacteria, viruses and fungi, hypoparathyroidism (low calcium)

38
Q

Bare lymphocyte syndrome

A

mutations in genes encoding transcription factors required for MHC 2 gene expression-> lack of MHC2 epxression and impaired CD4 T cell activation; defective cell mediated and humoral immunity

39
Q

defects in TCR complex expression or signaling

A

muations of deletion in genes encoding CD3 proteins (ZAP-70)-> decreased T cells or abnormal CD4 and CD8 subsets- decreased cell mediated immunity

40
Q

defects in Th1 differentiation

A

mutations in receptors for IL12 if INFg-> decreased T cell emdiated macrophage activation; susceptibility to infection

41
Q

defects in Th17 differentiation

A

mtations in genes encoding STAT3, IL17 and IL17R-> decreased T cell mediated inflamm response

mucocutanoeus candidiasis and bacterial skin abscesses

42
Q

X linked lymphoproliferative syndrome (Duncan syndrome)

A

mutations in gene encoding SAP- adaptor protein involved in signaling lymphocytes-> uncontrolled EBV induced B cell proliferation and CTL activation; defective NK cell and CTL function and ab responses

43
Q

SCID T-B-NK+

A

AR; RAG1 and 2 defic-> defects in V(D)J recombination-> granuloma formation

44
Q

Common Variable Immunodeficiency

A

defective B cell maturation-> loss of plama cells and antibodies

affects everyone, sporadic, no fam history, later onset

normal B cell count, absent antibodies, usually IgG, sometimes IgA and IgM

45
Q

Leukocyte Adhesion Deficiency

A

defective neutrophil/lymphocyte migration

most common: type 1, AR defect in CD18, forms beta subunit of several integrins, WBCs can’t roll, migrate

delayed separation of umbilical cord, recurrent bacterial infections, neutrophilia

46
Q

Chediak Higashi Syndrome

A

failure of lysosomes to fuse with phagosomes

mutation in LYST gene- causes microtubule dysfunction

recurrent bacterial infections- strp andstaph

oculocutaneous albinism- fair skin, blonde hair, light blue eyes

47
Q

Chronic Granulomatous Disease

A

loss of function of NADPH oxidase

phagocytes use NADPH oxidase to generate H2O2 from oxygen

catalase (+) bacteria-> no H2O2 to use-> recurrent infections0 staph aureus, pseudomonas serratia, nocardia, aspergillus

48
Q

multiple sclerosis

A

type 4 hypersens

numbness, paralysis, vision loss

inflamm lesions in myelin sheath caused by T cells

freq in african americans, norther hemisphere (north of 37th parallel), envirmental component and genetic

HLA DR2

49
Q

hashimoto’s thyroiditis

A

type 4 hypersensitity

target is thyroid antigens

goiter can form

hypothyroidism

50
Q

insulin dependent DM

A

type 4 hypersens

abs agaisnt beta/islet cells that produce insulin

HLA DR3/4

51
Q

rheumatoid arthritis

A

type 4 hypersens

chronis inflammation of joints

produce auto-abs that bind Fc portion of IgG circulating in blood that creates immune complexes

HLA DR4