Pathology - part 1 Flashcards
acute inflammation
Vascular events: 1) vasoconsctriction of arterioles (neurogenic reflex for seconds), 2) arteriole dilation (histamine, NO, etc) 3) ↑ permeability of venules (histamine) -4) edema - 5) d. blood flow neutrophile (PMN): 3-4 segmented nucleus; >5: megaloblastic anemia; B12 or folic acid deficiency rolling (selectin, for weak binding) - adhension (beta2 integrins: CD11a: CD18) integrin表达在neutrophil上,bind to ICAM/VCAM on endothelial cells; ICAM/VCAM activated by IL-1, TNF
2 important platelet disorders: TTP/HUS cause? symptoms? treatement
- TTP (thrombotic thrombocytopenic purpura) 血栓性血小板减少性紫癜 [mortality: 10 - 20% even with plasma exchange!!} deficiency in vWF-cleaving metalloprotease in endothelial cells, ↑ platelet adhension to areas of endothelial injury at arteriole-capillary junctions . platelets are consumed (but NOT DIC) pentad: fever, thrombocytopenia, renal failure, microangiopathic hemolytic anemia with schistocytes (裂红细胞), CNS deficits 血小板减少性紫癜、微血管病性溶血、中枢神经系统症状、发热以及肾脏损害,并称之为TTP五联征 2. HUS (hemolytic uremic syndrome): primarily in children < 10, caused by endothelial damage at arteriole - cap junction dues to E. coli toxin clinic sym: similar to TTP, but less CNS findings, bloody diarrhea in 75% cases treatment for TTP and HUS: plasma exchange transfusion; corticosteroids
mallory bodies
damaged (ubiquinated) cytokeratin intermediate filaments in hepatocytes note: Ub labels damaged intermediate
alkalosis vs. acidosis对血K+影响?
Alkalosis: H+ moves outside of cells, K+ move in → hypokalemia acidosis: H+ moves inside of cells, K+ move out → hyperkalemia
hypertrophic cardiomyopathy
most common cause of sudden death in young people, autosomal dominant mutation in heavy chain of b-myosin and in the troponins 病理: asymmetric hypertrophy of septum, anterior MV leaflet drawn against septum (obstruct blood flow). Aberrant conduction system causes sudden death
allergic transfusion reaction: cause, clinic, Rx?
IgE-mediated Type I HSR (hypersensitivity reaction) IgE triggers mast cells release of preformed mediators: histamine, serotonin, etc urticaria + pruritis, fever, tachycardia, wheezing, potential anaphylactic shock Rx: antihistamine
ataxia-telangiectasia
AR, ATM (DNA repair mutation) thymic hypoplasia, cerebellar ataxia, telangiectasias of eyes and skin, risk of lymphoma and leukemia, ↑ of serum α-fetoprotein
anemic infarcts vs. hemorrhagic infarcts
anemic infarcts: (pale or white color): happen in solid organs with single blood supply (spleen, kidney, heart) hemorrhagic infarcts: (red color): happen in solid organs with dual blood supply or collateral circulation (lung, intestines)
aspirin overdose
symp: NS: [tinnitus 耳鸣, vertigo (眩晕), change in mental status (confusion, seizures)], hyperthermia (damage mito membrane, uncouple oxidative phosphorylation and ATP synthase), fulminant hepatitis (爆发性肝炎,除了aspirin还有acetaminophen 扑热息痛也会引起) respiratory alkalosis occur initially (12-24 hrs) due to direct stimulation of the respiratory center, then shift to metabolic acidosis with ↑ anion gap
anemia associated with chronic alcoholism?
sideroblastic anemia [铁幼粒细胞贫血; 此病也可能由VB6 deficiency, Lead poisoning引起】 defect in heme synthesis in mitochondria! BM aspirate shows “ringed sideroblasts” (drak blue iron granules around the nucleus of developing normablasts-iron trapped in mito.)
Brunton’s agammaglobulinemia
XR; mutated Tyr kinase: pre-B cells fail to mature opsonization defect: neutrophils have membrane receptors for opsonin (调理素,包括IgG, C3b fragment of complement, etc). Binding enhances neutrophil recognition and attachment to bact. sinopulmonary infections. Maternal Ab protects up to 6 months, then ↓ immunoglobins
Budd–Chiari syndrome
caused by occlusion of the hepatic veins. congestion. It presents with the classical triad of abdominal pain, ascites and hepatomegaly
Calcium function in coagulation?
bind γ-carboxylated VitK-dependent factors (VII, XI, X, II)
cancer引起endocarditis的机制?
paraneoplastic syn: sterile vegetations on the MV: procoagulant effect of circulating mucin (粘蛋白)from mucin-producing tumors of the colon and pancreas embolization can be a complication
catalase-positive organisms
can produce H2O2, but H2O2 is degraded by catalase, so the bact. are not killed by the MPO system
chronic granulomatous desease (CGD) vs. MPO deficiency (myeloperoxidase)
CGD: absent NAPDH oxidase and respiratory burst MPO deficiency: normal respiratory burst, but no MPO to convert (H2O2 + CL-) to HOCL’ (hypochlorous free radicals), which kill bact.
Churg-strauss syndrome
autoimmune medium and small vessel vasculitis. p-ANCA. 几乎所有病例都以asthma起病,发展到血管炎阶段时常有腹痛(腹部血管被累及)。多数死于eosinophilic myocarditis usually (but not always) manifests in three stages. 1) Allergic stage: marked by airway inflammation: almost all patients experience asthma and/or allergic rhinitis; 2) Eosinophilic stage: abnormally high numbers of eosinophils, which causes tissue damage — most commonly to the lungs and the digestive tract. 3)final stage: vasculitis, which can eventually lead to necrosis and is potentially life-threatening.
clinic findings in VB12 deficiency
3大症状: 1)macrocytic/pernicious anemia; 2) smooth, sore tongue with atrophy of papillae; 3) neurological disease (peripheral neuropathy; dementia, etc) 与folate缺乏鉴别:叶酸缺乏没有神经症状!!! Pernicious anemia: 1)lack of gastric acid - Ab destruction of parietal cells [chronic atrophic gastritis, ↑ incidence of gastric adenocarcinoma] , 2) Ab: against proton pump; block binding of VB12 to IF 【intrinsic factor]; block binding of VB12-IF complexes to ileal receptors 其中Ab blocking binding of VB12 to IF is most specific for diagnosis
coagulation cascade?
Extrinsic: VII Intrinsinc: XII → XI → IX → VIII common pathway: X → (Xa + V + PF3 + Calcium) 这个complex叫做“prothrombin complex”, convert prothrombin to thrombin thrombin: 1) convert fibrinogen to fibrin monomer (still soluble) + fibrinopeptides A+B 2) activate factor XIII to cross-link fibrin (become insoluble)
coagulation cascade?
Extrinsic: VII Intrinsinc: XII → XI → IX → VIII common pathway: X → (Xa + V + PF3 + Calcium) 这个complex叫做“prothrombin complex”, convert prothrombin to thrombin thrombin: 1) convert fibrinogen to fibrin monomer (still soluble) + fibrinopeptides A+B 2) activate factor XIII to cross-link fibrin (become insoluble) 3) activate VIII:C in intrinsic pathway
coagulation necrosis
凝固性坏死, preserve structural outlines of dead cells mechanisms: 1) denaturatio of E. and proteins (accumulation of lactate or heavy metals such as lead, mercury, UV) 2) inactivation of E prevents dissolution (autolysis) of the cells 镜下特征: 1)indistinct outline of cells within dead tissue 2) absent nuclei or karyolysis ( fading of chromatin, 核溶解) e.g.: MI
coarctation (狭窄)of aorta: 分为infantile vs. adult2型
- infantile coarctation: associated with Turner syn, occurs between the subclavian A (锁骨下A) and ductus arteriosus 2. adult coarctation: constriction of aorta distal to the ligamentum arteriosum; 1) blood flow into the proximally located branch vessels ↑ (i.e., ↑ cerebral blood flow ⇛ ↑ risk of berry aneurysms), ↑ upper extremity BP, dilation of aorta and aortic valve ring (regurgitation) 2) blood flow below the constriction ↓ (i.e., ↓ renal blood flow ⇛ activate the renin -Ang -aldosterone system to ↑ BP ), ↓ BP in the lower extremity, leg claudication (pain in calf or buttocks when walking) 成人主动脉狭窄的特点:disparity between upper and lower extremity BP > 10 mmHg
common variable immunodeficiency
adult immunodeficiency disorder B cells fail to mature into plasma cells Sinupulmonary infections, GI infections, autoimmune disease, ↓ Ig
confirm SLE
主要靠血清检验确诊:serum ANA : anti-ds-DNA and anti-Sm Ab (highly specific, very few false-positive) anti-Ro Ab are positive for 25- 50% cases Note: lupus erythemastosus cells are NOT specific for SLE! (these are neutriphils containing phagocytosed altered DNA) Libman-Sacks endocarditis: associated with SLE, sterile vegetations located over the MV surface