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169 Cards in this Set

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What are the 4 signs of reversible cell injury?
1. Swelling of cell organelles and entire cell 2. dissociation of ribosomes from endoplasmic reticulum 3. decreased energy production by mitochondria 4. increased glycolysis -> decreased pH -> nuclear chromatin clumping
What are the 4 signs of irreversible cell enjury
1. dense bodies within mitochondria 2. release of cellular enzymes 3. nuclear degeneration 4. cell death
Hereditary thrombophilia can be caused by a deficiency in what?
Antithrombotic proteins: antithrombin III, protein C, and protein S
What is Leber Hereditary Optic Neuropathy?
Bilateral loss of vision (between ages 15-35), transmitted through mitochondrial DNA (from mother)
What is Tay Sach's disease?
Lysosomal storage disease (autosomal recessive, high prevalence for Ashkenazi Jews), deficiency of hexoaminidase A, associated with developmental delays/neurologic deficits, "cherry red macula"
What disease is characterized by multiple tandem repeats of CGG in DNA?
Fragile X Syndrome
increase in size of cells
increase in number of cells
failure of cell production
absence of an organ due to failure of production
decrease in cell production that is less extreme than that found in aplasia
decreasein mass of pre-existing cells
replacement of one differentiated tissue by another
associated with squamous metaplasia
chronic irritation (tobacco use), vitamin A deficiency [this is usually reversible]
obstruction of arterial blood flow
reduction in number of oxygen-carrying red blood cells
early stage of hypoxic cell injury
affects mitochondria resulting in decreased oxidative phosphylation and ATP synthesis
consequences of decreased ATP availability (in hypoxic cell injury)
1. failure of the cell membrane pump 2. disaggregation of ribosomes and failure of protein synthesis 3. stimulation of phosphofructokinase activity
What does failure of the cell membrane pump lead to?
failure of cell membrane pump (ouabain-sensitive sodium potassium pump) (leads to increased intracellular sodium and water, decreased intracellular potassium), causes cellular swelling, endoplasmic reticulum swelling, and mitochondrial swelling
late stage of hypoxic cell injury
membrane damage with two reversible morphologic signs: myelin figures and cell blebs
What happens with hypoxic cell death
The point of no return is marked by irreversible damage to cell membranes, leading to massive calcium influx, extensive calcification of the mitochondria, and cell death (also intracellular enzymes and various other proteins are released)
Describe how vulnerable various cells are to hypoxic injury
3-5 minutes for neurons, 1-2 hours for myocardial cells and hepatocytes, many hours for skeletal muscle cells
How are free radicals generated?
1. normal metabolism 2. oxygen toxicity 3. ionizing radiation 4. ultraviolet light 5. drugs and chemicals 6. reperfusion after ischemic injury
How are free radicals degraded?
1. intracellular enzymes (glutathione peroxidase, catalase, superoxide dismutase) 2. exogenous and endogenous antioxidants (vit A, C, E, cysteine, glutathione, selenium, ceruloplasmin, or transferrin) 3. spontaneous decay
Types of necrosis
1. coagulative necrosis 2. liquefactive necrosis 3. caseous necrosis 4. gangenous necrosis 5. fibrinoid necrosis 6. fat necrosis
chromatin clumping and shrinking with increased basophilia
fragmentation of chromatin
fading of chromatin material
gene regulators of apoptosis
bcl-2 (gene product inhibits apoptosis), bax (facilitates apoptosis), p53 (facilitates apoptosis by decreasing transcription of bcl-2 and increasing transcription of bax)
What endogenous pigments can accumulate?
melanin, bilirubin, hemosiderin, lipofuscin
How does melanin accumulate?
Formed from tyrosine from tyrosinase, synthasized in melanosomes, transferred to keratinocytes. Associated with suntanning and various diseases. Decreased pigmentation observed in albinism and vitiligo
How does bilirubin accumulate?
It is a catabolic product of hemoglobin (and myoglobin), accumulates from hemolytic jaundice (destruction of red cells) and hepatocellular jaundice (parenchymal liver damage), and obstructive jaundice (intra or extrahepatic obstruction of the biliary tract)
How does hemosiderin accumulate?
It is an iron-containing pigment that consists of aggregates of ferritin. Stained with Prussian blue dye. Hemosiderosis is accumulation of hemosiderin within tissue macrophages. Hemochromatosis is extensive accumulation occurs from Heriditary hemochromatosis and Secondary hemochromatosis
What is hereditary hemochromatosis?
Usually caused by a mutation of the Hfe gene on chromosome 6. Results in micronodular cirrhosis, diabetes mellitus, and skin pigmentation (referred to as "bronze diabetes"). Laboratory abnormalities: increased serum iron and decreased total iron-binding capacity (TIBC)
What is secondary hemochromatosis?
Most often caused by multiple blood transfusions adminstered to subjects \with hereditary hemolytic anemias.
How does lipofuscin accumulate?
End product of membrane lipid peroxidation (called "wear-and-tear" pigment), is a yellowish, fat-soluble pigment, accumulates in elderly patients
What are the two types of pathologic calcifications?
Metastatic calcification (occurs from hypercalcemia) and dystrophic calcification (occurs in previously damaged tissue, normal serum calcium levels)
What are disorders characterized by abnormalities of protein folding?
They involve failure of protein structural stabilization or degradation proteins known as chaperones
important chaperone which marks abnormal proteins for degradation
Abnormal protein aggregation
characteristic of amyloidosis (occurs in a number of neurodegenerative diseases: Alzheimer's, Huntington's, and Parkinson's)
Abnormal protein transport and secretion
characteristic of cystic fibrosis and alpha1-antitrypsin deficiency
5 cardinal signs of inflammation
rubor, dolor, calor, tumor, functio laesa
Dolor is mediated by?
Increased pressure exerted by the accumulation of interstitial fluid and mediators such as bradykinin
Bradykinin mediates?
increased vascular permeability and pain
C3a and C5a cause
degranulation of mast cells
What assists opsonization
IgG and C3b
Histamine mediates
endothelial cell contraction (increasing vascular permeability)
labile cells
cells that actively divide throughout life to replace lost cells (cells of epidermis and GI)
stable cells
cells that undergo few divisions but are capable of division when activited (includes hepatocytes, smooth muscle, connective tissue, etc.)
permanent cells
cells that are inapable of division and regeneration (neurons and myocardial cells), replaced by scar tissue after irreversible injury and cell loss
nodular collections of specialized macrophages referred to as epitheliod cells
diffuse hemorrhage (usually in skin and subcutaneous tissue)
localized increase in the volume of blood in capillaries and small vessels
active hyperemia
localized ateriolar dilation (ex. Blushing, inflammation)
chronic passive congestion of the lung
most often caused by left heart failure or mitral stenosis,congestion and distention of alveolar capillaries lelad to capillary rupture and passage of red cells into the alveoli, phagocytosis and degration of red blood cells result in intra-alveolar hemosiderin-laden macrophages called heart-failure cells, long standing congestion, fibrosis of interstitium and hemosiderin deposition result in brown induration of the lung
chronic passive congestion of the liver and lower extremeties
associated with right heart failure (nutmeg liver may appear)
necrosis resulting from ischemia
anemic infarcts
white or pale infarcts usually caused by arterial occlusions in heart, spleen, and kidney
hemorrhagic infarcts
red infarcts, usually in the lung and GI
intravascular coagulation of blood
platelets participate in endothelial repair through:
platelet-derived growth factor (PDGF)
platelets promote the coagulation cascade through
the platelet phospholipid complex
von Willebrand factor
mediates interaction between platelet surface glycoprotein receptor and subendothelial collagen
platelets release what after adhesion
ADP, histamine, serotonin, PDGF
TxA2 is
a potent vasoconstrictor and platelet aggregant (made by platelets)
inhibits further platelet aggregation (made by endothelial cells)
intact endothelial cells oppose coagulation by
produce heparin-like molecules, antithrombin III, tissue plasminogen activator, degrade ADP, inactivate thrombin, make protein C and S, make PGI2, make NO
extrinsic pathway of coagulation
initiate by tissue factor: factor VII works on IX and X which work on prothrombin which works on fibrinogen (measured by PT time)
intrinsic pathway
initiated by contact activation: XII -> XI -> IX -> X -> …
chromosome number that is a more than a multiple of 2 of the haploid number, usually results in spontaneous abortion
Down syndrome
trisomy 21 accounts for 95% of cases, 5% of cases from Robertsonian translocation, mental retardation, epicanthral folds, acute lymphoblastic leukemia, susceptibility to infection, ~100% incidence of Alzheimer's by age 35, congenital heart disease
Edwards syndrome
trisomy 18, mental retardation, micrognathia (small lower jaw), rocker bottom feet, congenital heart disease
Patau's syndrome
trisomy 13, mental retardation, microcephaly, cleft lip and palate, fused central face, congenital heart disease
Cri du Chat
5p-, high-pitched cry, mental retardation, heart defects
Angelman's syndrome
15q-, maternal deletion, stiff ataxic gait with jerky movements, inappropriate laughter, may be two copies of paternal 15 chromosome
Prader-Willi syndrome
15q-, paternal deletion, mental retardation, short stature and obesity (with uncontrolled appetite), may be two copies of maternal 15 chromosome
Klinefelter syndrome
47, XXY, male hypogonadism, always male phenotype, decreased testosterone, increase FSH, LH, estradiol, tallness, gynecomastia, female distribution of hair
Turner's syndrome
45, X, female hypogonadism, most common cause of primary amenorrhea, no Barr bodies seen on buccal smear, usually no retardation, ovaries are replaced by fibrous streaks, short stature/webbed neck, coarctation of aorta coommon
XYY syndrome
occurs with increased frequency among criminals demonstrating violent behavior
XXX syndrome
usually clinically silent, menstrual irregularities/mild retardation
Fragile X syndrome
CGG tandem repeats, 2nd most important cause of hereditary mental retardation (2nd to Down's), macro-orchidism
Huntington's disease
CAG repeats
Myotonic dystrophy
GCT repeats
Spinal-bulbar muscular atrophy
CAG repeats
Adult polycystic kidney disease
autosomal dominant, clinically manifests between 30-50 years of age, numerous bilateral cysts
familial hypercholesterolemia
autosomal dominant, defect in LDL receptors, early onset of athersclerosis, xanthomas (raised yellow lesions filled with lipid-laden macrophages in the skin and tendons)
hereditary hemorrhagic telangiectasia
autosomal dominant, telangiectases of the skin and mucous membranes, recurrent hemorrhages in thos lesions
hereditary spherocytosis
autosomal dominant, spheroidal erythrocytes, sequestered and destroyed in spleen, producing hemolytic anemia
Marfan syndrome
autosomal dominant, defect of connective tissue (deficiency of fibrillin), tall/thin patients with spiderlike fingers (arachnodactyly), dislocation of ocular lens (ectopia lentis), cystic medial necrosis leading to aortic dilation with aneurysm of the proximal aorta, and dissecting anneurysm of the aorta, loss of connective tissue may lead to mitral valve prolapse
autosomal dominant, multiple neurofibromas of the skin, schwannomas of the 8th nerve, café au lait spots, pigmented iris hamartomas (Lisch nodules), marked by skeletal disorders (such as scoliosis) and increased incidence of other tumors
tuberous sclerosis
autosomal dominant, presence of glial nodules and distorted neurons in the cerebral cortex, marked by seizures, mental retardation, associated with renal angiomyolipomas (lesions consisting of malformed blood vessels, smooth muscle, and fat cells)
von Hippel-Lindau disease
autosomal dominant, characterized by hemangioblastoma or cavernous hemangioma, adenomas, cysts, associated with increased incidence of renal cell carcinoma, gene for this disease is on the short arm of chromosome 3
Tay-Sachs disease
autosomal recessive, lyposomal storage disease, deficiency of hexosaminidase A, accumulates Gm2 gangliosides, characterized by CNS degeneration, blindness, cherry-red spot on macula
Gaucher disease
autosomal recessive, lyposomal storage disease, deficiency of glucocerebrosidase, accumulates glucocerebrosides, 3 types, Type 1: hepatosplenomegaly (no CNS involvement) type 2: infantile, severe CNS involvement, death before 1 year of age type 3: juvenile, CNS involvement as well but less severe than type 2
Niemann-Pick disease
autosomal recessive, lyposomal storage disease, deficiency of sphingomyelinase, accumulates sphingomyelins, characterized by foamy histiocytes, hepatosplenomegaly, half have cheery red spot in macula similar to Tay-Sachs, death occurs by 3 years of age
Hurler syndrome
autosomal recessive, lyposomal storage disease, deficiency of alpha-L-Iduronidase, accumulates heparan sulfate and dermatan sulfate, hepatosplenomegaly, mental retardation, death by 10 years of age
Hunter's syndrome
sex recessive, lyposomal storage disease, deficiency of sc-l-iduronosulfate sulfatase, accumulates heparan sulfate and dermatan sulfate
von Gierke disease
AR, glycogen storage disease, deficiency of glucose 6 phosphatase, so glycogen accumulates in liver and kidney, hepatomegaly, sometimes intractable hypoglycemia
Pompe disease
AR, glycogen storage disease, deficiency a-1,4-glucosidase, accumulates glycogen in liver, heart, skeletal muscle, characterized by cardiomegaly, muscle hypotonia, splenomegaly, death occurs from cardiorespiratory failure before 3 years of age, can have intractable hypoglycemia
Cori disease
glycogen storage disease type III, deficiency of the debranching enzyme, amylo-1-6-glucosidase, leading to variable accumulation of glycogen in heart, liver,muscle, so stunted growth, hepatomegaly, hypoglycemia
McArdle syndrome
glycogen storage disease type V, deficiency of muscle phosphorylase, painful muscle cramps and muscle weakness following exercise
deficiency of galactose-1-phosphate uridyl transferase, failure to thrive, infantile cataracts, mental retardation, cirrhosis, can be prevented by removal of galactose from diet
galactokinase-deficiency galactosemia
much less frequent than class galactosemia, usually only marked by infantile catacracts
mutation of phenylalanine hydroxylase gene, mental deterioration, mousy/musty body odor, treat with phenyalanine-free diet
incomplete metabolism of phenylalanine and tyrosine from deficiency of homogentisic oxidase, accumulate homogentisic acid, urine turns black, ochronosis (dark pigmentation of fibraous tissues and cartilage)
Maple Syrup Urine disease
defect in keto-dehydrogenase complex, mental and physical retardation
cystic fibrosis
mutations in cystic fibrosis transmembrane conductance regulator (chromosome 7), malfunction of exocrine gland, increased viscosity of mucus, increased chloride concentration in sweat and tears, Sweat test, leads to chronic pulmonary disease, pancreatic insufficiency, meconium ileus
Fabry disease
x linked disorder, deficiency of alpha galactosidase, accumulate ceramide trihexoside, skin lesions,renal failure
classic hemophilia
factor VIII deficiency, hemorrhage from minor wounds and trauma
Lesch Nyhan syndrome
deficiency of hypoxanthine-guanine phosphoribosyltransferase, so impaired purine metabolism and excess production of uric acid, characterized by gout, mental retardation, self-mutilation
true hermaphrodite
has X and Y chromosomes and both ovarian and testicular tissue, very rare
has gonads of only one sex but appearance of external genitalia does not correspond with gonads present
anaphase lag
results in the loss of a chromosome during meiotic or mitotic division (in early embryonic life, can result in mosaicism)
B cells stay where in lymph nodes and spleen
germinal centers of lymph nodes and in the lymphoid follicles of the spleen
T cells stay where in lymph nodes and spleen
paracortical and deep medullary areas of lymph nodes and periarteriolar sheaths of the spleen
How can natural killer cells lyse cells
via antibody-dependent cell-mediated cytotoxicity
macrophages participate in which type of hypersensitivity reactions?
delayed hypersensitivity reactions
What cells have Birbeck granules?
Langerhans cells of the skin
What are two antigenn presenting cell types?
dendritic cells of lymphoid tissue and Langerhans cells of the skin
What is the complement system?
About 20 plasma proteins and their products which can be activated by the classic or alternate pathway to form a final product - the membrane attack complex that lyses target cells
What is the classic complement pathway
It is initiated by reaction with antigen-antibody complexes, the final lytic form of activated complement is the result of a series of enzymatic cleavages and recombinations of cleavage products
What is the alternative complement pathway
Initiated directly by nonimmunologic stimuli, such as invading microorganisms, and also leads to cleavage products that cause lysis. It bypasses the initial stages of the classic pathway
What is the human leukocyte antigen system?
group of related proteins referred to as HLA antigens, the genes that code for these are called histocompatibility genes and are located on the short arm of chromosome 6, known as the major histocompatibility complex (MHC), very important in organ transplantation (HLA typing and matching are now widely used to predict tissue compatibility)
What are class I antigens?
These include HLA-A, HLA-B, HLA-C, found on almost all human cells, principal antigens in tissue graft rejection (looked at by killer T cells)
What are class II antigens?
HLA-DP, HLA-DQ, HLA-DR antigens, chiefly found on immunocompetent cells (macrophages, dendritic cells, Langerhans cells, B cells, and some T cells)
What is the HLA-B27 antigen associated with?
ankylosing spondylitis
Which hypersensitivity reactions require antibody by plasma cells?
Types I, II, III
Type IV hypersensitivity reaction is mediated by?
T cells and macrophages
Type I hypersensitivity reaction
(anaphylactic) - antigen reacts with IgE bound to surface of basophils/mast cells which release histamine and other substances (which are vasoactive, smooth muscle spasm-inducing, or chemotactic) Examples: hay fever, allergic asthma, hives, anaphylactic shock
Type II hypersensitivity reaction
(cytotoxic) Antibodies react with antigens that are intrisic components of cell membrane or other structures, resulting in direct damage etc. Examples: warm antibody autoimmune hemolytic anemia, hemolytic disease of the newborn, Goodpasture syndrome, Graves disease
Type III hypersensitivity reaction
(immune complex) insoluble complement-bound aggregates of antigen-antibody complexes are deposited in vessel walls or on serosal surfaces or other extravascular sites; neutrophils are chemotactically attracted and release lysozomal enymes etc. Ex. Serum sickness, Arthus reaction, polyarteritis nodosa, SLE, immune complex-mediated glomerular diseases
Type IV hypersensitivity reaction
(delayed hypersensitivity) proliferation of antigen-specific CD4+ memory T cells with secretion of IL-2 and other cytokines, recruit and stimulate phagocytic macrophages Ex. Tuberculin reaction, contact dermatitis, tumor cell killing, virally infected cell killing
What are the three methods of Type II hypersensitivity reactions
1. complement fixing antibodies (complement results in cell lysis and destruction) 2. antibody dependent cell mediated cytotoxicity (free Fc portion of antibody reacts with cytotoxic leukocytes (especially NK cells) and the target cells are killed) 3. reaction of anti-receptor antibodies with cell-surface receptor protein (ex. Graves disease)
serum sickness
systemic deposition of antigen-antibody complexes in multiple sites (especially the heart, joints, and kidneys)
What are the three types of transplant rejection
hyperacute rejection, acute rejection, chronic rejection
hyperacture rejection
is primarily antibody-mediated, occurs in presence of pre-existing antibody to donor antigens, occurs within minutes, is a localized Arthus reaction marked by acute inflammation, fibirnoid necrosis of small vessels, and extensive thrombosis
acute rejection
primarily T-cell mediated, occurs days to months after transplantation, is characterized by infiltration of lymphocytes and macrophages
chronic rejction
is primarily caused by antibody-mediated vascular damage, may occur months to years after an otherwise successful transplantation, is characterized by marked vascular fibrointimal proliferation, often resulting in a small, scarred kidney (is becoming more common with the success of immunosuppression in overcoming acute rejection)
Graft versus host disease
significant problem in bone marrow transplantation because immunocompetent cells are transplanted in this procedure, can also be caused by whole blood transfusion in patients with severe combined immunodeficiency (SCID), characterized by rejection of host cells by engrafted T and B cells, clinical features: fever, rash, hepatosplenomegaly (principal target organs are liver, skin, and gastrointestinal mucosa)
X-linked agammaglobulinemia
X-linked disorder in male infants, failure of antibody synthesis due to block of maturation of pre-B cells to B cells due to a mutation in tyrosine kinase gene, absence of plasma cells, absent or poorly defined germinal centers in lymphoid tissue, therefore recurrent bacterial infections
Isolated IgA deficiency
most common inherited B cell defect, inability of IgA B cells to mature to plasma cells, characterized by occasional anaphylacic reactions to transfused blood and infections
Common variable immunodeficiency
failure of terminal B cell maturation, resulting in fewer plasma cells and thus hypogammaglobulinemia
DiGeorge syndrome
congenital T cell deficiency resulting from aberrant embryonic development of the third and fourth branchial arches, hypoplasia of thymus and parathyroid, failure of T cell maturation, leading to lymphopenia, recurrent viral and fungal infections and tetany from hypoparathyroidism with hypocalcemia
Severe combined immunodeficiency disease (SCID)
characterized by marked deficiency of B and T cells, profoudn lymphopenia, occurs in X and AR forms, many AR cases are due to adenosine deaminase deficiency leading to accumulation of deoxyadenosine and deoxy-ATP (toxic to lymphocytes), clinical: severe infections, failure to thrive, graft versus host disease (from blood transfusions), thymic hypoplasia, treat with bone marrow, ADA gene transplantation
Immunodeficiency with thrombocytopenia and eczema (Wiskott-Aldrich syndrome)
X-linked, eczema, thrombocytopenia, recurrent infections, and poor antibody response to polysacharide antigens, usually has normal total immunoglobulins
HIV expressed gp120, binds CD4+ T cells, proviral DNA integrated into host genome, diagnosis by ELISA, paradocxically demonstrate hypergammaglobulinemia from polyclonal B cell activation, opportunistic infections, increased incidence of Kapasi sarcoma
connective tissue (collagen) diseases
mostly feature fibrinoid change in connective tissue
prototype connective tissue disease, 80% female, marked by presence of ANAs and extensive immune complex-mediated inflammatory lesions (greatest importance are those in kidney)
clinical manifestations of SLE
fever, malaise, lymphadenopathy, weight loss, joint systems, butterfly rash, Raynaud phenomenon, serosal inflammation, diffuse interstitial pulmonary fibrosis, endocarditis, glomerular changes (wire loop appearance, thickening of basement membrane)
lab findings of SLE
LE test, ANA tes (reacts with double stranded DNA and Smith antigens), serum complement decreased, false-positive tests for syphilis
Progressive systemic sclerosis
(PSS, scleroderma), widespread fibrosis and degenerative changes, marked by ANA anti-Scl-70, ANA with anti-centromere activity is characteristic of the CREST syndrome (calcinosis, Raynoud phenomenon, esophageal dysfunction, sclerodactyl, and telangiectasia)
Clinical manifestations of Progressive systemic sclerosis
hypertrophy of collagen fibers (leads to fixed facial appearance), sclerodactyl (claw like hands), Raynoud phenomenon (75% of patients), visceral organ involvement (esophagus, pulmonary fibrosis, hypertension)
Sjogren syndrome
xerostomia, keratojunctivitis, one of several connective tissue or other autoimmune disease, most often rheumatoid arthritis (Sicca syndrome only has xerostomia and keratoconjunctivitis), involvement of salivary glands (usually bilaterally enlarged parotids), involvement of lacrimal glands
Clinical findings of Sjogren syndrome
ANAs, including the highly specific anti-SS-B
chronic inflammatory process especially involving proximal muscles of the extremities (when skin is involved, characteristic reddish-purple rash over exposed face/neck (called dermatomyositis)), characterized by increased serum creatine kinase and usually some ANAs (muscle biopsy demonstrates necrotic muscle)
mixed connective tissue disease (MCTD)
mainly female (80%), clinically like other connective tissue disorders expect renal involvement is uncommon, characterized by high-titer anti-nRNP and immunofluorescent speckled nuclear appearance on morphologic ANA analysis)
polyarteritis nodosa
immune complex vasculitis characterized by segmental fibrinoid necrosis in the walls of small and medium arteries of almost any organ (mainly in men), antigen (Hep B in 30%, drugs), clinical manifestations: abdominal pain, hypertension, asthma, splenomegaly...)
group of disorders characterized by deposition of amyloid (a proteinaceous material)
amyloid structure
not a single substance but a group of substances that share a common physical structure that can be formed by a number of proteins, always in a beta-pleated sheet configuration
amyloid - morphologic features
extracellular in distribution, amorphous eosinophilic appearance, characteristically stained by Congo red dye, demonstrating apple green birefriegence
primary amyloidosis
caused by deposition of amyloid fibrils derived from immunoglobulin light chains (called AL protein for amyloid light chain), frequently associated with plasma cell disorders such as multiple myeloma and Waldenstrom macroglobulinemia
secondary amyloidosis
caused by deposition of AA protein (formed from a precursor SAA (serum amyloid-associated protein which is increased during chronic tissue destruction), usually involves the kidney (nephrotic syndrome common), liver, adrenals, pancreas, lymph nodes, and spleen. Characteristically a comlication of chronic inflammatory disease (such as rheumatoid arthritis, TB, osteomyelitis, syphilis, or leprosy)
Portuguese type of polyneuropathy
associated with transthyretin (serum protein that transports thyroxine and retinol), severe peripheral nerve involvement
Alzheimer disease
A4 amyloid (also known as amyloid beta-protein), gene that codes for the protein precursors is on chromosome 21
Familial Mediterranean fever
AA amyloid, characterized by episodic fever and polyserositis
Medullary carcinoma of the thyroid
amyloid protein from calcitonin
Diabetes mellitus
deposit of amyloid in islet cells (called amylin or islet amyloid polypeptide (IAPP))
senile amyloidosis
transthyretin, found in autopsy of very elderly (no listed clinical signs)
dialysis associated amyloid
beta-microglobulin, found in joints of patients undergoing years of hemodialysis (no listed clinical signs)