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33 Cards in this Set
- Front
- Back
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Plasma Cell Neoplasms
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A group of disorders involving cloncal expansion of Ig-producing cells and formation of a solid tumor with these cells...this results in monocloncal Ig and Ig component production and appearance of these monocloncal elements in serum and urine.
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Monoclonal gammopathy
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The excess immunoglobulins produced are usually functional. Considered benign and potential to convert to multiple myeloma.
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Multiple myeloma
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1. Comprised of myeloma cells (the cells of plasma cell tumors in marrow). The myeloma cells produce excess intact IgG or IgA and excess light chains, osteoclast activating factor (IL6), which produces osteolytic lesions, IL-1.
2. Bence-Jones proteins - free monoclonal K or gamma chains 3. amyloidosis 4. hypercalcemia 5. anemia and leukocytopenia 6. increased susceptibility to infections 7. M-spike on electrophoresis, represents high amount of monocloncal protein present |
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Waldenstrom's
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Primary macroglobulinemia....males > females. comprised of flame cells (eosinophilic neoplatic plasma cells), hyperviscosity syndrome due to the overproduction of IgM. See fatigue, weakness, skin and mucosal bleeding, headache, visual problems, and neurologic problems.
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Phlebothrombosis risk factors
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Virchow's triad: blood stasis, hypercoagulability, endothelial cell damage.
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Trousseau's syndrome
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migratory venous thrombosis that may occur as an extreme example of phlebothrombosis.
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DVT
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Results from benous thrombosis of the veins of the lower extremities of pelvis. If untreated, it may embolize to form a life-threatening pulmonary embolus.
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4 types of arterioschlerosis
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Atherosclerosis, Monckebergs, Arterioloschlerosis, Arteriolosclerosis
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Atheroschlerosis
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Due to high LDL-cholesteral, contains foam cells (cholesteral-laden macrophages), increases the risk for CVA, PVD, Ischemic heart disease, mesenteric ischemia, and aortic aneurysm. The lesion is an therosclerotic plaue, the lesions form primarily in large and medium-sized arteries (aorta, carotid arteries, iliac arteries, coronary arteries). The plaue is formed by the deposition of LDL cholesteral into the inteima of blood vessels, with subsequent accumulation of foam cells and inflammatory mediators. The plaues have three major effects: decrease luminal dimensions, lead to eventual atrophy of media, leading to blood vessel wall weakening. When disrupted, attract platelets that form an overlying platelet clot, the platelet clot either completely occludes the blood vessel or breaks off to embolize downstream to where it will obstruct blood flow.
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Monckebergs
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Benign...calcium deposits in blood vessel media produce lumps. Most common to radial and ulnar arteries.
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Arteriolosclerosis
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Results from malignant hypertension, affects small arteries and arterioles. Histologically is characterized by onion skin hyperplasia (cross-section of the blood vessel demonstrates multiple layers of the media, like the layers of a cross sectioned onion).
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Arteriolosclerosis
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Results form inflammation and "use". Etiologies include: HTN, old age, diabetes mellitus. Overtime, the blood vessel basement membrane becomes thick, causing narrowing of the lumen. It affects small arteries and arterioles. Is nephrosclerosis when associated with kidney.
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Vasculitis Types
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Arteritis (inflammation of blood vessels except veins) and phlebitis (inflammation of veins).
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Hypersensitivity arteritis
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Type III hypersensitivity to drugs, tumors, HCV, EBV, HIV. +p-ANCA. Because of small vessel involvement, affects primarily the blood vessels of skin, muscles, brain, and kidney. Palpable purpura +/- macules, papules (especially on buttocks) - painful, pruritic
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Henock-Schonlein purpura
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A variant of hypersensitivity arteritis. A vasculitis with IgA immune complex deposition on small vessel walls. Occurs primarily in children. Palpable purpura, arthralgias, blood diarrhea, maybe glomerulonephritis.
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Polyarteritis nodosa (PAN)
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A transmural necrotizing inflammation of arterial walls. White blood cells invade blood vessel walls to cause inflammatory damage. Occurs secondary to HBV, HCV, or hairy cell leukemia. 30% of patients have hepatitis B at time of DX. Widely scattered involvement, with sporadic repeat occurrence. Fever, abdominal pain and melena, renal disease with hypertension, neuritis of motor neurons, diffuse myalgia, fatal in most untreated cases.
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Microscopic polyangiitis
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Involves small blood vessels...histologically similar to polyartertitis nodosa. Usually initiated by a reaction to drugs. +p-ANCA. Causes severe glomerulonephritis and pulmonary capillaritis. Palpable purpura, hemoptysis, hematura, myalgia.
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Thromboangiitis obliterans
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A hypersensitivity reaction to tobacco. Affects primarily the tibial and radial arteries: collections of neutrophils surrounded by granulomas form in the vessel walls. Causes blood vessel destruction, leading to severe pain in the distal imb that progresses to gangrene.
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Giant Cell arteritis
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Granulomatous inflammation of vessel walls. Can affect aorta, carotid and vertebral arteries, and temporal artery. If it occurs in the temporal arteries, it is a temporal arteritis. TETRAD: Fever, fatigue, temporal headache, hi ESR. May also cause jaw claudication. If left untreated, may cause sudden monocular blindness.
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Polymyalgia rheumatica
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Systemic manifestation of giant cell arteritis. flu-like symptoms with arthralgia and myalgia. Fever and very high ESR, may lead to limb claudication, CVA, MI, aortic aneurysm, and visceral organ infarction.
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Wegener's Granulomatosis
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Granulomatous development in vessel walls. +c-ANCA. Sinusitis, glomerulonephritis, lung lesions. Often presents with sysmptoms associated with sinusitis and resulting sinus polyp formation.`
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Takayasu Arteritis (Aortic Arch Syndrome)
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A granulomatous vasculitis. Causes thickening of aortic arch with huge granuloma formations. Peak age: 15-40 years old, with predilection for females of asian descent. Night sweats, chest pain, and weak or absent peripheral pulse, eventual organ ischemia occurs.
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Kawasaki disease
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Hisotlogically similar to PAN. Believed to be triggered by viral illness. Fever, conjunctivitis, maculopapular rash, coronary artery aneurysms, oral mucosal erthema and cervical lymph node enlargement also occurs. Usually occurs at less than four years of age. First line treatment is IV immunoglobulins and aspririn.
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Churg-Strauss
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Features granulomas and PAN-like histologic features in the vessel wall and massive eosinophil invascion both in vessel wall and surrounding tissues. +pANCA. Affects mostly blood vessels of skin, nerves, muscle, lung, and heart. Causes severe, recurrent astham like attacks, lung infiltrates, death usually occurs by way of coronary vessel destruction +/- myocarditis. Mostly confined to occuring in patients with a history of asthma.
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Behcet's Synrdome
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Not an arteritis, a vasculitis of veins and venules. Recurrent painful oral and genital ulcerations (looks like herpes). May cause life-threatening brain and GI damage, high risk for DVTs and PEs. Affects thos eliving in or descendent from the Mediterranean, middle east, and far east. Blacks not affected. Males an dfemales affected equally, but males get MUCH more severe diseases. + anti-human oral mucosa antibodies.
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Atherosclerotic aneurysm
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Wall thinning due to medial destruction secondary to intimal plaques. Due to elevated LDL-cholesteral.
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Hyaline arterioloschlerotic aneurysm
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Thickened basement membrane of hyaline arterioloschlerosis eventually leads to medial atrophy, allowing for eventual ectasia and aneurysm. Asossicated wiht those older than 50, particularly more common in those with diabetes mellitus.
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Syphilitc aneurysm
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Occurs in tertiary syphilis. Treponema pallidum causes an obliterative endarteritis of the basa vasorum of the throacic aorta. Histologically, causes ischemia dn atrophy of media...the shrinkage causes the intima to buckle, leading to an appearance of tree-barking of the intima. May result in aotic regurgitation.
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Berry aneurysm
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Most common type of intracranial aneurysm. Aneurysm on the circle of willis. More common in those with adult Polycystic Kidney Disease. May rupture, especially at times of increased blood pressure, resulting in a subarachnoid hemorrahage.
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Aortic Dissection
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The intima tears and split occurs between laminar planes of media, allowing for collection of blood within the aortic wall. Can rupture to cause massive hemorrhage and death or can dissect towards arterial branches, causing their occlusion.
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Micro aneurysm
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small cerebral aneurysm due to hypertension or small retinal aneurysms secondary to diabetes
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Mycotic aneurysm
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due to infections of vessel wall, usually bacterial. The infection damages the media, allowing for wall weakening.
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aneurysms of the aortic arch can cause....
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Dyspnea, due to compression of respiratory structures. Recurrent, persistent cough due to pressure on recurrent laryngeal nerves. Heart failure if the aneurysm has caused aortic regurgitation. Rupture and death.
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