Category Archives: Vascular

Amyloidosis

Amyloidosis

Different types of Amyloidosis:

•    AL: Multiple myeloma

•    AA: Chronic inflammatory conditions (chronic osteomyelitis, bronchiectasis, TB, rheumatoid arthritis, inflammatory bowel disease, ankylosing spondylitis, chronic skin infections in heroin users, renal cell carcinoma, Hodgkin lymphoma, familial Mediterranean fever)

•    Aβ: Alzheimer’s dementia

•    Aβ2m: Long-term hemodialysis

•    A Cal: Medullary thyroid cancer

•    ATTR: Systemic senile amyloidosis, familial amyloidotic neuropathies

•    AIAPP: Type II diabetes

•    AANF: Isolated atrial amyloidosis

•    PrPSC: Various prion diseases of the CNS

Ultrastructural features of amyloid

•    Non-branching fibrils of varying lengths with a diameter of 7.5 to 10 nm

Histochemical stains to demonstrate amyloid

•    Congo red

•    Crystal violet

These histochemical stains intercalate between the β-pleated sheets of the amyloid fibril, limiting their steric configuration

•    The alignment of the dye molecules allows for the phenomenon of dichroism, such that at on polarization, the amyloid fibrils take on a green colour

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Pulmonary hypertension

Pulmonary hypertension

Causes of Pulmonary Hypertension

•    Chronic obstructive or interstitial lung disease (eg. UIP)

•    Antecedent congenital or acquired heart disease (eg. mitral stenosis)

•    Recurrent thromboemboli

•    Autoimmune disease (eg. systemic sclerosis)

•    Idiopathic (primary) pulmonary hypertension

Microscopic features seen with increasing severity

•    Grade 1 = medial hypertrophy of arteries and muscularisation of arterioles

•    Grade 2 = intimal proliferation in arteries

•    Grade 3 = intimal concentric laminar fibrosis prominent in muscular arteries

•    Grade 4 = dilatation of small arteries with development of plexiform lesions

•    Grade 5 = plexiform and angiomatoid lesions prominent; hemosiderin deposition

•    Grade 6 = necrotising arteritis

Pulmonary hypertension complications

•    Cor pulmonale

•    Thromboembolism

•    Pneumonia

•    Pulmonary artery atherosclerosis

•    Sudden death

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Emboli

Emboli

Emboli definition

Emboli are detached intravascular mass of solid, liquid or gaseous material that is carried by the blood to a site distant from its point of origin

Embolic material

•    Air/nitrogen embolus

•    Fat/bone marrow embolus

•    Thromboembolus

•    Amniotic fluid embolus

•    Foreign bodies (eg. bullet)

•    Tumour embolus

•    Cholesterol (atherosclerotic debris) embolus

Air Emboli

o    Air emboli may enter the circulation during obstetric procedures or after chest wall injury

o    Excess of 100cc needed to cause clinical effect

The air bubbles coalesce and physically obstruct blood flow to right side of heart and pulmonary circulation causing sudden death

o    Decompression sickness caused when individuals are exposed to sudden changes in pressure which can be seen in scuba/deepsea divers, underwater construction workers, individuals in unpressurized aircraft

o    Gas breathed in under pressure bubbles out into vasculature when these individuals undergo rapid ascent

o    Gas bubbles within skeletal muscle and supporting tissues in and around joints causes “the bends”

o    May cause focal ischemia in organs such as the brain, heart, etc.

o    In lungs, edema, hemorrhage, and focal atelectasis or emphysema may occur leading to dyspnea

o    Chronic decompression disease (caisson disease) occurs in workers working in pressurized tunnels under bridges

o    Results in persistent gas emboli of the skeletal system causing ischemic necrosis of femoral, humeral and tibial heads

Fat and bone marrow emboli

o    Occurs after long bone fractures or after soft tissue trauma and burns

o    Occurs in 90% of patients with severe skeletal trauma, however <10% manifest symptoms

o    Characterized by pulmonary insufficiency, neurologic symptoms, anemia, and thrombocytopenia

o    Symptoms begin 1-3 days after injury with sudden onset of tachypnea, dyspnea, and tachycardia

o    Neurologic symptoms include irritability, restlessness, delirium and coma

o    Platelets adhere to fat globules and are removed from circulation causing thrombocytopenia

o    Erythrocytes aggregate and undergo hemolysis

o    Diffuse petechial rash is seen in non-dependent areas in 20-50% of patients

o    Fatal in up to 10% of cases

Thrombus

Pulmonary thromboembolus

-    >95% are due to above knee deep venous thrombosis

-    60-80% are clinically silent because of their small size

-    Complications include:

•    Sudden death

•    Pulmonary hypertension (especially from multiple thromboemboli)

•    Cor pulmonale (right heart failure)

•    Cardiovascular collapse

•    Pulmonary hemorrhage

•    Pulmonary infarct

Systemic thromboembolus

-    80% arise from intracardiac mural thrombi (2/3 associated with left ventricular wall infarcts and ¼ associated with atrial fibrillation secondary to mitral valve disease)

-    Remainder arise from aortic aneurysms, thrombi on ulcerated atherosclerotic plaques, fragmentation of valvular vegetation, or paradoxical emboli

-    Can cause abscesses (if septic), death

Major sites for embolization (and ischemic necrosis) are:

•    Lower extremities (75%)

•    Brain (10%)

•    Intestines

•    Kidney

•    Spleen

•    Upper extremities

Amniotic fluid

o    Uncommon complication of labour and post-partum period

o    Mortality rate of 20-40%

o    Due to infusion of amniotic fluid into maternal circulation via tear in placental membranes or rupture of uterine veins

o    Characterized by severe dyspnea, cyanosis, hypotensive shock, seizures and coma

o    If patient survives initial crisis, pulmonary edema can occur and 50% develop DIC

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Temporal Arteritis

Temporal Arteritis

Clinical features

•    Facial pain or headache (often most intense and along course of superficial temporal artery)

•    Pain to palpation of superficial temporal artery

•    Ocular symptoms (diplopia, transient or complete vision loss)

•    Jaw and or tongue pain on chewing/talking (“jaw claudication”)

•    Fever, fatigue, weight loss

•    Diminished pulses, intermittent claudication of extremities, Raynaud phenomenon, paresthesia, myocardial infarct, angina (large vessel disease)

Microscopic features

•    Segments of affected arteries develop nodular thickenings with reduction of the lumen and may often become thrombosed

•    Granulomatous inflammation of inner half of media, centered on the internal elastic membrane, marked by mononuclear cell infiltrate, multinucleate giant cells of foreign body and Langhans type (2/3 of cases)

•    Fragmentation of internal elastic lamina

•    A Less common pattern consists of non-specific panarteritis with mixed inflammatory infiltrate composed of lymphocytes, macrophages, neutrophils and eosinophils

•    Healed stage of both patterns reveals collagenous thickening of vessel wall; organization of luminal thrombus sometimes transforms artery into fibrous cord

Temporal artery biopsy grossing

•    A 2-3 cm length of temporal artery is ideal; cross-sections of the temporal artery should be taken every few mm; several levels should be taken from each section

Temporal arteritis negative biopsy

•    Negative biopsy doesn’t rule out condition since giant cell arteritis can occur focally and segmentally.  Clinicians will still treat with steroids to prevent ocular symptoms (ie. blindness)

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Hematoma

Hematoma

Hematoma

An image of a hematoma in a placenta. Evident are the Lines of Zahn.

Lines of Zahn

Lines of Zahn.

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Leukocytoclastic Vasculitis

Leukocytoclastic Vasculitis

Histological features

1.    Fibrinoid (eosinophilic) necrosis of vessel wall

2.    Accompanied by fragmented neutrophils, nuclear “dust”

3.    Affects arterioles, capillaries, venules

4.    Also associated with fibrin thrombi in vessel lumina with extravasation of red blood cells around affected vessels

Diseases associated with Leukocytoclastic Vasculitis

1.    Henoch-Schonlein purpura

2.    Drug reactions

3.    Lupus SLE

4.   Rheumatoid arthritis

5.    Cryoglobulinemia

6.    Viral hepatitis

7.    Infection (eg. Streptococci)

8.    Underlying malignancy

Henoch-Schonlein Purpura Immunofluorescence

1.    Kidney biopsy demonstrates mesangial deposition of IgA, sometimes with IgG and C3

2.    IgA deposition is also seen in small dermal vessels involved by necrotizing vasculitis (ie. leukocytoclastic vasculitis)

Useful serological tests

1.    ?ANA, ?RF, ?HBcAb

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PEComa Lesions

PEComa Lesions

1.    Clear cell (sugar) tumour of lung

2.    Lymphangioleiomyomatosis

3.    soft tissues ( PECOMA)

4.    cortical tubers

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Immune Complex Vaculitis

Immune complex vasculitis

Pathogenesis of immune mediated  Immune Complex Vaculitis

1.    Due to antigen-antibody complex formation

2.    Tissue damage elicited by inflammation generated at sites of immune complex deposition

3.    Reaction initiated when antigen combines with antibody within circulation (circulating antigen-antibody complexes) which are deposited in vessel walls

4.    Immune complex deposition cause inflammation via activation of the complement cascade, and activation of neutrophils and macrophages through their Fc receptors

Immunofluorescence in late immune vasculitis

1.    Granular deposits of immunoglobulin and complement

Examples of immune complex mediated vasculiits

1.    SLE – systemic lupus erythematosus

2.    Cryoglobulinemic vasculitis

3.    Serum sickness

4.    Poststreptococcal infection

5.    Henoch schonlein purpura

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Reactive vascular proliferations

Reactive vascular proliferations

i.    Bacillary angiomatosis

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Vascular ectasias

Vascular ectasias

1.    Nevus flammeus

2.    Spider telangiectasia

3.    Hereditary hemorrhagic telangiectasias (Osler-Weber-Rendu syndrome)

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