General Pathology Blood Vessels Part II PDF

FIRST SEMESTER: GENERAL PATHOLOGY PREFINALS BLOOD VESSELS PART II #03 Floranne Margaret L. Vergara, MD, FPSP...

FIRST SEMESTER: GENERAL PATHOLOGY PREFINALS BLOOD VESSELS PART II #03 Floranne Margaret L. Vergara, MD, FPSP NOV - 09 - 2023 OUTLINE Aneurysms A. Abdominal Aortic Aneurysm I B. Thoracic Aortic Aneurysm C. Aortic Dissection Vasculitis II A. Noninfectious Vasculitis B. Infectious Vasculitis Disorders of Blood Vessel Hyperreactivity III A. Raynaud Phenomenon B. Myocardial Vessel Vasospasm Veins and Lymphatics A. Varicose Veins B. Thrombophlebitis and Phlebothrombosis IV C. Superior and Inferior Vena Caval Syndromes D. Lymphangitis and Lymphedema Vascular Tumors A. Benign Tumors and Tumor-Like V Conditions B. Intermediate-Grade (Borderline) Tumors C. Malignant Tumors Pathology of Vascular Intervention VI A. Endovascular Stenting B. Vascular Replacement VII APA References VIII Review Questions IX Freedom Wall Figure 2. False Aneurysm I. ANEURYSMS Localized abnormal dilation of a blood vessel or the Dissections - Blood enters a defect in the vessel wall and heart tunnels through the medial or medial-adventitial planes Congenital or Acquired True aneurysm - involves all layers of the vessel or wall of the heart → Ex. Atherosclerostic and congenital vascular and ventricular aneurysms following MI Figure 3. Dissection of a Blood Vessel Figure 1. True aneurysms All three forms may lead to rupture leading to catastrophic False aneurysm (aka pseudoaneurysms) - defect in the consequences vascular wall -> extravascular hematoma that Aneurysms may be classified according to size and communicates w/ the intravascular space macroscopic shape: → Saccular - Spherical outpouchings (only a portion of the vessel wall) ▪ 2-20 mm in intracranial vessels (TWG) | (TEG) | (TC) Costales, D. NMD2026  Lesson 3: BLOOD VESSELS PART II NMD2026 ▪ 5-10 cm in diameter in aorta and often contain thrombus → Fusiform - diffuse, circumferential dilation of a long vascular segment ▪ In the aorta, still, generally 5-10 cm ▪ Not specific for any disease or clinical manifestations Pathogenesis Normally -> Arterial walls maintain their integrity by synthesizing, degrading, repairing damage to their ECM constituents In aneurysms -> problems in structure or function of the connective tissue of the vessel wall Various defects in synthesis and breakdown lead to its pathogenesis: → Intrinsic quality of the vascular wall connective tissue is poor ▪ Defective type III collagen synthesis - hallmark of the vascular form of Ehlers-Danlos syndrome → Abnormal TGF-B signaling ▪ Excessive TGF-B activity alters vascular wall remodeling ▪ Mostly in ascending aorta ▪ Leads to diminished ECM content (hence integrity of the vessel wall) ▪ Marfan syndrome - defective synthesis of fibrillin leads to the inability to sequestered produced TGF-B ▪ Loyes-Dietz syndrome - Increased TGF-B signaling results to mutations in: − TGF-B receptors − SMAD3 Figure 4. Cystic Medial Degeneration − TGF-B3 itself − Aneurysms found here are prone to rupture even Based on figure 4(a), there are areas (marked by the in small sizes hence follow an ‘aggressive’ course asterisks), that are devoid of elastin (stained black) → Balance of collagen degradation and synthesis is Atherosclerosis and hypertension - most important altered by inflammation and associated proteases causes of aortic aneurysms ▪ Inflammation or atherosclerosis of aorta -> → Atherosclerosis - most common in AAA and common inflammatory cells (mainly macrophages) produce iliac arteries MMP w/c contribute to breakdown of ECM → Hypertension - most common in ascending AA constituents Other factors include ▪ Also loss of TIMP (tissue inhibitors of → Advanced age; metalloproteinases), for obvious reasons → Smoking ▪ This may involve Th2 production of cytokines - IL-4 → Trauma;; and IL-10 that drives the macrophages to produce → Vasculitis; increased amounts of MMP → Congenital defects; → Vascular wall is weakened through loss of SMCs or → Dysplasia and berry aneurysms (typically in the circle of the inappropriate synthesis of noncollagenous or Willis); and non elastic ECM → Infections (aka mycotic aneurysms) ▪ Systemic hypertension -> significant narrowing of ▪ Originate from: (1)embolization of a septic embolus, arterioles of the vasa vasorum -> outer medial (2) extension of an adjacent suppurative process; or ischemia (3) circulating organisms directly infecting the arterial ▪ Tertiary syphilis -> obliterative endarteritis in the vasa wall vasorum of the thoracic aorta -> medial ischemia -> SMC loss, elastic fiber loss, and inadequate or A. ABDOMINAL AORTIC ANEURYSM inappropriate ECM synthesis All these mechanisms lead to changes in the arterial wall More frequent in men and in smokers primarily medial degeneration Rarely occur before age 50 Atherosclerosis is the main contributing factor Morphology Usually between renal arteries and bifurcation of the aorta Can be saccular or fusiform >3cm but often >5.5cm and up to 25 cm in length W/ severe complicated atherosclerosis and thinning of the aortic media Three variants → Inflammatory AAAs ▪ 5-10% ▪ In younger patients who have back pain and elevated inflammatory markers (CRP, ESR, etc.) ▪ Abundant lymphoplasmacytic inflammation w/ many macrophages associated w/ dense periaortic scarring 2 of 12  Lesson 3: BLOOD VESSELS PART II NMD2026 ▪ Localized immune response to the abdominal aortic wall → Vascular manifestation of IgG4-RD (related disease) ▪ Storiform (like woven fabric) fibrosis and IgG4-positive infiltrating plasma cells in affected tissues ▪ Responds well to steroid and anti-B cell therapies → Mycotic AAAs ▪ Lesions that have become infected by lodging of circulating microorganisms into the vascular wall ▪ Suppuration leads to further medial damage w/c leads to rapid dilation and rupture Clinical Features Most cases are asymptomatic → Incidentally found as a pulsating abdominal mass that Figure 5. Aortic Dissection simulates a tumor Other manifestations: Note the intramural hematoma (asterisk) w/c → Rupture : into peritoneal or retroperitoneal spaces demonstrates an intimal tear in the vessel wall leading to fatal hemorrhage → Obstruction : of a downstream blood vessel resulting to Clinical Features tissue ischemia Morbidity and mortality depend on the part of the aorta → Embolism : from atheroma or mural thrombus involved → Impingement on an adjacent object (ex. compression of Dissections between aortic valve and distal arch - has the a ureter or erosion of vertebrae) most serious complications Risk of rupture is directly related to the size Dissections are classified as: → No chance if 6m and the descending aorta Most aneurysms expand at a rate of 0.2-0.3 cm/year ▪ Type I and II Debakey classification If >=5cm, it should be managed aggressively → Type B dissections → Open surgeries w/ prosthetic grafts or endovascular ▪ More distal lesions approaches through the femoral artery deploying a ▪ Does not involve the ascending aorta stent graft ▪ Usually beginning distal to the subclavian artery ▪ Debakey type III B. THORACIC AORTIC ANEURYSM Recall: most commonly caused by hypertension Symptoms include: → Chest pain - encroachment or erosion into bone → Myocardial ischemia - compression of a coronary artery → Difficulty swallowing - compression of the esophagus → Hoarseness - irritation or pressure on the recurrent laryngeal nerves → Respiratory complications - compression of the bronchi C. AORTIC DISSECTION When blood separates the laminar planes of the media to form blood-filled channel w/in the aortic wall Two age groups: → 40-60 y.o men w/ antecedent hypertension (90%) → Younger patients w/ syndromic diseases affecting the aorta (ex. Marfan syndrome) Can be iatrogenic Pathogenesis Hypertension - major risk factor (remember: same w/ Thoracic Aortic Aneurysm) Aortas have decreased SMC and ECM content - medial degenerative changes (in hypertensive patients) Figure 6. Classification of Dissections Also seen in defective TGF-B signaling Aggressive pressure-reducing therapy may be effective in Classical symptoms: limiting the evolving dissection → Sudden onset excruciating pain, usually in the anterior chest radiating to the back between the scapulae, Morphology moving downwards as dissection progresses Elastic fiber fragmentation and loss Rupture of the dissection: most common cause of death Mucoid ECM accumulation → W/ bleeding into pericardial, pleural, or peritoneal SMC attrition cavities Inflammation is characteristically absent (recall: it may be present in aneurysms as it may be the cause of such) Dissections usually starts w/ an intimal tear Most spontaneous dissections occur in the ascending aorta 3 of 12  Lesson 3: BLOOD VESSELS PART II NMD2026 II. VASCULITIS → ANCAs react w/ these cytokine-activated cells causing either direct injury of further activation General term for vessel wall inflammation w/ varying → ANCA activated neutrophils cause damage by manifestations depending on the vascular bed affected releasing granule contains and ROS Clinical manifestations often include fever, myalgias, arthralgias, and malaise GIANT CELL (TEMPORAL) ARTERITIS All vessels of any organ may be affected Small vessels - most commonly affected Chronic granulomatous inflammation of large to Diseases tend to affect only a particular set of vessels small-sized arteries that principally affects arteries in the (mostly according to size or location) head Check appendix for the tabulated info on primary forms of Temporal arteries (ironically) are not particularly vasculitis vulnerable; The disease is called as such because this Two pathogenic mechanisms: artery is the most readily biopsied in making the diagnosis → 1. Immune-mediated inflammation Ophthalmic artery may be involved -> leads to permanent → 2. Direct invasion of vascular walls by infectious blindness pathogens ▪ Infections may also cause non-infectious vasculitis Pathogenesis -> Generation of immune complexes or triggering Likely due to a T-cell mediated attack on an unknown vascular cross-reactivity (molecular mimicry) vessel wall antigen → Important to distinguish between the two because Presence of granulomatous inflammation, association w/ immunosuppression (for immune-mediated) may be certain MHC class II haplotypes, and excellent response detrimental for those w/ infectious etiologies to steroids supports an immune etiology → Physical and chemical injury can also cause vasculitis Morphology Intimal thickening of arterial segments A. NONINFECTIOUS VASCULITIS → Reduces luminal diameter Classic lesions show medial granulomatous IMMUNE COMPLEX DEPOSITION inflammation centered on the internal elastic membrane w/ elastic lamina fragmentation In immunologic disorders (i.e. SLE) -> production of autoantibodies Identification of antibody involved is usually impossible (extremely low sensitivity and specificity) Immune complex deposition is commonly seen in: → Drug hypersensitivity vasculitis ▪ Drugs may be haptens or immunogens ▪ Leads to formation of immune complex formation ▪ Skin lesions - most common manifestation → Vasculitis secondary to infections ▪ Infections may lead to formation of immune complexes -> these circulate and deposit in vascular lesions ▪ HbsAg and anti-HBsAg deposition -> seen in patients w/ polyarteritis nodosa ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES (ANCA) ANCAs are antibodies that react to neutrophil cytoplasmic antigens Two important ANCAs Table 1. Classification of ANCAs Figure 7. Giant cell arteritis Anti-Proteinase-3 Anti-myeloperoxidase AKA c-ANCA AKA p-ANCA TAKAYASU ARTERITIS Proteinase 3 is a component Myeloperoxidase is a Granulomatous vasculitis of medium and larger arteries of neutrophil azurophilic lysosomal granule Ocular disturbances granule; constituent Marked weakening of pulses in the upper extremities Shares homology w/ Induced by several (hence the name pulseless disease) different microbial peptides therapeutic agents Transmural fibrous thickening of the aorta -> responsible for particularly propylthiouracil Severe luminal narrowing of major branch vessels generation of this ANCA If >50 y.o. - Giant cell aortitis Commonly seen in Commonly seen in If elicits the release of cytokines (like TNF) -> upregulate leads to weakness of peripheral pulses (remember: surface expression of PR3 and MPO on cells pulseless disease!) Histologically: 4 of 12  Lesson 3: BLOOD VESSELS PART II NMD2026 → Mononuclear infiltrates in adventitia → Perivascular cuffing of the vasa vasorum KAWASAKI DISEASE → Intense mononuclear inflammation in the media → Indistinguishable from giant cell arteritis Acute, febrile, and self-limiting disease of infancy and childhood Affects large to medium-sized vessels 80% are children Systemic vasculitis of small to medium sized muscular suggests that a single episode of antibody or immune arteries complex deposition → Typically the renal and visceral vessels → Pulmonary circulation is spared Pathogenesis No ANCAs Drugs, microorganisms, heterologous proteins, or tumor Associated w/ chronic hepatitis B proteins may lead to immune response -> immune → HBsAg-HbsAb complexes deposit in affected vessels complex deposition or trigger secondary immune responses (may include ANCAs) that are pathogenic Morphology Most cases due to MPO-ANCA Segmental transmural necrotizing inflammation of small to medium sized arteries GRANULOMATOSIS WITH POLYANGIITIS (GPA) → Often w/ superimposed aneurysms and/or thrombosis Lesions usually only involve a part of the vessel Formerly Wegener granulomatosis circumference Is a necrotizing vasculitis Based on figure 9 below, a portion (shown by the Composed of a triad of arrowhead) is an unaffected, typically seen in polyarteritis → Acute necrotizing granulomas - of either upper, nodosa (remember segmental necrosis only) lower, or both respiratory tract → Necrotizing or granulomatous vasculitis - small to medium sized vessels, most prominent in lungs and upper RT → Focal necrotizing, often crescentic, glomerulonephritis Pathogenesis T cell mediated hypersensitivity against innocuous inhaled microbial or environmental agents PR3-ANCAs present in 95% of cases Morphology Granulomas with geographic patterns of central necrosis and accompanying vasculitis Granuloma formation is similar to mycobacterial and fungal infections Figure 9. Polyarteritis Nodosa 5 of 12  Lesson 3: BLOOD VESSELS PART II NMD2026 III. DISORDERS OF BLOOD VESSEL HYPERREACTIVITY Characterized by inappropriate or excessive blood vessel vasoconstriction A. RAYNAUD PHENOMENON Exaggerated vasoconstriction of arteries and arterioles in responses to cold or emotion Most commonly affects the extremities, particularly the fingers and toes Can either be primary or secondary → Primary Raynaud phenomenon ▪ 3-5% ▪ Most often in young women ▪ Symmetrical ▪ Does not progress in severity and extent of involvement ▪ Intrinsic hyperreactivity of medial SMCs ▪ Ulcerations and ischemic gangrene are rare → Secondary Raynaud phenomenon Figure 10. Granulomatosis with Polyangiitis ▪ Vascular insufficiency due to arterial disease caused by other diseases (SLE, scleroderma, etc.) CHURG-STRAUSS SYNDROME ▪ Asymmetric involvement of extremities AKA allergic granulomatosis and angiitis ▪ Progressively worsens over time Small-vessel necrotizing vasculitis Associated w/: → Asthma → Allergic rhinitis → Lung infiltrates → Peripheral eosinophilia → Extravascular necrotizing granulomas → Striking eosinophilic infiltration of vessels and tissues Likely a consequence of hyperresponsiveness to some normal allergic stimulus MPO-ANCAs in minority of cases To differentiate from other vasculitides : presence of both Figure 11. Raynaud Phenomenon granulomas AND eosinophils B. MYOCARDIAL VESSEL VASOSPASM THROMBOANGIITIS OBLITERANS (BUERGER If cardiac vessels spasm too long (20 to 30 minutes), it DISEASE) can result to MI or sudden cardiac death Segmental, thrombosing, acute and chronic inflammation → May be caused by endogenous and exogenous of medium and small-sized arteries vasoactive mediators Especially the tibial and radial arteries -> often leads to → Elevated thyroid hormone can also cause a similar vascular insufficiency of the extremities effect -> increases blood vessel sensitivity for catecholamines Pathogenesis → Autoantibodies and T cells and scleroderma can cause Strong relationship between cigarette and disease excessive vasospasm occurrence → Extreme psychological stress -> release of → Either by direct damage to epithelium; or catecholamines → Immune response to some tobacco component that have modified host vascular wall proteins IV. VEINS AND LYMPHATICS Morphology Varicose veins and phlebothrombosis/thrombophlebitis -> 90% of clinical venous disease Affected vessels show acute and chronic inflammation with luminal thrombosis A. VARICOSE VEINS Thrombus may contain microabscesses composed of neutrophils with granulomatous inflammation Abnormally dilated, tortuous veins Inflammation extends to other contiguous veins and Produced by prolonged increased intraluminal pressure w/ nerves -> rare in other forms of vasculitis vessel dilation and incompetence of venous valves Superficial veins of upper and lower leg - commonly B. INFECTIOUS VASCULITIS involved because of venous pressures that are higher on those sites produced by prolonged dependent posture Direct invasion of infectious agents → Usually bacteria or fungi -> particularly Aspergillus Clinical Features and Mucor Incompetent venous valves -> stasis, congestion, edema, Vascular infection weakens arterial walls and culminate in pain, and thrombosis mycotic aneurysms or induce thrombosis Tissue ischemia: due to chronic venous congestion w/ poor vessel drainage -> stasis dermatitis (aka brawny induration) and ulcerations → Brawny color comes from hemolysis of extravasated RBCs 6 of 12  Lesson 3: BLOOD VESSELS PART II NMD2026 Varicosities in two other sites are worth mentioning: Chronic edema can lead to ECM deposition and fibrosis -> → Portal hypertension (mostly by cirrhosis) -> opening of producing brawny induration or peau d’ orange portosystemic shunts to increase blood flow into veins appearance of the overlying skin at the gastroesophageal junction (esophageal varices), rectum (hemorrhoids), and periumbilical veins (caput V. VASCULAR TUMORS medusa) Arise either from endothelium or from cells that support or ▪ Esophageal varices - most important because they surround blood vessels can rupture -> massive upper GI hemorrhage Large vessels - mostly sarcomas → Hemorrhoids -may also result from primary varicose Benign tumors dilation of the venous plexus at the anorectal junction → Usually contain obvious vascular channels filled w/ blood cells B. THROMBOPHLEBITIS AND → Lined by normal looking ECs PHLEBOTHROMBOSIS Malignant tumors Thrombophlebitis/phlebothrombosis - terms used to → More solidly cellular and more proliferative describe venous thrombosis and inflammation → Exhibit cytologic atypia Deep leg veins account for more than 90% of cases → Usually do not form well-organized vessels Decreased blood flow (during prolonged immobilization) - most common cause of lower extremity DVT A. BENIGN TUMORS AND TUMOR-LIKE Common risk factors: CONDITIONS → Extended bed rest or sitting Ectasia - local dilation of a structure → Postoperative state Telangiectasia - permanent dilation of preexisting small → CHF vessels → Pregnancy use → Usually in the skin or mucous membranes → Oral contraceptive use → Not true neoplasms → Obesity → Malignancy Nevus Flammeus ▪ Particularly in adenocarcinomas -> AKA birthmarks hypercoagulability is a form of paraneoplastic Most common vascular ectasia syndrome Light pink to deep purple flat lesion on the head and neck ▪ Usually venous thromboses in these cases appear in composed of dilated vessels one site, disappear, then occur at another sit - Most regress spontaneously migratory thrombophlebitis (aka Trousseau Port wine stain syndrome) → Type of nevus flammeus Homan sign - pain behind the knee upon forced → Grows during childhood dorsiflexion of the foot → Thickening of the associated skin surface → Persists over time C. SUPERIOR AND INFERIOR VENA CAVAL SYNDROMES Superior vena cava - usually by neoplasms that compress or invade SVC → Bronchogenic carcinoma or mediastinal lymphoma Inferior vena cava - neoplasms that compress and invade the IVC and also thrombosis of the hepatic, renal, or lower extremity veins → Hepatocellular carcinoma D. LYMPHANGITIS AND LYMPHEDEMA Primary disorders of lymphatics - less common Secondary to inflammation or malignancies - more frequent Lymphangitis Acute inflammation of the lymphatics caused by bacterial infection Group A streptococcus - most common agent Red, painful subcutaneous streaks w/ painful enlargement of the draining lymph nodes (lymphadenitis) Figure 12. Mikhael Gorbachev is known, among others, for his noticeable Port wine stain in the head Lymphedema Collection of lymph fluid in a body part Sturge-Weber syndrome Primary lymphedema → If lesions is distributed according to the trigeminal nerve → Congenital (ex. simple congenital lymphedema) innervation → Familial Milroy disease (heredofamilial congenital → AKA encephalotrgeminal angiomatosis lymphedema → Facial port wine nevi ▪ Results in lymphatic agenesis or hypoplasia → Ipsilateral venous angiomas in the cortical Secondary lymphedema are caused by: leptomeninges → Tumors - block lymphatic channels → Intellectual disability → Seizures → Surgical procedures - sever lymphatic connections → Postradiation fibrosis → Hemiplegia → Filariasis → Skull radiopacities → Postinflammatory thrombosis and scarring 7 of 12  Lesson 3: BLOOD VESSELS PART II NMD2026 Can also occur in the liver, spleen, and kidneys Histologically - thin-walled capillaries with scant stroma Figure 14. Capillary Hemangioma Figure 13. Sturge-Weber Syndrome Juvenile hemangiomas AKA strawberry-type hemangiomas Majority of port wine stains - somatic single nucleotide Extremely common and can be multiple missense mutation in GNAQ Completely regress by age 7 in most cases Spider Telangiectasias Nonneoplastic vascular malformations grossly resembling a spider Dilated subcutaneous arteries or arterioles Blanch w/ pressure Common in face, neck, or upper chest Associated w/ hyperestrogenic states (pregnancy women or patients w/ cirrhosis) Figure 15. Juvenile Hemangiomas Cavernous Hemangiomas Large, dilated vascular channels Vs. capillary hemangiomas : more infiltrative, frequently involved deep structures, and do not spontaneously Figure 14. Spider Telangiectasias regress Unencapsulated Hereditary Hemorrhagic telangiectasias Composed of large, cavernous blood-filled vascular AKA Osler-Weber-Rendu disease spaces separated by connective tissue stroma Autosomal dominant Can be seen in von Hippel-Lindau disease Mutations in genes encoding for components of the TGF-B signaling pathway of ECs Lesions can spontaneously rupture → Serious epistaxis → GI bleeding → Hematuria HEMANGIOMAS Tumors composed of blood-filled vessels Most are present from birth and initially increase in size but regress spontaneously Angiomatosis - more extensive and diffuse form of hemangiomas ⅓ of internal lesions occur in the liver Malignant transformation is rare Figure 16. Cavernous Hemangioma Capillary Hemangiomas Most common type Occur in the skin, subcutaneous tissues, mucous membranes of the oral cavity and lips 8 of 12  Lesson 3: BLOOD VESSELS PART II NMD2026 Pyogenic granulomas Bacillary Angiomatosis Capillary hemangiomas that present as rapidly growing red pedunculated lesions in the skin, gingiva, or oral Vascular proliferation in immunocompromised hosts mucosa Caused by Bartonella bacteria Bleed easily and often ulcerate → Bartonella henselae Cured by curettage and cautery ▪ Principal reservoir domestic cats Granuloma gravidum - pregnancy tumor that occurs in the ▪ Cat-scratch disease gingiva of pregnant women → Bartonella quintana ▪ Human body lice ▪ Trench fever during World War I B. INTERMEDIATE-GRADE (BORDERLINE) TUMORS Kaposi sarcoma (KS) - vascular neoplasm caused by HHV8 Most common in patients w/ AIDS Figure 17. Pyogenic Granuloma of the Lip LYMPHANGIOMAS Benign lymphatic counterpart of hemangiomas Simple (capillary) lymphangiomas Histologically : exhibit networks of endothelium-lined spaces Distinguished from capillary channels using lymphatic Figure 19. Kaposi Sarcoma endothelial markers OR by the absence of erythrocytes → VEGFR-3 Classic KS → LYVE-1 Associated w/ malignancy or altered immunity NOT associated w/ HIV infection Cavernous lymphangiomas (cystic hygromas) Multiple red-purple skin plaques or nodules, usually in the Typically on the neck or axilla of children distal lower extremities Rarely in the retroperitoneum Tumors are asymptomatic and remain localized Massively dilated lymphatic spaces lined by ECs and separated by connective tissue stroma containing Transplant-associated KS lymphoid aggregates Those having solid-organ transplant w/ T-cell Cavernous lymphangiomas in neck - common in Turner immunosuppression syndrome AIDS-associated (epidemic) KS Glomus Tumor (Glomangioma) Most common HIV-related malignancy Exquisitely painful tumors Often involves lymph nodes and disseminates widely to Arise from modified SMCs of the glomus bodies viscera → Arteriovenous structures involved in thermoregulation Commonly found in distal portion of digits (under the Morphology of KS fingernails) Patches -> Raised Plaques -> Nodules Excision is curative Patches → Red-purple macules → Histologically : dilated irregular EC-lined vascular spaces w/ interspersed lymphocytes, plasma cells and macrophages Raised Plaques → Dermal accumulations of dilated, jagged vascular channels lined and surrounded by plump spindle cells Nodules → Sheets of plump, proliferating spindle cells, mostly in the dermis or subcutaneous tissues → This stage often heralds lymph node and visceral involvement C. MALIGNANT TUMORS (ANGIOSARCOMA) Angiosarcoma - malignant endothelial neoplasms Figure 18. Glomus Tumor Range from highly differentiated tumors to anaplastic lesions More common in older adults Most often involve skin, soft tissue, breast, and liver Hepatic angiosarcomas 9 of 12  Lesson 3: BLOOD VESSELS PART II NMD2026 → Exposure to arsenic, Thorotrast, polyvinyl chloride A. Both statements are true B. Both statements are false VI. PATHOLOGY OF VASCULAR C. Only A is true INTERVENTION D. Only B is true Pathologic changes seen during therapeutic intervention What is the most common manifestation of drug involving the vasculature hypersensitivity vasculitis Interventions that injure endothelium -> induce intimal A. Optic neuritis 3 thickening B. Jaundice C. Acute Kidney Injury A. ENDOVASCULAR STENTING D. Skin lesions This drug is known to induce the formation of Stenosed arteries can be dilated by inflating a balloon Anti-myeloperoxidase catheter to pressures sufficient to rupture the occluding A. Probenecid plaque (balloon angioplasty) 4 B. Propylthiouracil If angioplasty alone -> abrupt reclosure frequently occurs C. Propanolol due to luminal compression caused by D. Tegathiouracil angioplasty-induced vascular dissection, by vessel wall spasm, or by thrombosis Giant Cell arteritis may lead to permanent blindness Therefore, angioplasty and concurrent coronary stent 5 A. True placement is necessary B. Flase → Stents are expandable tubes of metallic mesh Characteristically has granulomas and eosinophils Even after stent placement, thrombosis is still a risk A. GPA → Patients must receive antithrombotic agents after the 6 B. Kawasaki Disease procedure C. PAN Drug eluting stents D. Churg-Strauss syndrome → Prevents restenosis by releasing antiproliferative drugs The following are true about varicose veins, except: into the adjacent vessel wall -> blocks SCM activation A. Higher incidence in women B. Hemorrhoids are the most important complication 7 C. Obesity increases risk of developing varicose veins D. A family predilection most likely results from a familial hypertensive condition Most common causative agent for Lymphangitis A. Group A streptococcus B. Listeria monocytogenes 8 C. Staphylococcus aureus D. Wuchereria bancrofti E. Group B streptococcus Single nucleotide missense of which gene results in port wine stains A. HLAP 9 B. GQNAC Figure 20. Endovascular Stenting C. GNAQ D. PLAQ B. Vascular Replacement Malignant transformation of hemangiomas to cancers is common Vascular grafts may replace damaged vessels 10 A. True Some problems encountered are: B. False → Thrombosis Typical location of berry aneurysm → Intimal thickening A. Both → Vein graft atherosclerosis B. Abdominal Aorta 11 C. Neither D. Thoracic aorta VII. APA REFERENCES [LECTURER] (2023). TITLE [Powerpoint Slides]. College of Medicine, Davao Hemangioma in infancy Medical School Foundation, Inc. A. Malignant tumor involving blood vessels Kumar, V., Abbas, A. K., & Aster, J. C. (2017). Robbins Basic Pathology (10th 12 B. Regress spontaneously in many cases ed.). Elsevier - Health Sciences Division C. Surgery is first line of treatment D. Rare tumor of infancy VIII. REVIEW QUESTIONS DeBakey type B dissection involves A. Pulmonary artery # QUESTION 13 B. Vessel Distal to the subclavian artery The following has hypertension as the main risk factor C. Ascending and Descending Aorta for disease development, except: D. Ascending Aorta A. Thoracic aortic aneurysm NOT a common complications of 1 varicose veins B. Abdominal aortic aneurysm C. Aortic dissection A. Stasis dermatitis 14 D. None of the above B. Ulcerations Dissection and Aneurysms result in loss of ECM C. Poor wound healing 2 constituents. Inflammation is commonly seen in aortic D. Thrombosis dissections Most frequently associated with 15 hyperestrogenism 10 of 12  Lesson 3: BLOOD VESSELS PART II NMD2026 A. Osler-weber-rendu disease B. Spider telangiectasia C. Port-wine stain D. Sturge-weber syndrome Which of the ff is a large vessel vasculitis A. Kawasaki disease 16 B. Takayasu arteritis C. Wegener granulomatosis D. Polyarteritis nodosa Most common variant of hemangioma A. Cavernous hemangioma 17 B. Juvenile hemangioma C. Capillary hemangioma D. Granuloma gravidarum # QUESTION B. Recall that atherosclerosis is the main risk factor for 1 AAA 2 C. Inflammation is rarely seen in aortic dissections 3 D 4 B 5 True. If the ophthalmic artery is involved 6 D. B. Esophageal varices are the most important 7 complication because they may lead to upper GI bleeding 8 A. 9 C 10 B 11 C. Berry aneurysms occur in cerebral vessels 12 B 13 B 14 D 15 B 16 B 17 C IX. FREEDOM WALL 11 of 12 INDEX: APPENDIX 12 of 12