Pathology 1 - Block 1 - Part 4

davidwurbel7's version from 2016-04-12 18:56

Hemodynamic Disorders Part 2

Question Answer
Injury to endothelium exposes the subendothelial, vWF, and collagen, which promote platelet adherence and activationPrimary Hemostasis(Platelet Plug)
Tissue factor is exposed. Tissue factor is a pro-coagulant that is normally expressed by subendothelial cells. Tissue factor binds and activates Factor VII. This triggers a cascade of events that results in Thrombin generation. Thrombin cleaves circulating Fibrinogen into insoluble Fibrin. Fibrin creates a meshwork, and is a potent activator of plateletsSecondary Hemostasis
(Insert vWF, Gp IIb/IIIa, Intrinsic and Extrinsic cascade)
Question Answer
The intrinsic and extrinsic cascade pathway leads to the production of thisThrombin
Thrombin cleaves this to produce fibrinFibrinogen
Fibrinogen is cleaved into fibrin by thisThrombin
Intravascular mass formed from blood components adherent to the vessel wallThrombosis
Endothelial injury, stasis or turbulence of blood flow and blood hypercoagulability are factors that can result in thisThrombosis
Exposure of vWF and Tissue factor in a blood vessel is due toEndothelial Injury
(Insert 20)
Question Answer
An autosomal recessive mutation leading to amino acid substitution in the gene coding for this clotting faction. The mutant form becomes resistant to inactivation by protein CFactor V (Leiden) Mutation
Persons with this condition have an increase tendency for thrombosis. Also seen is a higher incidence of thrombosis during pregnancy resulting in a loss of the pregnancy early onFactor V (Leiden) Mutation
Condition that is autoantibody mediated, acquired. It is characterized by venous/artery thrombosis, recurrent fetal loss, cardiac valve vegetations, thrombocytopenia, presence of anti-phospholipid antibodies. There is a higher in African-Americans and Hispanics. Can be seen in association with SLEAntiphospholipid Antibody Syndrome (Hughes Syndrome)
Antibodies are directed against phospholipids bound to plasma proteins (Anti-cardiolipin antibody, Lupus anticoagulant, Anti- β2 glycoprotein antibody). These antibodies inactivate anticoagulantsAntiphospholipid antibody syndrome (Hughes syndrome)
The clinical features of this condition are deep vein thrombosis (DVT), pulmonary embolism, cerebral ischemia (CVA), migraines (cerebral artery thrombosis), renal. For the heart, Libman-Sacks endocarditis and coronary heart disease. There is a history of pregnancy loss around 10th week (very typical)Antiphospholipid Antibody Syndrome (Hughes Syndrome)
Diagnosis for this condition is assay for anti-phospholipid antibodies (aCL test)Antiphospholipid antibody syndrome (Hughes syndrome)
Antiphospholipid antibody syndrome (Hughes syndrome) is diagnosed by this testAnti-Phospholipid Antibodies (aCL test)
Thrombi that occur within cardiac chambers or aortic lumen, adhere to the wallArterial Thrombi
Usually seen in the veins of lower extremities, peri-prostatic plexus, ovarian, peri-uterine. (sometimes involve dural sinus, portal vein, hepatic vein). It forms cast of the vein lumenPhlenothrombosis
This is characterized by chicken fat and currant jelly pattern on gross morphologyPost Mortem Clot
These are produced by alternating ‘pale’ areas of platelets admixed with some fibrin and ‘darker’ layer containing more red cellsLines of Zahn
A detached intravascular solid, liquid, or gaseous mass that is carried by the blood to a site distant from its point of originEmbolism
The serious consequences of this is respiratory compromise due to the non-perfused, although ventilated, segment, and hemodynamic compromise due to increased resistance to pulmonary blood flow caused by the embolic obstructionPulmonary Embolism
Pulmonary thromboembolism that sits astride the pulmonary bifurcationSaddle Embolus
Emboli pass into the (L) side of heart through an inter-ventricular defectParadoxical Embolus
When emboli obstructs more than 60% or more of the pulmonary circulationSudden Death (Cor Pulmonale)
On x-ray, pulmonary embolism will show a shadow in the lower right field and is called thisHamptom’s Hump
This is characterized by pulmonary insufficiency, neurologic symptoms, anemia (hemolysis) and thrombocytopeniaFat Embolism Syndrome
This is associated with fractures of long bones, rupture of bone marrow vascular sinusoids, rarely seen with, soft tissue trauma, liposuction and burnsFat Embolism
Fat embolism can be diagnosed by staining with this stainOil Red O
This can cause a mechanical obstruction of pulmonary microvasculature or fatty acids released from fat globules are toxic to endothelium platelet activation, leukocyte recruitment, free radicalsFat Embolism
Nitrogen comes out of solution in the tissues & blood there is formation of gas bubbles in skeletal muscles and within joints. And, pulmonary vasculatureAir Embolism
Ischemic necrosis in muscles, and bones (femoral head, tibia) leading to avascular necrosisCaisson Disease
Rare condition in which amniotic fluid, fetal cells, hair or other debris enter into maternal circulation leading to cardiorespiratory collapseAmniotic Fluid Embolism
Protein found in the GI and respiratory tract of a fetusMucin
Very fine hair present at birthLanugo
Fatty like substance that is found on the skin at birthVernix
Forms laminated squames in the pulmonary arteriesFetal Squamous Cells
Mucin from amniotic fluid may act as “procoagulants” and play a role in triggering thisDisseminated Intravascular Coagulation (DIC)
An area of ischemic necrosis caused by occlusion of either the arterial supply or the venous drainage in a particular tissueInfarction
Usually occurs in neonates or adolescent males. Blood supply to the testicle is compromised due to twisting of the spermatic cord within the tunica vaginalis resulting in ischemia to the epididymis and the testisTesticular Torsion
Most commonly seen in neonates or adolescent females, the ovary and fallopian tube rotate as a single entity around the broad ligament due to a cyst. Blood supply to the ovary and fallopian tube is compromised due to the twisting resulting in ischemia to the ovary and fallopian tubeOvarian Torsion
Occurs in loose tissues with collaterals - liver, lungs, intestines. May be seen following reperfusionRed Infarction
Occurs in solid organs - heart, kidney spleen. Usually forms a wedge shape, pale structureWhite Infarction
Usually seen in IV drug users and infective endocarditis. Bacterial vegetation from heart dislodge to embolize leads to metastatic infection, following embolizationSeptic Infarction
Many small infections that can be seen over the whole bodyMultiple Microabscess

Hemodynamic Disorders Part 3

Question Answer
Refers to sepsis accompanied by hypotension that cannot be corrected by the infusion of fluidsSeptic Shock
This can be seen in elderly patients with chronic diseases, association with AIDS, widespread use of immunosuppressive drugs, indwelling catheters, and mechanical devicesSeptic Shock
Microbial cell wall constituents (LPS)engage receptors on cells of the innate immune system this triggers pro-inflammatory response (TLRs, PAMPs) which leads toCytokine Upregulation
Activation of the innate immunity leads to release of TNF, IL-1, IFN- γ, IL-12. These mediators upregulate adhesion molecule expression causing thisAmplification
Characterized by acute renal failure. Since necrosis is not invariable, the preferred term would be acute tubular injury. Focal tubular epithelial necrosis (tombstone), occlusion of tubular lumen by casts(shed of cellsAcute Tubular Necrosis
Injury to pneumocytes and pulmonary endothelium. Endothelial activation: circulating inflammatory mediators, activation of adhesion molecules, procoagulant activity, adhesion and extravasation of neutrophils. Accumulation of intralveolar fluid: leaky pulmonary capillaries, damage and necrosis of Type II alveolar pneumocytes, formation of ‘hyaline membrane’Acute Respiratory Distress Syndrome
This condition mostly seen in pediatrics is seen with bacterial infection (Neisseria meningitidis), leading to sepsis. Rapidly progressive hypotension, disseminated intravascular coagulation (DIC). Rapidly developing adrenocortical insufficiency associated with massive bilateral adrenal hemorrhageWaterhouse-Friderichsen Syndrome