Vascular System

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Spider telangiectasia: Dilated small vessel surrounded by radiating fine channels
Associated with hyperestrinism (seen in liver dz and pregnancy)
Another name for hereditary hemorrhagic telangiectasia: Osler-Weber-Rendu syndrome
What is hereditary hemorrhagic telangiectasia?: Autosomal dominant condition
localized dilation and convolution of venules and capillaries of skin and mucous membranes
Complicated by epistaxis adn GI bleeding
What is hemangioma?: Malformation of larger BV filled with blood
Most common tumor of infancy
--> Port wine stain birthmarks
Capillary hemangioma Location: tangle of closely packed capillary channels seen in skin, subQ tissue, lips, liver, spleen, and kidney
Cavernous hemangioma Location Who Component of what other dz Histology: Large cavernous vascular spaces in the spleen, liver, pancreas, and brain
Can occur in von Hippel Lindau dz
Often seen inchildren
Microscopically: Huge dilated spaces lined with stroma
Mode of transmission of von Hippel-Lindau disease What else is this dz associated with? Features of this dz: Autosomal dominant
Renal cell carcinoma
Component of cavernous hemangioma
Hemangioblastoma of cerebellum , brain stem and retina
Adenomas and cysts are also common in liver, kidney, and pancreas
Glomangioma: Small purplish painful subungual nodule in finger or to e
Cystic hygroma: cavernnous lymphangioma that occurs in neck or axilla
Lymphangioma Location Gross appearance Microscopic appearance: In head, neck, axilllary subQ tissue
Grossly: Cutaneous nodules/pedunculated lesions/well demarcated compressible pink grey masses
Microscopically: Networks of EC lined spaces. Contain no RBCs
Hemangioendothelioma Behavior and histology: Behavior is between malignant and benign
Ascular channels with masses and sheets of pleiomorphic spindle shaped large plump cells of endothelial origin
Hemangiopericytoma Behavior and location: Behavior is between malignant and benign
Arise in lower extremities adn retroperitoneum
Hemangiosarcoma Behavior, histology, location: Most malignant
Very rare
small, well-demarcated nodules --> large fleshy gray-white soft tissue
Occurs in skin, musculoskeletal system, breast and/or liver
What are risk factors for hemangiosarcoma: Arsenic
Thorium dioxide (thorostat)
PVC (associated with angiosarcoma of the liver)
Can also occur in setting of chronic lymphedema (ex: 10 yrs after radical mastectomy for breast CA. Tumor arises from dilated lymphatic vessels)
Also caused by chronic indwelling of foreign objects
4 types of KS and who gets it: Classic KS- Ashkenazi Jews/Mediterraneans
Endemic (African) KS- young African men and children; 10% of all cancers in Africa; restricted to lymph nodes
Epidemic KS- AIDS, esp homosexual males; caused by KS herpesvirus. Lesions in skin, mucous membranes, LN, GI, viscera
Transplant KS- transplant pts (who are immunosuppressed). Cutaneous and visceral involvement. Stops if immunosuppressants are stopped
Characteristics of polyarteritis nodosa: Necrotizing immune complex inflammation of small and medium sized arteries
Arterial media and internal elastic lamella are destroyed --> aneurysmal nodules
What is associated with polyarteritis nodosa?: HBV
Clincal manifestations of polyarteritis nodosa?: Fever
Weight-loss
Malaise
Abdominal pain
HA
Myalgia
HTN
ARthritis
Ischemic heart dz
Where can polyarteritis nodosa occur: kidneys
coronary arteries
musculoskeleta system
GI tract
CNS, PNS
Eye
Skin
What is elevated in polyarteritis nodosa: P-ANCA
(serum AB to neutrophilic myeloperoxidase)
Most common cause of death from polyarteritis nodosa: renal lesions and HTN
Churg-Strauss syndrome Location Clinical manifestations: Necrotizing vascuitis (considered a variant on polyarteritis nodosa)
Involvement of pulmonary vasculature
Marked peripheral eosinophilia
Clnical manifestations of asthma
What is elevated in Churg-Strauss syndrome: P-ANCA
Microscopic polyangitis: Distinct from PAN by involvement of small BV (PAN is small and medium sized BV)
Lesions all at same histological stage
Acute inciting event --> immune complexes in previously sensitized host
Lesions can be only in skin, or can combine lung, brain, heart, and kidney
Histology of mcroscopic polyangitis: fibrinoid necrosis
Affected BV may show only fragmented neutrophilic nuclei around and within small BV walls
Histology of PAN? Acute lesions Healing lesions Healed lesions: Acute lesions are well-demarcated, induce thrombosis --> distal ischemic injury

Healing lesions show fibroblast proliferation superimposed on ongoing fibrinoid necrosis

Healed lesions show marked fibrotic thickening of arteries with elastic lamina fragmentation and sometimes aneurysmal dilation
Hypersensitivity vasculitis: Immune complex mediated vasculitides with acute inflammation of small BV
Lesions are all the same age
Clinical features of hypersensitivity vasculitis: palpable purpura when skin is involved
can also involve glomeruli or GI tract
Causes of hypersensitivity vasculitis: drugs
food
infectious organisms
complication of systemic illness (CT d/o or malignancy)
Dinstinctive syndromes seen in hyperesensitivity vasculitis: Henoch-Schonlein purpura
Serum sickness
Henoch-Schonlein purpura: Common in young kids
hemorrhagic urticaria of extensor surfaces of arms, legs, buttocks
Fever, arthraliga, GI and renal involvement are all common
CAn sometimes be post-streptococcal
Associated with antecedent upper respiratory infections
Serum sickness: Deposition of Ag-AB complexes in heart, joints, and kidneys
Wegener granulomatosis Etiology Characteristics Affected organs: Unknown
necrotizing granulomatous vasculitis of small and medium sized vessels
Respiratory tract, kidneys
Clinical features of Wegener granulomatosis: Respiratory tract signs and symptoms
Paranasal sinuses and lungs are especially affected
Necrotizing glomerulonephritis
histology of WEgener granulomatosis: Fibrinoid necrosis of small arteries and veins
Granuloma formation with giant cells prominent
Marker for Wegener granulomatosis: C-ANCA
Giant Cell arteritides: Seen in medium to large sized arteries
Characterized by granuloma formation with giant cells and infiltrates of mononuclear cells, neutrophils, eosinophils
2 types of giant cell arteriritis: Temporal arteritis
Takayasu arteritis
Temporal arteritis: most frequently occurring vasculitis
Systemic
Common in elderly
Most commonly affects branches of carotid artery (esp temporal artery)
Clinical manifestations of temporal arteritis: malaise
fatigue
HA
Jaw claudication
Tenderness
Absent pulse
Palpable nodules along course of involved artery
visual impairment
polymyalgia rheumatica
Elevated ESR
Polymyalgia rheumatica: Component of temporal arteritis
proximal muscle pain
Periarticular pain
morning stiffness
Takayasu arteritis: inflammation and stenosis of medium and large sized arteries
Aortic arch and branches are frequently involved
Clinical features of Takayasu arteritis: Absent pulses in carotid, radial, or ulnar arteries
Fever, night sweats, malaise, myalgia, arthritis
Eye problems
Painful skin nodules
Another name for Kawasaki Dz: Mucocutaneous lymph node syndrome
What is Kawasaki Dz?: Acute, self limited ilness of infants and children
Acute necrotizing vasculitis of small and medium sized BV
Clinical features of Kawasaki dz What can it lead to?: Fever
emorrhagic edema of conjunctivae, lips, and oral mucosa
Cervical lymphadenopathy

Coronary artery vasculitis with aneurysm formation
Another name for Buerger dz: Thromboangiitis obliterans
What is Buerger dz? Population Risk factor: Acute inflammation involving small and medium sized arteries of extremities
Extends to adjacent veins and nerves
Jewish population, young men
Cigarette smoking
Clinical features of Buerger dz? Histologic features of Buerger dz?: Painful ischemia --> gangrene
Neutrophilic abscesses
Mural thrombi with giant cell formation
Late lesions show organiziation and recanalization
Lymphomatoid granulomatosis: rare grnulomatous vasculitis
Infiltration by atypical lymphocytoid and plasmacytoid cells
Chronic inflammatory condition --> lymphoproiliferative neoplasm (T-cell non-hodgkin's lymphoma)

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