Pathology of MSK non-tumor diseases Flashcards

1
Q

Osteogenesis imperfecta

A
  • Pts have long arms relative to legs, multiple fractures in the legs from early life that healed improperly
  • They have thin, weak tibias w/ little calcification (abnormal osteoid matrix)
  • Usually is dominant (less severe) form
  • Pts do not produce normal amount of osteoid so the bones (especially weight-bering ones) are susceptible to fractures
  • Can also see blue sclera syndrome
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2
Q

Osteomyelitis 1

A
  • Irregular lesion w/ sclerosis (encapsulation), suggests benign process
  • Histologically see inflammatory cells (PMNs) eroding the bone
  • Were recruited there due to an abscess from bacteria (usually staph aureus)
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3
Q

Osteomyelitis 2

A
  • The bone is not viable, and is called sequestrum: necrotic bone detached from blood supply (looks dark)
  • The new bone being formed around the lesion is the involucrum: body’s attempt to make new bone around abscess (is the sclerotic region)
  • Looks irregular and w/ pockets of bone formation
  • Common complication: chronic suppurative osteomyelitis (draining sinuses btwn infected bone and skin), squamous cell CA and secondary amyloidosis
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4
Q

Pagets disease 1

A
  • Pts (often men) usually over 40 have pain at site of lesion, w/ markedly elevated serum alk phos levels (w/o having other liver nzs or PTH elevated)
  • Bone is thickened and deformed, a mix of sclerosis and lysis
  • Can see cement (mosaic) lines: lines btwn resorbed and newly formed bone
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5
Q

Pagets disease 2

A
  • There is normal calcification but abnormal resorption and production
  • Variability in thickness of tabeculae
  • Abnormal activity of both osteoclasts and osteoblasts, but osteoblast activity eventually is higher (phase 3), leading to thick bones and increases alk phos
  • Phase 1: increased osteoclast activity
  • Phase 2: mixed osteoclast and osteoblast activity both increased
  • Complications: hemodynamic derrangement (due to hypervascular bone) and secondary osteosarcoma
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6
Q

Pott disease (TB infection of vertebrae)

A
  • Pts have soft tissue lesions usually in the lower back, and are PPD positive (due to TB), often immunocompromised
  • The mass is of lymph nodes and other soft tissue, there are granulomas of cartilaginous disks and collapsed vertebras
  • Histologically, the masses contain lymphocytes and plasma cells, zones of degeneration (necrosis), some giant and epithelioid cells
  • Positive for acid fast stain (TB), can culture and PCR for Dx
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7
Q

RA 1

A
  • Chronic process of symmetrical joint destruction, 25% show rheumatoid nodules (subQ nodules on extensor surfaces)
  • Joints are warm, tender, edematous, stiff
  • There is pannus (fibrous outgrowth/thickening of synovium)
  • The rheumatoid nodules show granulomas of chronic inflammation
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8
Q

RA 2

A
  • Synovial fluid has high abundance of PMNs, which are drawn to the joint by chemokines/complement
  • Chemokines released by plasma cells and lymphocytes, which are found in the joint tissue
  • These are drawn to the joint due to the immune complexes formed from the RF-IgG complexes that are deposited in the joints (autoimmune) and phagocytosed by synoviocytes
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9
Q

Osteoarthritis

A
  • Leads to gradually progressive pain and limited range of motion in the affected joints (due to aging), NOT due to inflammation
  • There can be deformities of subchondral bones (osteophytes indicating new bone formation), loss of articular cartilage and sclerosis
  • The loss/fragmented articular cartilage also can lead to bone resorption
  • Fibrocartilage shows uneven distribution of chondrocytes
  • There is clefting/fraying and then loss of articular cartilage
  • Some areas of thick bone, other areas of weak bone
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10
Q

Fibrous dysplasia 1

A
  • Circumscribed uneven thinning of bone (ground glass appearance), progressive replacement of a localized area of bone by abnormal proliferation of fibrous tissue
  • Middle of lesion is jelly-like substance (fibrous tissue + hemorrhage)
  • Histologically there is osteoid getting calcified around edges, bone haphazardly formed but not at the stress lines (not lined by osteoblasts
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11
Q

Fibrous dysplasia 2

A
  • Non-linear formation of bone, randomly organized into circles and semicircles (woven, not lamellar bone)
  • Two types: monostotic (usually on ribs, causing deformities and pain/fracture) and polyostotic (usually affects face, also causing deformity and pain/fractures)
  • The Rx is bone grafts
  • Not a malignant process
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12
Q

Osteoporosis 1

A
  • Decrease in total mass of bone with micro-architectural deterioration
  • Ratio of mineralized:non-mineralized (osteoid) bone is normal
  • Bone loss produces pain and increases chance of fracture
  • Due to dysregulation of osteoclast formation/function via RANK pathway
  • Very common, especially primary osteoporosis in elderly (resorption outpaces formation)
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13
Q

Osteoporosis 2

A
  • Secondary osteoporosis: due to immobilization, drugs, malnutrition/absorption, or endocrine d/o
  • Affects all bones in body, but pain primarily in weight bearing bones
  • Ca, PO, alk phos values generally normal
  • Histology: thinned cortical and trabecular bone (changes may be subtle, or very apparent)
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14
Q

Osteomalacia

A
  • AKA rickets (when in children), structural abnormality of bone caused by defective mineralization of osteoid (osteoid is present in normal or increased amounts but not calcified)
  • Due to lack of vit D (deficiency), which is required for mineralization of osteoid
  • Rickets can cause deformities of bone and osteopenia, but osteomalacia only causes osteopenia (no deformities)
  • Lab values: increased alk phos, decreased Ca, PO
  • Histology: trabecular are characterized by peripheral “seams” of uncalcified osteoid
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15
Q

Gout 1

A
  • Urate crystals are deposited in connective tissue, associated w/ increased uric acid in serum
  • Primary: mainly elderly men w/ increased uric acid production, decreased uric acid excretion or both
  • Secondary: rapid cell destruction releases uric acid (chemo), and decreased excretion (renal failure)
  • Acute gouty arthritis: deposition of sodium urate crystals in synovial membrane
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16
Q

Gout 2

A
  • Chronic tophaceous gout: deposition of large masses of urate crystals (tophi) in joints and soft tissues
  • Histology of acute gouty arthritis: hyperplasia and acute inflammation of synovium, needle-shaped, elongate, birefringent crystals in fluid and WBCs
  • Histology of chronic tophaceous gout: hyperplastic synovium with basophilic masses of crystals (tophi), chronic inflammation, and foreign body rxn around tophi
17
Q

Achondroplasia, osteopetrosis, marfan syndrome

A
  • Achondroplasia: mutation in fibroblast GF 3 (FGFR3) leads to defect in cartilage synthesis and failure of growth at epiphyseal plates (dwarfism)
  • Osteopetrosis: defect in osteoclast reabsorption leading to dense, brittle bones. Causes limited marrow space
  • Marfan’s syndrome: mutation in fibrillin leads to tall stature, long finger, CV problems