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Pathology IV > Quiz 4 > Flashcards

Quiz 4 Flashcards

1
Q

3 types of bone cells:

A

osteocytes
osteoblasts
osteoclasts

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2
Q

3 general bone functions:

A

mechanical
biomechanical reserve
metabolic

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3
Q

What are the mechanical functions of bones?

A

protection
shape
movement
sound transduction (hearing)

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4
Q

What is the biomechanical reserve function of bones?

A

blood production in the marrow

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5
Q

What are the metabolic functions of bones?

A 
mineral storage
growth factor storage
fat storage
acid-base balance
detox (store heavy metals)
endocrine function (FGF, osteocalcin)
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6
Q

Genetic bone d/o referred to as brittle bone dz:

A

osteogenesis imperfecta (OI)

born w/defective CT d/t deficiency of type-I collagen
8 subcategories, most incompatible with life

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7
Q

Idiopathic avascular osteonecrosis of the epiphysis of the femoral head leading to an interruption of the blood supply of the head of the femur close to the hip joint:

A

Legg-Calvé-Perthes syndrome

aseptic necrosis not d/t trauma or septic dz

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8
Q

Softening of bones in children potentially leading to fractures and deformity, and among the most frequent childhood dz in many developing countries:

A

rickets

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9
Q

MC cause of rickets:

A

Vitamin D deficiency

also:
calcium deficiency
mb 2° to severe diarrhea/vomiting

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10
Q

Toddlers who contract rickets get a __________ deformity, vs older children who get ______________.

A

toddler - genu varum

children - genu valgum OR “windswept” - both same direction

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11
Q

Prominent knobs of bone at the costochondral jts of rickets pts are known as:

A

rachitic rosary - beading of the ribs

d/t deficiency of calcium -> lack of mineralization -> overgrowth of cartilage

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12
Q

Dz characterized by low bone mass and structural deterioration of bone tissue:

A

osteoporosis

mb generalized loss or localized (i.e. casted limb)

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13
Q

The inc porous nature of osteoporotic bone leads to fragility and inc susceptibility to fractures, esp of the ____, _____, and _____, although any bone can be affected.

A

hip, spine, and wrist

affects ~10 million in the US [F:M 4:1]

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14
Q

1° osteoporosis is a term used to describe _________ d/t advanced age or menopause.

A

osteopenia (bone thinning)

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15
Q

2° osteoporosis implies dec bone mass d/t:

A
  • medications (corticosteroids, anticonvulsants, anticoag, chemo, EtOH)
  • malabsorption (malnutrition, low vit C or D)
  • immobilization
  • medical conditions
  • hyperparathyroidism
  • hypo/hyperthyroidism
  • hypogonadism
  • Cushing’s
  • Addison’s
  • DM
  • liver dz
  • neoplasm (mult myeloma)
  • ectopic hormone prodution (PTHrP, ACTH)
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16
Q

T/F: Osteoporosis cannot be reliably detected by plain x-rays until 15% of bone mass has been lost.

A

False

actually can’t see reliably until 30-40% loss!

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17
Q

T/F: Serum calcium, phosphorus, or alk phos can be measured to dx osteoporosis.

A

False - serum levels are buffered by bone catabolism, and are NOT diagnostic

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18
Q

Diagnostic testing for osteoporosis:

A

DEXA scan (dual-energy x-ray absorptiometry)

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19
Q

T/F: Postmenopausal osteoporosis is characterized by a hormone dependent acceleration of bone loss primarily d/t estrogen deficiency.

A

true

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20
Q

Diminished estrogen results in secretion of cytokines:

which are in large part responsible for __________ activity.

A

IL-1, IL-6, TNF-alpha

inc osteoclast activity

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21
Q

Risk factors for osteoporosis:

A
  • female sex
  • thin / small frame
  • low bone mass
  • advanced age
  • FHx
  • PMHx of fracture after 50 y.o.
  • Hx of early fracture in 1st degree relative
  • estrogen deficiency d/t menopause (esp early/surgical)
  • amenorrhea
  • anorexia nervosa
  • low testosterone in males
  • low lifetime calcium intake
  • Vit D deficiency
  • sedentary lifestyle
  • cigarette smoking / excessive EtOH
  • Caucasian / Asian descent
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22
Q

Exaggerated curvature of the thoracic spine that results in a rounded or hunched back:

A

kyphosis

commonly seen in pts w/osteoporosis d/t compression fractures of vertebrae

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23
Q

Dz characterized by high bone turnover with accelerated osteoclast and osteoblast activity:

A

Paget’s dz (osteitis deformans)

Inc calcification of bone - predilection for inc bone deposition in skull, pelvis, tibia, femur

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24
Q

Clinical signs of Paget’s dz:

Labs:

A
  • bone pain
  • bone deformities
  • fractures
  • markedly elev alk phos
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25
Q

Characteristic histological finding in Paget’s:

A

mosaic pattern of lamellar bone - jigsaw puzzle
“woven bone”
lytic and sclerotic

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26
Q

T/F: Woven bone is produced when osteoblasts produce osteoid rapidly, which is always abnormal.

A

false

occurs initially in all fetal bones, later replaced by lamellar bone; in adults woven bone occurs after fracture or in Paget’s

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27
Q

Pts w/Paget’s dz have inc risk for development of ______________, such as ___________ in the area of abn bone growth.

A

malignant neoplasms

osteosarcoma

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28
Q

Paget’s dz on x-ray:

A

cotton ball appearance - sclerotic bone with areas of lucency

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29
Q

T/F: Paget’s dz typically affects a single bone.

A

false
multiple bones - 85% of cases
first warning sign - bone pain, warmth

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30
Q

Rare inherited disorder whereby the bones harden and become more dense:

A

osteopetrosis - “stone bone”

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31
Q

Infection of the bone or bone marrow:

A

osteomyelitis

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32
Q

Osteomyelitis in children typically affects the ______ bones, whereas in adults the _______ and ______ are MC affected.

A

long bones

vertebrae and pelvis

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33
Q

Organism MC isolated from all forms of osteomyelitis:

A

Staph aureus

[in the vertebral bodies, 50:50 with TB, called Pott’s dz]

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34
Q

T/F: In osteomyelitis, mixed infections are rare.

A

false

“mixed infx are the rule, rather than the exception”

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35
Q

Term used to describe all of the skeletal changes of chronic renal dz:

A

renal osteodystrophy

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36
Q

Changes seen in renal osteodystrophy:

A
  • inc osteoclast bone resorption
  • delayed matrix mineralization (osteomalacia)
  • osteosclerosis
  • osteoporosis
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37
Q

Children with renal dystrophy are at risk of:

A
  • rickets

* growth retardation

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38
Q

In chronic renal failure pts, the is significant interrelationship btw:

A
  • inadequate kidney function
  • 2° hyperparathyroidism
  • altered vit D metabolism
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39
Q

Fractures are classified as ______ or ______ when the overlying tissue is intact, and _______ or _______ when the fracture site communicates with skin surface.

A

simple or closed

compound or open

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40
Q

A fracture is displaced when:

A

the ends of the bone at fracture site are not aligned.

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41
Q

A fracture is comminuted when:

A

the bone is splintered or crushed.

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42
Q

A pathologic fracture is when:

A

the break occurs in bone already altered by dz process

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43
Q

A stress fracture is:

A

a slowly developing fracture that follows a period of inc physical activity in which bone is subjected to repetitive loads (sports training, long distance running).

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44
Q

Imaging used to dx a stress fracture:

A
  • plain film x-ray (poor view of aligned fractures)
  • bone scan
  • uses radioactive tracer via IV, taken up in areas of osteoblast and inflammatory activity (also for tumor, infx)
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45
Q

T/F: Bone is unique in its ability to repair itself.

A

True

bone can completely create new bone by reactivating processes that normally occur during embryogenesis

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46
Q

Solitary bone cysts are MC in ________, and are predominantly in the _______ ends of ______ bones.

A

young males - 1st or 2nd decade

distal ends of long bones (humerus, femur)

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47
Q

X-ray of solitary bone cyst reveals:

A
    • smooth thin cortex
  • usu symmetrical
  • close to epiphysis
  • diameter of bone mb inc
  • cavity filled with clear or bloody fluid
  • lacking specific microscopic features
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48
Q

Early tx of solitary bone cyst aims at:

More aggressive tx:

A

preventing pathologic fractures

scraping sides of cyst (curettage), inserting bone chips

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49
Q

T/F: Recurrence of solitary bone cysts is common.

A

false

vs. aneurysmal - 25% recurrence

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50
Q

Aneurysmal bone cysts are MC in ____________, and are MC in the ________ of _____ bones, and are also found in __________.

A

females - 2nd or 3rd decade (mb in children)
metaphysis
long
vertebrae

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51
Q

Aneurysmal bone cysts are (benign/malignant) lesions that are (locally/distantly) aggressive.

A

benign

locally

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52
Q

Gross pathology of aneurysmal bone cysts:

A
  • distortion of bone
  • less symmetrical shape
  • honeycomb appearance on x-ray
  • spongy appearance on cut surface
  • variable size of cystic spaces, usu contain blood
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53
Q

T/F: The MC bone tumors are primary.

A

false

METASTATIC - by far

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54
Q

Benign, bone-forming neoplasm characterized by small area of neoplastic tissue surrounded by wide zone of mature, reactive bone:

A

osteoid osteoma

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55
Q

Common findings of osteoid osteoma:

A
  • less than 2cm
  • MC solitary
  • multiple lesions seen in Gardner syndrome
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56
Q

Osteoid osteoma usu occurs in ______________.

Common presenting sx:

A

children and young adults

night pain w/localized tenderness

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57
Q

X-ray of osteoid osteoma shows:

A
  • small, well-circumscribed lesion
  • round or oval
  • near cortex
  • surrounded by densely sclerotic bone
  • radio-luscent or radio-opaque aspects
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58
Q

Gross examination of osteoid osteoma:

A
  • gritty
  • cherry-red
  • surrounded by dense sclerotic bone
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59
Q

MC primary malignant bone tumor:

A

osteosarcoma

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60
Q

Osteosarcomas may occur in all age groups, but 75% of pts are _____________.

A

younger than 20 y.o.

3rd MC malignancy in adolescents (after leukemia, lymphoma)

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61
Q

Osteosarcomas MC occur in the distal _______ or around the ______.

A

distal femur
around the knee

75%

10% present w/METS

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62
Q

X-ray findings of osteosarcoma:

A
  • combo of bone destruction/formation
  • rarely involves jt space
  • soft tissue spicules of calcium
    25% lytic / 35% sclerotic / 40% mixed
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63
Q

Classic osteosarcoma:

A
  • aggressive, rapid growth
  • high risk of local spread
  • “skip” lesions
  • soft tissue involvement possible
  • common early pulmonary mets
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64
Q

Histopathology of osteosarcoma:

A
  • atypical cells
  • large nuclei
  • small amt cytoplasm
  • osteoid formation
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65
Q

Uncommon bone tumor usually found around the knee in either the distal femur and/ or the proximal tibia:

A

fibrosarcoma

tumor produces collagen matrix but does not produce osteoid or chondroid

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66
Q

T/F: Fibrosarcoma is a primary bone tumor.

A

true, but mb 1° or 2° to:

  • Paget’s
  • chronic osteomyelitis
  • prior bone infarct
  • prior irradiation for bone cancer
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67
Q

Characteristic histopathology of fibrosarcoma:

A

sheets of spindle-shaped cells

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68
Q

An aggressive tumor composed of oval tumor cells and multinucleated tumor cells, referred to as giant cells, which are uniformly distributed throughout the lesion:

A

Giant cell tumor (GCT)

usu tumor of adulthood (>20) - MC in 3rd decade

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69
Q

X-ray of GCT shows:

A
  • destructive radiolucent lesion
  • usu oval shaped
  • centered in epiphysis
  • cortical erosion / thinning
  • expands bone contour w/o border or new bone or sclerosis
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70
Q

Histopathology of GCT:

A

lg multinucleate cells formed by fusion of several cells (50+ nuclei)

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71
Q

Malignant tumor of bone composed of small, rounded cells that are uniform in size and densely packed:

A

Ewing’s sarcoma

avg age - 13-14

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72
Q

Histopathology of Ewing’s sarcoma:

A
  • uniform, densely-packed cells
  • single round/oval nucleus
  • indistinct cellular borders
  • resemble lymphocytes
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73
Q

X-ray of Ewing’s sarcoma:

A

“moth eaten” destructive lesion

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74
Q

Malignant dz of plasma cells in the bone marrow, with monoclonal overgrowth of one clone of plasma cells:

A

multiple myeloma

pathological fractures common

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75
Q

X-ray of multiple myeloma:

A

lytic lesions

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76
Q

In adults, more than ___% of skeletal metastasis originate from cancers of the prostate, breast, kidney and lung.

A

75%

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77
Q

In children, bony metastasis MC originate from:

A
  • neuroblastoma
  • Wilm’s tumor
  • bone tumor
  • osteosarcoma
  • Ewing sarcoma
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78
Q

Skeletal mets are typically (uni/multifocal).

A

multifocal

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79
Q

Nonsynovial joints are “solid”, in that:

A

they lack a joint space and provide for minimal movement.
Nonsynovial joints provide structural integrity.

pubic symphysis, cranial sutures

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80
Q

Synovial joints consist of:

A

two (or more) bone ends connected through a joint capsule of dense fibroconnective tissue.
joint may be strengthened by ligaments and muscles.

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81
Q

The articular surfaces of bone and cartilage are covered by a thin synovium capable of producing a clear fluid that acts as a __________ and provides nutrition for the _____________.

A

lubricant

articular hyaline cartilage

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82
Q

Hyaline cartilage is composed of:

A

type 2 collagen
water
proteoglycans
chondrocytes

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83
Q

Hyaline cartilage is a unique connective tissue ideally suited to serve as an:

A

elastic shock absorber and wear resistant surface

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84
Q

Dz that destroy articular cartilage do so by activating the ___________ and decreasing the production of ________, thereby accelerating the rate of matrix breakdown.

The chondrocytes react by increasing _____________ however, the response is usually inadequate.

A

catabolic enzymes
inhibitors

matrix production

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85
Q

Cytokines such as ____, _____, and _____ trigger the degradative process, and their sources include:

A

IL-1, IL-6 and TNF-alpha

  • chondrocytes
  • synoviocytes
  • fibroblasts
  • inflammatory cells
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86
Q

MC type of joint dz:

A

osteoarthritis

aka degenerative joint dz (DJD)

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87
Q

T/F: Osteoarthritis is characterized by the progressive erosion of articular cartilage.

A

true

considered to be an intrinsic disease of cartilage - biochemical and metabolic alterations result in breakdown

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88
Q

T/F: Secondary arthritis refers to arthritis due to the aging process and involves no other predisposing disease.

A

false

primary (idiopathic) arthritis

89
Q

DJD damages are often irreversible because articular cartilage is:

A

avascular
aneural
alymphatic

90
Q

When chondrocytes release IL-1, IL-6, and TNF-alpha, this inhibits synthesis of:

A

type 2 collagen

proteoglycans

91
Q

X-ray of DJD shows:

A
  • jt space narrowing
  • marginal osteophyte formation
  • subchondral bone cysts
  • sclerosis
92
Q

Nodes assoc w/DJD:

A
  • Heberdon - DIPs

* Bouchard’s - PIPs

93
Q

Histopathology of DJD:

A
  • fibrous lined cysts in subchondral bone

* osteophytes

94
Q

Chronic systemic inflammatory dz of unknown cause, characterized by polyarthritis which is often progressive and deforming:

A

rheumatoid arthritis

95
Q

RA is MC assoc with rheumatoid factors, which are:

A

anti-IgG autoantibodies

[unclear; notes also say IgM, IgA]

96
Q

RA jt involvement is typically (oligo/poly) articular, (unilateral/bilateral), and (a/symmetrical).

A

poly
bilateral
symmetrical

97
Q

Commonly affected joints of RA:

A
  • MCPs
  • PIPs
  • wrist
  • elbow
  • knee
  • ankle
  • MTPs
98
Q

The primary joint lesion in RA occurs in the ___________ of affected joints and is called:

A

synovial membrane

diffuse proliferative and exudative synovitis

99
Q

Histopathology of rheumatoid synovitis:

A
  • multiple layers
  • proliferative (hyperplastic) synoviocytes
  • lymphocytic infiltration
100
Q

In RA, deformity and dislocation of misshapen bone ends are caused by:

A
  • muscular imbalance and contracture

* laxity of capsule and ligaments previously distended by effusion

101
Q

The extra-articular manifestations of RA occur more often in sero(negative/positive) pts with (mild/severe) dz and circulating complexes of ___________.

A

positive
severe
rheumatoid factor

102
Q

Describe rheumatoid nodules.

A
  • subQ
  • 20-25% of pts
  • firm, nontender
  • over bony prominence
  • lungs, heart, eyes, tendon sheaths as well
103
Q

Describe rheumatoid arteritis.

A
  • inflammatory vascular lesions
  • venules, capillaries, arterioles, arteries of skin/organs
  • mb systemic necrotizing vasculitis of sm/med arteries
104
Q

In RA, enlargement of regional lymph nodes is (un/common) and palpable splenomegaly occurs in __% of pts.

A

common

10%

105
Q

Felty’s syndrome:

A
  • RA
  • splenomegaly
  • leukopenia
106
Q

Multiple factors and complex mechanisms are implicated in the pathogenesis of RA, among them:

A
  • autoimmunity
  • enzymatic
  • increased cytokine activity
  • other mechanisms of jt inflammation/destruction
  • genetic predisposition
  • environmental influences
107
Q

80% of RA pts test positive for RF in ______ and _________.

A

serum
synovial fluid

(10-20% in JIA)

108
Q

RF is not exclusively found in RA, but also tests positive in conditions such as:

A
  • Sjogren’s
  • SLE
  • progressive systemic sclerosis
  • infectious hepatitis / HCV
  • PM/DM
  • syphilis
  • TB
109
Q

Because RF is non-specific, new tests have been developed for RA, such as:

A
  • anti-citrulinated protein antibodies (ACPAs or anti-CCP)
110
Q

Chronic systemic polyarthritis of unknown etiology with onset at less than 16 years of age:

A

juvenile idiopathic arthritis (JIA)

aka JRA or Still’s dz

111
Q

JIA and RA are similar, but differences of JIA include:

A
  • high fever
  • rash
  • lymphadenopathy
  • less jt pain
  • less frequency of RF
  • less subQ nodules
112
Q

A group of diseases characterized by inflammatory arthritis and an assoc w/ HLA-B27 or triggering infx:

A

seronegative spondyloarthropathies

113
Q

Examples of seronegative spondyloarthropathies include:

A
  • reactive arthritis
  • ankylosing spondylitis
  • enteropathic arthritis
  • psoriatic arthritis
114
Q

Chronic inflammatory jt dz of the axial skeleton, esp SI jts:

A

ankylosing spondylitis (AS)

Associated complications: uveitis, aortitis, amyloidosis, ++

115
Q

___% of AS pts are HLA-B27 positive.

A

90%

116
Q

Progression of AS:

A
  • HLA-B27 immunogenetic phenotype may predispose to development of autoantibodies directed at joints after infx
  • chronic synovitis causes destruction of articular cartilage
  • Inflammation of tendon/lig insertions leads to bony growths
117
Q

Autoimmune rxn initiated by response to a prior infx, characterized by triad of arthritis, non-gonococcal urethritis or cervicitis, and conjunctivitis:

A

reactive arthritis (Reiter’s)

118
Q

Reactive arthritis MC affects:

A

males in 20’s & 30’s

HLA-B27 + (80%)

119
Q

Reactive arthritis is MC assoc with which GI and GU infx?

A

GI - salmonella, shigella, yersinia, campylobacter

GU - chlamydia

120
Q

50% of reactive arthritis pts have waxing and waning sx that resolve in ____ to _______.
50% have recurrent _________, _________, and ______.

A

weeks to 6 months

arthritis, tendonitis, and LS back pain

121
Q

Reactive arthritis MC affects which jts?

A 
SI
knees
ankles
feet
toes
fingers (sausage)
122
Q

Enteropathic arthritis MC affects which jts?

A

knees and ankles

but also mb involve wrists/fingers/toes

123
Q

Enteropathic arthritis refers to a non-reactive arthritis which occurs following _____ infx by ___________________, and usu occurs w/in (hours/days/weeks) of the initiating infx.

A

bowel
Salmonella, Shigella, Yersina, Campylobacter
days

124
Q

Proposed mechanism of enteropathic arthritis:

A

ab response to LPS

THE ORGANISMS DO NOT INFECT JTS

125
Q

T/F: Psoriatic arthritis describes the jt involvement assoc with psoriasis.

A

false

psoriatic arthritis is a distinct condition
5% of pts with psoriasis are affected

126
Q

First jts usu affected by psoriatic arthritis:

A

DIP jts of fingers and toes
“sausage” appearance d/t tendon sheath inflammation

may become widespread - wrists, shoulders, ankles, knees, hips
20-40% develop SI and spinal dz

127
Q

At what age does psoriatic arthritis usu develop?

A

35-45

128
Q

Distinctive features of psoriatic arthritis:

A
  • nail (fairy) pitting
  • onycholysis
  • histo and jt destruction similar to RA
  • more frequent remissions than RA
129
Q

Mechanisms of infectious (septic) arthritis infx:

A
  • hematogenous spread
  • trauma / foreign body
  • adjacent infx (abscess, osteomyelitis)
130
Q

Classic presentation of infectious arthritis:

A

sudden development of single acutely painful and swollen joint with restricted ROM

131
Q

MC organisms in infectious arthritis:

A
  • staph (MC in adults)
  • strep (2nd MC)
  • gonococcus (pathognomonic trunkal vessicles)
  • H influenza (MC in kids except where vax)
  • Salmonella (sickle cells pts)
  • gm neg bacilli
  • E coli (elderly, IV drug users, seriously ill)
  • psuedomonas (kids w/puncture wound)
132
Q

T/F: Pts with artificial jts are less likely to contract infectious arthritis, but sx and tx are the same.

A

false

more risk w/artificial jts
different organisms
different sx, tx

133
Q

Organisms notable for being seen in spinal septic arthritis:

A
  • M tuberculosis
  • Salmonella spp
  • Brucella spp
134
Q

_________ arthritis is often “migratory” with multiple jts flaring up, and fever is less common.

A

Gonococcal

135
Q

Misdiagnosis or delayed tx of infectious arthritis leads to:

A

joint destruction

dx by aspirating fluid (hospital)

136
Q

T/F: Tuberculous arthritis is decreasing in incidence as TB is further eradicated.

A

false - increasing as TB reemerges as an important infx dz

Spread to jt usu from

  • adjacent TB osteomyelitis
  • hematogenous spread of pulmonary TB

The onset of jt involvement is usu slow / insidious

137
Q

Jts MC affected by TB arthritis:

The involved joint often shows:

A

wt bearing jts

  • hips (more)
  • knees
  • ankles (less)
  • confluent granulomas
  • central caseous necrosis
138
Q

Tuberculosis of the spine (aka Pott’s dz) MC affects the (cervical/thoracic/lumbar) vertebrae and usu comprises both tuberculous ________________. The inflammatory and destructive processes involve ___________________, leading to (kyphotic/lordotic) angulation and cord compression.

A

thoracic and lumbar
osteomyelitis and arthritis
adjacent vertebral bodies and IV discs
kyphotic

139
Q

Principle vector of Lyme dz:

A

deer tick, Ixodes scapularis

140
Q

Spirochete infx that causes Lyme arthritis:

A

Borrelia burgdorferi

141
Q

Dominant feature of late Lyme dz:

A

arthritis

tends to be remitting and migratory

142
Q

MC jts involved in Lyme arthritis:

A
  • knees
  • ankles
  • shoulders
  • elbows

usu 1-2 jts affected

143
Q

Characteristic rash of Lyme dz:

A

erythema migrans

144
Q

Viral arthritis can result from numerous viruses, including:

A
  • parvovirus B19
  • rubella
  • HCV
  • HIV
145
Q

The 2 MC crystalline arthritis conditions are:

A
  • gout

* pseudogout

146
Q

Gout is d/t jt space deposition of _____________ crystals whereas pseudogout is d/t deposition of ______________ crystals.

A

monosodium urate (uric acid)

calcium pyrophosphate

147
Q

T/F: Increased uric acid levels lead to gout.

A

not necessarily

~10% of western hemisphere have hyperuricemia
less than 0.5% develop gout

148
Q

The multifactorial development of gout includes:

A
  • genetic predisposition
  • increasing age
  • lead toxicity
  • lifestyle
  • obesity
  • heavy alcohol intake
  • drugs
  • aspirin
  • thiazide diuretics
  • loop diuretics
149
Q

Hyperuricemia may develop d/t:

A
  • inc uric acid production

* dec uric acid excretion

150
Q

Term used for gout developing as a consequence of ingesting lead contaminated alcohol (moonshine):

A

saturnine gout

151
Q

T/F: Synovial fluid is an efficient solvent for monosodium urate, which causes slow formation of crystals.

A

false

poor solvent! hyperuricemia leads to supersaturation leads to crystals precipitating out

152
Q

Term used to describe the optical property assoc w/ certain transparent crystals in which the speed of propagation of light along the major and minor axes of the crystal differs, causing the plane of polarized light to be rotated:

A

birefringence

seen in gouty arthritis

153
Q

When polarized filters are used in microscopy, the background appears (light/dark), and birefringent materials like _____ and ______ appear (brighter/darker).

A

dark
MSU and CPPD
brighter

154
Q

Impact of crystals in jts:

A
  • chemotactic to leukocytes
  • activate complement
  • macrophages phagocytose uric acid, release toxic free radicals and leukotrienes
  • activated neutrophils release lysosomal enzymes
  • deposition in kidney -> gouty nephropathy, mb renal failure
155
Q

Pathognomonic lesion of gout:

A

tophus - masses of crystalline or amorphous urate deposits surrounded by an inflammatory reaction of macrophages, fibroblasts, and giant multi-nucleated cells.

156
Q

Classic joint involvement of gout:

A

1st MTP jt

157
Q

MC jts involved in pseudogout:

A
  • knees
  • ankles

ranges from asx/mild to significant jt damage

158
Q

Bursitis is often assoc with:

A 
* chronic trauma
secondary to:
* RA
* gout
* jt infx
* calcification in/around bursa
159
Q

Small (1-1.5 cm) cyst usu located near a jt capsule or tendon sheath:

A

ganglion cyst

common in wrist
usu pea-sized, firm, fluctuant

160
Q

A ganglion arises as a result of:

A

cystic degeneration of CT
lacks a true cell lining
fluid similar to synovium
NO communication w/jt space

161
Q

Herniation of synovial fluid through jt capsule may produce:

A

a synovial cyst

162
Q

A synovial cyst in the popliteal fossa is called:

A

Baker’s cyst

common in RA (but not exclusive to)

163
Q

Term to describe several closely related benign neoplasms that develop in the synovial linings of joints, tendon sheaths and bursae, characterized by inflammation and overgrowth of the jt lining:

A

villonodular synovitis

MC forms:

  • pigmented (PVNS)
  • giant cell tumor of tendon sheath (GCTTS)
164
Q

MC jts assoc w/villonodular synovitis:

A

hip or knee

also affects shoulder, ankle, elbow, hand, foot

165
Q

Histopathology of villonodular synovitis:

A
  • mononuclear cell infiltrate

* hemosiderin-laden macrophages

166
Q

MC jts affected by synovial sarcoma:

A

knee and ankle

close to jts, tendon sheath, bursa

167
Q

Two basic types of skeletal muscle atrophy:

A
  • disuse
  • generalized (immobilization)
  • localized (casting)
  • neurogenic
168
Q

T/F: A muscle cell and a muscle fiber are the same thing.

A

true

each cell contains hundreds to thousands of myofibrils
exercise causes increase of myofibrils (not cells)

169
Q

____________ often occur in neurogenic atrophy as a result of opposing muscle groups encountering (reduced/increased) resistance.

A

Contractures

reduced

170
Q

Term used to describe a broad range of inherited dz characterized by progressive muscle damage and weakness [over 100 different forms]:

A

muscular dystrophy

171
Q

MC form of muscular dystrophy:

A

Duchenne’s

also one of the most serious

172
Q

Duchenne’s is d/t a defective gene on (X, Y) chromosome, leading to inability to produce one of the proteins in the skeletal muscle cell membrane, ________, absence of which permits excess _______ to penetrate the sarcolemma (mm membrane).

A

X
dystrophin
calcium

173
Q

Duchenne’s ago of onset is usu:
MC muscles affected first:
Survival time:

A

2-6 y.o. (often wheelchair-bound by 12)
pelvis, hips, upper arms and legs
rare beyond 20’s

174
Q

How does excess calcium entry harm muscle cells?

A

causes the creation of more ROS than cell’s oxide-scavenging enzymes can process. Continued calcium entry causes further oxidative stress, ultimately resulting in cell death. They necrose and are replaced by fibrous CT or adipose (late).

175
Q

Characteristic muscle biopsy of Duchenne’s:

A
  • degen and atrophy of fibers
  • some fiber regeneration
  • hypertrophy of remaining fibers
  • scattered inflammatory cells (early)
176
Q

Duchenne’s is an (X, Y) linked (dominant, recessive) dz, occurring almost exclusively in (boys, girls).

A

X
recessive
boys

177
Q

Neuromuscular dz leading to fluctuating muscle weakness and fatigability:

A

myasthenia gravis

178
Q

In myasthenia gravis, weakness is usu caused by _________ that block _________ receptors at the post-synaptic neuromuscular junction, inhibiting the (stimulative, inhibitive) effect of the neurotransmitter.

A

circulating antibodies (present in 90%)
acetylcholine
stimulative

179
Q

Risk factors for myasthenia gravis:

A
  • female sex
  • age 20-40
  • familial Hx of MG
  • presence of autoimmune dz
  • D-penicillamine ingestion (drug-induced MG only)
180
Q

The antibodies in MG are produced by _____ cells, that have been derived from __ cells.

A

plasma

B

181
Q

The hallmark of myasthenia gravis is:

The MC first presenting symptom is:

A

muscle weakness that worsens during periods of activity and improves after periods of rest.

weakness of eye muscles

182
Q

Which muscles are especially susceptible to MG:

A

Muscles that control eye and eyelid movement, facial expression, chewing, talking, and swallowing.

mb also neck and limb muscles, breathing muscles.

183
Q

10% of pts with MG, esp older males, have ________, and most other pts have___________________.

A

thymomas

follicular hyperplasia of the thymus

184
Q

MG can usu be controlled with meds, used for what 2 reasons:

What surgery may help?

A
  • direct improvement of weakness
  • reduction of autoimmune process

thymectomy (esp w/out thymoma)

185
Q

T/F: MG is an insidious and progressive autoimmune condition.

A

false

sx may come and go, but don’t usu get worse as pt ages. For some, sx decrease after 3–5 years.

186
Q

MC thymic abn in females?

MC in males?

A
  • thymic hyperplasia - women

* thymoma (benign tumor) - men

187
Q

The classic biopsy of MG shows:

A
  • lg collections of lymphocytes
  • necrotic muscle fibers
  • pale
  • atrophic
188
Q

T/F: In the worst myasthenia crisis, paralysis of the heart occurs.

A

false - the respiratory muscles can be paralyzed, requiring assisted breathing

the heart is never affected by MG

189
Q

Basis for MG diagnosis:

A
  • Hx
  • PE
  • 2 positive dx tests
  • serological (anti-ACHR abs)
  • electrodiagnostic (nerve stim)
190
Q

Syndrome that causes progressive muscle weakness, usually first noticed in the upper legs and upper arms:

A

Lambert-Eaton syndrome (LES)

mb good strength initial, and dec w/prolonged exertion

191
Q

60% of cases of Lambert-Eaton are assoc with:

A

SCC (small cell lung cancer)

[syndrome dx may precede cancer detection]

192
Q

In LES, abs directed against _________ calcium channels in the neuromuscular jct prevent normal function, which inhibits _____ release essential for normal muscle tone.

A

presynaptic

Ach

193
Q

Dx of LES is by:

A
  • chest x-ray - poss malignancy
  • abs to calcium channels
  • nerve conduction studies
194
Q

A group of closely related, muscle-specific autoimmune diseases occuring as a result of immune mediated inflammation and vascular damage to striated muscle:
MC forms:

A

idiopathic inflammatory myopathies (IIM)

  • dermatomyositis
  • polymyositis
  • inclusion body myositis
195
Q

Definitive dx of IIM requires:

Other tests used to support dx:

A

muscle bx

  • EMG
  • MRI
196
Q

Connective-tissue dz characterized by inflammation of skeletal muscle and skin:

A

dermatomyositis (DM)

197
Q

The mechanism of injury in DM is:

A

complement-mediated damage to blood vessels in muscle and skin, with secondary atrophy and lymphocytic inflammation d/t ischemia.

198
Q

The cause of DM is unknown, but mb:

A
  • post-viral infection autoimmune reaction

* paraneoplastic syndrome

199
Q

Although DM is primarily a dz of adults, it also is observed in children, usually aged ________.

A

5-14 years

200
Q

Dx of DM is based on:

A
  • Hx of muscle weakness
  • skin involvement
  • inc CPK, ESR
  • abn EMG
  • abn muscle bx
201
Q

2 classic histopathology findings of DM:

A
  • mixed B- and T-cell perivascular inflammatory infiltrate
  • perifascicular muscle fiber atrophy
  • invading inflammatory cells
  • necrotic muscle fibers
  • regenerating muscle fibers
202
Q

A characteristic clinical finding of DM is:

A

periorbital heliotrope rash

  • symmetric
  • purple-red
  • macular eruptions of eyelids and periorbital tissues

mb Gottron’s sign

  • purple-red papular rash
  • dorsal MCPs and IP jts

mb Shawl sign

  • erythematous rash
  • shoulders, delts, neck
  • V sign - if on the ant neck
203
Q

Pts with polymyositis (PM) usu present with:

A
  • symmetric weakness
  • proximal UEs and LEs
  • neck flexors
  • muscle pain / tenderness
  • distal weakness (late)
204
Q

A poor prognostic sign of PM seen in 1/3 is:

A

dysphagia secondary to oropharyngeal / esophageal involvement

205
Q

T/F: PM has a bimodal onset, in adults and children 5-14.

A

false - that’s DM

PM is rare in under-20s

206
Q

Histopathology of PM - early? late?

A

Early -
* pale enlarged muscle fibers
* macrophages surrounding w/some invasion
Late -
* complete muscle fiber invasion by macrophages

207
Q

Muscle bx of PM classically shows ___ lymphocyte inflammation.

A

T8 lymphocytes

208
Q

Inclusion body myositis (IBM) is characterized by:

A

slow, steadily-progressive weakness and wasting of the distal and proximal muscles, most apparent in the muscles of the arms and legs.

209
Q

Muscle fiber bx of IBM is characterized by:

A
  • focal areas of WBCs and other inflammatory cell
  • deposits of amyloid-related proteins
  • inclusions (buildup of abn proteins)
  • vacuoles
210
Q

Benign tumors of striated muscle that usu present as a round mass in the region of the neck (90%) are called:

A

rhabdomyomas

211
Q

Characteristic histopathology of rhabdomyoma:

A
  • well-differentiated lg cells that resemble striated muscle
  • deeply eosinophilic
  • polygonal
  • small, peripheral nucleus
  • occasional vacuoles
212
Q

2 non-head and neck varieties of rhabdomyoma:

A
  • genital (usu female, asx)

* cardiac (peds, hamartoma)

213
Q

Malignancy that arises from embryonic mesenchymal cells with potential to develop into skeletal muscle and the MC soft tissue tumor in children:

A

Rhabdomyosarcoma

214
Q

Rhabdomyosarcomas are MC dx’d in what body region:

A
  • head and neck (ORBIT, face, scalp)
  • GU system
    but may occur anywhere
215
Q

What are the 2 MC subtypes of rhabdomyosarcoma (of 5)?

A
    • embryonal
    • alveolar
  • botryoid
  • spindle cell
  • anaplastic (undiff)
216
Q

Histopathology of rhabdomyosarcoma:

A
  • pleomorphic cells - various size, shape
  • bundles of spindle-shaped cells
  • elongated hyper-chromatic nuclei
  • poorly differentiated / anaplastic
  • mitotic figures
217
Q

~90% of cases of rhabdomyosarcoma occur in pts under ____ years, with most cases occurring in the _____ decade.

A

25

1st decade

218
Q

How many chemo txs does a myosarcoma pt need?

A

tumor

the end

Pathology IV (6 decks)

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