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67 Cards in this Set
- Front
- Back
Monoclonal gammopathy
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Excess functional immunoglobulins
potential to convert to MM |
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Excessive osteoclast activating factor seen in MM leads to
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Osteolytic lesions
(IL-6) (IL-1 as well) |
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Bence-Jones protein seen in
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MM
free monoclonal kappa or gamma chains (light chains) |
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Other things seen in MM
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-amyloidosis (tissue deposit light chains)
-hypercalcemia -anemia & leukocytopenia -increased susceptibility to infections -M-spike |
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Tests for MM
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UPEP
SPEP (M-spike on electrophoresis) |
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Pathology in Waldenstrom's disease
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overproduction of IgM
--> hyperviscosity syndrome (fatigue, weakness, skin & mucosal bleeding, HA, visual, neurologic problems) |
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Histologically seen in Waldenstrom's
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Flame cells
(eosinophilic neoplastic plasma cells) |
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Virchow's triad
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-Stasis
-Hypercoagulability -Endothelial cell damage |
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Trousseau's syndrome
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Migratory venous thrombosis
|
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Path MM
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excess intact IgG or IgA
excessive light chains |
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Path atherosclerosis
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high LDL-cholesterol
-foam cell (cholesterol-laden macrophage) -deposition large & medium arteries in intimal layer |
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Atherosclerosis increases risk of
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CVA
PVD Ischemic HD Mesenteric ischemia Aortic aneurysm |
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Monckeberg's - path & common places
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Ca deposits in blood vessel media
Common to radial and ulnar a. |
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Cause arteriolosclerosis (hyperplastic)
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malignant HTN (SBP > 210 or DBP > 150)
|
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What vessels does arteriolsclerosis (hyperplastic) affect?
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Sm arteries and arterioles
|
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Histological arteriolosclerosis (hyperplastic)
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Onion skin hyperplasia
(medial hyperplasia --> narrowing of lumen) |
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Cause arteriolosclerosis (hyaline)
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Inflammation & use
(HTN, aging, DM) |
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Histological arteriolosclerosis (hyaline)
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BM thickened
(subluminal fibrosis) --> weakening elastic laminae --> media atrophy |
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Vessels affected by arteriolosclerosis (hyaline)
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Sm arteries & arterioles
Kidney - nephrosclerosis |
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What arteritises are associated w/ Lg vessels
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Giant cell
Takayasu |
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What diseases are affected by medium vessels arteritis
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Polyarteritis nodosa
Kawasaki Thromboangiitis obliterans |
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What diseases are affected by small vessel arteritis
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Wegener's
Henoch-Schonlein Hypersensitivity arteritis Churg-Strauss Microscopic polyangitis Thromboangiitis obliterans |
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What type of hypersensitivity is hypersensitivity arteritis? Where does it affect?
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Type III
SKIN (mostly) muscles brain kidney |
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Causes of hypersensitivity arteritis
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Drugs (PEN)
Tumors HCV EBV HIV |
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Ab in hypersensitivity arteritis
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p-ANCA
|
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S/S hypersensitivity arteritis
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palpable purpura (+/- macules)
papules (buttocks) Painful pruritic |
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Henoch-Schonlein purpura
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(variant of hypersensitivity arteritis)
vasculitis of IgA immune complex small vessel walls Mostly kids |
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S/S henoch-schonlein purpura
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palpable purpura (buttocks esp.)
Arthralgias Bloody diarrhea +/- glomerulonephritis |
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Polyarteritis nodosa (PAN)
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transmural necrotizing inflammation arterial walls
-WBC invade blood vessel walls --> inflam. damage |
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PAN secondary to
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HBV
HCV Hairy cell leukemia |
|
S/S PAN
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Fever
Abd. pain & melana Renal dz (w/o glomerulonephritis) HTN neuritis of motor neurons diffuse myalgia ***FATAL if untx |
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Microscopic polyangiitis
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Small blood vessels
Transmural necrotizing inflam. arterial wall p-ANCA |
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Cause of microscopic polyangiitis
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Drugs (PEN)
|
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S/S microscopic polyangiitis
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palpable purpura, hemoptysis, hematuria, myalgia
|
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Microscopic polyangiitis leads to
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Severe glomerulonephritis (-->Rapidly progressive glomerulonephritis)
Pulmonary capillaritis |
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Thromboangiitis obliterans
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(Buerger's disease)
hypersens. to tobacco (< 35 yo jewish male) |
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Path thromboangiitis obliterans
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Tibial & radial arteries
Neutrophils surrounded by granulomas form in vessel wall |
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Thromboangiitis obliterans leads to
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blood vessel destruction
severe pain distal limb --> progessive to gangrene |
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Giant cell arteritis
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granulomatous inflammation of vessel wall
(affect aorta, carotid, vertebral a., temporal a.) Female > 50 yo |
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Temporal arteritis
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Giant cell arteritis in temporal a.
Tetrad - fever, fatigue, temporal HA, high ESR, jaw claudication |
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What is systemic manifestation of giant cell arteritis
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Polymyalgia rheumatica
|
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S/S polymyalgia rheumatica
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Flu-like
arthralgia & myalgia fever, high ESR |
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Wegener's granulomatosis
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granulomatous development in small vessels
|
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Ab Wegener's granulomatosis
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c-ANCA
|
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Clinical Wegener's granulomatosis
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Sinusitis (sinus polyps)
Glomerulonephritis Lung lesions |
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Takaysau arteritis
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thickening aortic arch w/ huge granulomas
|
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Peak age & characteristics of pt w/ takaysau arteritis
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15-40 yo
Female Asian |
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S/S Takaysau arteritis
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Night sweats
CP Weak or absent peripheral pulse |
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Kawasaki disease path? trigger?
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(mucocutaneous LN syndrome)
similar to PAN - transmural necrotizing Viral trigger |
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S/S Kawasaki
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Fever
Conjunctivitis Maculopapular rash (hands & soles) Coronary a. aneurysms Oral mucosal erythema Cervical LN enlargement |
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Typical pt w/ Kawasaki disease
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usually < 4 yo
Japanese |
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First line tx Kawasaki disease
|
IV immunoglobulins
ASA (even though pt typically children) |
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Churg-Strauss path?
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Granulomas
PAN-like histologic features massive eosinophil invasion |
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Ab Churg-Strauss
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p-ANCA
|
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Churg-Strauss - Affects blood vessels where
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Skin
Nerves Muscle LUNG HEART |
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S/S Churg-Strauss
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severe, recurrent asthma-like
lung infiltrates coronary vessel destruction +/- myocarditis (death) typically pt w/ hx asthma |
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Behcet's syndrome path? Ab?
|
vaculitis v. and venules
Ab - anti-human oral mucosa Ab |
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S/S Behcet's syndrome
|
recurrent painful oral and genital ulceration (looks like herpes)
+/- uveitis/iritis, artheritis knee |
|
Outcome Behcet's syndrome
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life-threatening brain & GI damage
high risk DVTs & PEs |
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Population affected by Behcet's syndrome
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Mediterranean
Middle east Far east |
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Aneurysm that occurs w/ syphilis
|
(luetic)
endarteritis of vasa vasorum Thoracic aorta --> ischemia & atrophy media --> media shrinks --> intima buckles ("tree-barking") Can lead to aortic regurg |
|
Aneurysm of circle of willis? associated w/ ___ dz?
|
Berry
adult Polycystic Kidney disease Rupture = subarachnoid hemorrhage |
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Aortic dissection risk factors
|
HTN
Marfans |
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Path aortic dissection
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intima tears --> split b/t laminar planes of media
blood collects w/i aortic wall |
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Micro aneurysm
|
Cerebral - HTN
Retinal - DM |
|
Mycotic aneurysm
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d/t infections of vessel wall
--> damage media |
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Aneurysms of aortic arch can cause
|
-dyspnea (compression resp. structures)
-recurrent, persistent cough (pressure on recurrent laryngeal n.) -HF (aortic regurg) -rupture & death |