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Diseases Flashcards

(51 cards)

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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

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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

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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

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A

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

classic triad- mucocutaneous candidiasis, hypoparathyroidism and adrenal failure

24
Q

Guillain Barre syndrome

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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
Corticosteroids- 2 immune defic
depletes CD4 cells, decreases eosinophils and basophils inhibit T and B cell maturation and cytokine synthesis
26
cycolosporin immune defic
inhibits IL2 dependent signaling
27
HIV
depletion of CD4 helper T cells
28
irradiation and chemotherapy treatments
acquired immunodefi decreased bone marrow precursors for all leukocytes
29
immunosuppresion for graft rejection and inflamm disease
acquired immunodefic depletion or functional impairment of lymphocytes
30
Removal of spleen
acquired immunodefic decreased phagocytosis of microbes (encapsulated)
31
B cell deficiencies
absent or reduced follicles and germinal centers in lymphoic organs reduced serum Ig levels common pyogenic bacterial infections., enteric bacterial and viral infections
32
T cell deficiencies
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
X linked SCID
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
ADA/RD SCID
T-B-NK- ADA deficiency leads to accumulation of toxic metabolities in lymphocytes-> progressive decrease in T and B cells
35
X linked agammaglobulinemia (Brutons)
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
Ig heavy chain deficiencies
chromosomal deletion involving Ig heavy chain locus and 14q32-> deficiency of IgG subclasses sometiems associated with absent IgA or IgE
37
DiGeorge Syndrome
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
Bare lymphocyte syndrome
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
defects in TCR complex expression or signaling
muations of deletion in genes encoding CD3 proteins (ZAP-70)-> decreased T cells or abnormal CD4 and CD8 subsets- decreased cell mediated immunity
40
defects in Th1 differentiation
mutations in receptors for IL12 if INFg-> decreased T cell emdiated macrophage activation; susceptibility to infection
41
defects in Th17 differentiation
mtations in genes encoding STAT3, IL17 and IL17R-> decreased T cell mediated inflamm response mucocutanoeus candidiasis and bacterial skin abscesses
42
X linked lymphoproliferative syndrome (Duncan syndrome)
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
SCID T-B-NK+
AR; RAG1 and 2 defic-> defects in V(D)J recombination-> granuloma formation
44
Common Variable Immunodeficiency
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
Leukocyte Adhesion Deficiency
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
Chediak Higashi Syndrome
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
Chronic Granulomatous Disease
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
multiple sclerosis
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
hashimoto's thyroiditis
type 4 hypersensitity target is thyroid antigens goiter can form hypothyroidism
50
insulin dependent DM
type 4 hypersens abs agaisnt beta/islet cells that produce insulin HLA DR3/4
51
rheumatoid arthritis
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

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