Term
| what is characteristic of takayasu arteritis? how does it progress? |
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Definition
| HLA A24, B52, & DR2. giant cells may be patchy and cause necrosis of the media, and also like giant cell, blindness may occur - but unlike giant cell, coldness/numbing of extremities, HTN, and neurologic defects are also seen with takayasu arteritis. it may progress rapidly or slowly and affects medium to large arteries. |
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Term
| what on an angiogram would be characteristic of takayasu's? |
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Definition
| a lack of filling in certain areas, which can cause neurologic defects if the aorta is affected and tingling/coldness/decreased hand pulse if carotids and brachiocephalic arteries are affected |
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Term
| how would a vessel affected by takayasu's appear macroscopically? |
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Definition
| a very narrowed lumen (similar wall thickening as atherosclerosis) |
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Term
| how would a vessel affected by takayasu's appear microscopically? |
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Definition
| giant cells, fragmentation of the media, and infiltration by neutrophils |
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Term
| what is infectious arteritis caused by? |
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Definition
| infectious entry by TB or other bacterial pneumonia, as well as some fungi that *directly invade the vessel wall |
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Term
| what can infectious arteritis lead to? |
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Definition
| mycotic aneurysms: weakening of the vessel wall which can potentially cause a thrombus and lead to an infarction, which is potentiated and worsened by vessel narrowing |
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Term
| what is seen microscopically with infectious arteritis? |
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Definition
| polymorphonuclear cells, possible isolated organisms, and thrombi |
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Term
| what other disorders can cause vasculitis? |
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Definition
| RA, SLE, malignancy/systemic illness - all of which will often resemble the histology of PAN |
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Term
| what is kawasaki disease? |
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Definition
| kawasaki disease, also known as mucocutaneous lymph node syndrome is an autoimmune disease where oral mucosa, conjunctiva, palms/soles, and coronary arteries are attacked by the pt's own immune system |
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Term
| who does kawasaki syndrome affect? |
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Definition
| typically, kawasaki is seen in children and is the leading cause of aquired heart disease |
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Term
| what do pts with kawasaki syndrome present with? |
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Definition
| erosions of the oral mucosa (particularly red), rash on palms/soles of feet, characteristic cervical lymphadenopathy, fever, conjunctivitis, and artery involvement |
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Term
| what is the most serious presentation of kawasaki syndrome? |
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Definition
| involvement of the coronary arteries, which can lead to a weakening of the vessel walls, cause dilation and significant inflammation -> decreased blood flow/infarction (*how young kids might have an MI) |
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Term
| what is kawasaki similar to microscopically? |
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Definition
| PAN, w/transmural involvement, some fibrotic lesions (chronic), and obstructive intimal narrowing |
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Term
| what are some cellular associations with kawasaki syndrome? |
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Definition
| T cell/macrophage activation, various cytokines, B cell hyperactivities, autoantibodies to endothelial/smooth muscle cells (why heart is affected) |
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Term
| what is the mnemonic for kawasaki disease? |
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Definition
| 3 Ks: Kids, Konjunctivitis, and Koronary |
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Term
| what is wegener granulomatosis? |
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Definition
| necrotizing vasculitis of small-medium arteries which can lead to necrotizing granulomas of the upper respiratory tract, focal necrotizing glomerulonephritis, and inflammatory sinusitis from mucosal granulomas |
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Term
| what are the effects of granulomas associated with wegener's? |
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Definition
| granulomas are necrosis surrounded by lymphocytes, plasma cells, macrophages, and giant cells. these ulcerative lesions in/on the nose can cause “saddle” deformities as the break down of cartilage forms indentations - can involve pharynx, hard, and soft palates when these coalesce to form larger nodules and cavitation |
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Term
| what does wegener's granulomatosis mimic? |
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Definition
| TB (though not caseating granulomas) |
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Term
| what is the most likely cause of wegener's? |
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Definition
| problematic immunologic mechanisms |
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Term
| what are two significant effects of wegener's? |
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Definition
| significant alveolar hemorrhage (hemoptysis), focal to diffuse glomerular lesions that can progress to cresentric glomerulonephritis |
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Term
| who does wegener's affect most commonly? |
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Definition
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Term
| how do people present with wegener's? |
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Definition
| lesions, pneumonia from granulation tissue, and necrotizing areas, sinusitis/nasopharyngeal lesions |
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Term
| what is seen in 80% of wegener's cases? |
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Definition
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Term
| what is seen histologically with wegener's? |
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Definition
| granulation tissue, significant areas of necrosis (to the point of an ill-defined lumen), all lymphocytes, plasma cells, necrotic debris, and giant cells |
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Term
| what is thromboangiitis obliterans? |
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Definition
| thrombus formation, vessel inflammation, and obliteration/occulusion of vessels in medium-small arteries in the legs due to hypersensitivity to tobacco extracts |
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Term
| what else is thromboangiitis obliterans known as? |
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Definition
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Term
| how doe lesions in thromboangiitis obliterans appear? |
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Definition
| sharply segmented acute or chronic vasculitis (similar to PANS), separated by normal vessels |
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Term
| how do pts with thromboangiitis obliterans appear physically? |
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Definition
| they present with pain in walking, there may be (possibly extensive) microabscesses superimposed with thrombi, occluded vessels can lead to chronic ischemia and infarction of lower extremities (severe pain, gangrene, etc.) |
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Term
| what is the demographic for pt with thromboangiitis obliterans? |
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Definition
| male smokers under 35 as well as HLA-A9 and HLA-B5 |
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Term
| what would an angiogram for pts with thromboangiitis obliterans look like? |
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Definition
| vessels with lacking flow or partially obstructed |
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Term
| how does thromboangiitis obliterans appear histologically? |
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Definition
| abundant inflammatory cells, neutrophils, microabscesses with superimposed thrombus-vessel wall lining shows the elastic membrane: should have lumen in center but completely occluded by thrombus and inflammation |
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Term
| what are cavernous hemangiomas? |
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Definition
| these are common on skin & mucosal surfaces and degree of danger correlates with location, for ex: associated bleeding would be very problematic if a cavernous hemangioma appeared in the brain or retina. von Hippel-Lindau disease is an ex of this occuring in the brain |
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Term
| how do cavernous hemangiomas appear physically? |
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Definition
| 2-3 cm red/blue, spongy and blood-filled with *sharp, demarcated borders (indicative of benign lesion). they have large, cavernous, blood filled lesions with endothelial linings and abnormal architecture |
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Term
| what is a capillary hemangioma? |
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Definition
| these benign/non-infiltrative lesions are smaller than cavernous hemangiomas, and are composed of closely packed capillaries w/connective tissue - usually involving the skin and subcutaneous tissue. they can range from red to blue and from a few mm to several cm |
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Term
| how do capillary hemangiomas present? |
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Definition
| they may be seen on the mucosal surface of the tongue, as well circumscribed lesions with significant bleeding - but no other worrying factors |
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Term
| how do capillary hemangiomas differ from cavernous hemangiomas histologically? |
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Definition
| they have smaller blood vessels with very prominent connective tissue surrounding the stroma |
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Term
| what is a glomus tumor? where are they seen? |
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Definition
| this vascular tumor is derived from neurovascular bodies, so it can be painful. they tend to be involved with under fingernail and toenail beds and are red-blue in color. they consist of branching and vascular channels and a bx is not always necessary |
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Term
| what is a hemangioendothelioma? |
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Definition
| this is an intermediate grade between benign and malignant tumors, there are discernable vascular channels, *well differentiated endothelial cells, and can be locally aggressive and infiltrating - can lead to further destruction if not removed (need to be fully removed to prevent recurrence) |
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Term
| how do hemangioendotheliomas appear histologically? |
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Definition
| vascular channels may not be initially obvious, but can be stained for w/factor 8. they have closely packed cells, vessels, and are fairly well differentiated |
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Term
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Definition
| a vascular tumor of mesenchymal origin, it be poorly differentiated enough that it doesn't even appear as a vascular tumor, and vascular channels may not be apparent. it may resemble a rhabdomyosarcoma or other mesenchymal malignancy |
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Term
| how do angiosarcomas appear? |
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Definition
| they typically have anaplastic spindle/cigar shaped cells, poorly formed vascular channels (RBCs only may be visible), and abnormal endothelial cell linings. the cells are not well differentiated, their polarity is messed up, they tend to have a high N:C ratio, clumped chromatin, mitotic figures, and haphazard growth. they may appear as completely solid. |
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Term
| what are causative agents of angiosarcomas? |
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Definition
| vinyl chloride (industrial usage), arsenic (industrial usage), and thorotrast (old contrast medium) **VAT |
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Term
| how do angiosarcomas appear grossly? |
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Definition
| disfiguring, irregular, granular involvement on the extremities, in the soft tissue or deeper in the viscera |
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Term
| will an angiosarcoma appear as such immediately? |
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Definition
| no, it could be skeletal tissue lesion, smooth muscle, fibrotic lesion, etc.- need special stain with antibodies to target structural components |
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Term
| how would an angiosarcoma appear on an angiogram? |
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Definition
| poorly differentiated, indistinct vascular formation |
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Term
| where is a common location for angiosarcomas? |
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Definition
| in the liver, common b/c it is a highly vascular organ |
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Term
| what is the classic form of kaposi sarcoma? endemic? |
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Definition
| classic form: that seen in older men, ashkenazi jews, and people of mediterranean descent. endemic form: that seen in african men and children |
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Term
| what is the epidemic form of kaposi sarcoma? |
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Definition
| that caused by HIV, HHV-8 |
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Term
| how do kaposi sarcomas appear? |
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Definition
| papular, plaque-like, nodular or patchy. they enlarge as they progress, and can grossly mimic a benign lesion, small nodule, or angiosarcoma |
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Term
| what disease is kaposi's sarcoma an indentifier for? |
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Definition
| AIDs (which drops immune ability allowing HHV-8 to kick off kaposi) |
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Term
| how do kaposi sarcomas appear histologically? what is helpful in dx? |
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Definition
| not all different from angiosarcoma, examination shows large spindle shaped cells, high N:C ratio, some vascularity, not normal appearance. herpes virus occlusions are useful in dx |
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Term
| what do people with kaposi sarcoma also present with? |
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Definition
| cough, lymphadenopathy, abdominal pain |
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Term
| how does kaposi's sarcoma present grossly? |
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Definition
| a dark blotch, sort of psoriasis-like |
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Term
| how does kaposi's sarcoma progress? |
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Definition
| it initially causes nodularity and enlarged patches which can coalesce and form raised nodules, associates with abnormal vessels |
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Term
| what is von-hippel lindau disease? |
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Definition
| an autosomal dominant disease associated with the *VHL gene on chromosome 3 (tumor suppressor gene), it has been linked to hemangioblastomas, and cavernous hemangiomas of the cerebellum, brainstem, retina or adenomas/cysts of the liver, kidney, and pancreas (depending on the location of the lesion) |
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Term
| what are symptoms of van hippel lindau disease? |
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Definition
| ataxia, vision loss, *increased incidence of renal cell carcinoma and pheochromocytoma |
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Term
| what is osler-weber-rendu syndrome? who is it seen in? |
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Definition
| an autosomal dominant mutation in TGF binding proteins causing a dilation of vessels in the GI, urinary tract, and other areas. it can lead to skin hemorrhage, epistaxis, and normocytic/normochromic anemia. it is seen more in utah mormons. |
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Term
| what is sturge-weber syndrome? |
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Definition
| a congenital disorder associated with an ipsilateral port wine stain of the face, which can cause glaucoma, hemangiomatous masses of the meninges, and severe mental retardation, seizures, and retinal detachment |
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