DISEASES OF BLOOD

VESSELS
DR ANTENEH.B(MD,PATHOLOGIST)
THE BLOOD VESSELS
 Vascular disease is responsible for more morbidity and
mortality than any other category of human disease.

 Although the most clinically significant lesions involve

arteries, venous pathology can also cause clinical
disorders.

 Two principal mechanisms

 1. Narrowing or completely obstructing the lumina
 2. Weakening of the walls, leading to dilation or rupture
NORMAL VESSELS
 Three concentric layers:-
 Intima - has one layer of endothelial cells supported by a
thin subendothelial layer of loose connective tissue
 intima is separated from the media by an internal elastic
lamina

 Media –the middle layer, consists chiefly of concentric

layers of helically arranged smooth muscle cells
 the media has a thinner external elastic lamina, which
separates it from the tunica adventitia
 Adventitia - composed of relatively loose connective
tissue, nerve fibers, and smaller vessels (vessels of the
vessels)
- Based on their size & structural features, arteries are
divided into
 Large or elastic arteries including , the aorta & its large
branches

 Medium-sized or muscular arteries including coronary &

renal arteries

 Small arteries & arterioles

The principal points of physiological resistance to blood
flow
CONGENITAL ANOMALIES
 Three are particularly significant
1.Developmental or berry aneurysms
 Small, spherical dilatations typically in the circle of
Willis

 Fear is rupture

2.Arteriovenous fistulas
 Abnormal, typically small, direct connections between
arteries and veins that bypass the intervening capillaries

 High-output cardiac failure can occur if it is large

3.Fibromuscular dysplasia
 Focal irregular thickening of the walls of medium and
large muscular arteries including renal, carotid,
splanchnicand vertebral vessels

 Cause is unknown but is probably developmental

 Focal wall thickenning is due to combination of irregular

medial and intimal hyperplasia and fibrosis causing
stenosis
HYPERTENSIVE VASCULAR DISEASE
 Elevated blood pressure is called Hypertension

 A sustained diastolic pressure greater than 90 mm Hg, or

a sustained systolic pressure in excess of 140 mm Hg,
constitutes hypertension

 Although hypertension is a common health problem with

occasionally devastating outcomes, it typically remains
asymptomatic until late in its course.
Etiological classification
 95% of hypertension is idiopathic (Essential
hypertension) has an unknown etiology,

 5% have underlying renal or adrenal disease ( secondary

hypertension)
 primary aldosteronism
 Cushing syndrome
 pheochromocytoma
 narrowing of renal artery
Clinical course classification
Benign hypertension (95%)
-a chronic and relatively mild increase in systemic arterial
blood pressure (to a diastolic level not higher than 110 to
120 mm Hg), which may or may not have an underlying
cause

- It is compatible with long life, unless a myocardial

infarction, cerebrovascular accident, or other
complication supervenes
Accelerated or Malignant hypertension
 About 5% of hypertensive persons show a rapidly rising
blood pressure that if untreated leads to death within1or
2 years.

 The clinical syndrome is characterized by

 severe hypertension (diastolic pressure over
120mmHg)
 renal failure
 retinal hemorrhages and exudates, with or without
papilledema.
Mechanisms of Essential Hypertension
 Essential hypertension results from an interplay of
multiple genetic and environmental factors affecting
cardiac output and/or peripheral resistance

 Genetic factors
- Combined effect of mutations or polymorphisms at
several gene loci that influence blood pressure ( such as
genes involved in aldosterone metabolism or sodium
transporters).
 Environmental factors
 modify the expression of any underlying genetic
determinants of hypertension
 Stress
 obesity
 smoking
 physical inactivity and
 heavy consumption of salt
VASCULAR PATHOLOGY IN HYPERTENSION
 Hypertension is associated with two forms of small blood
vessel disease
1.Hyaline Arteriolosclerosis
 Arterioles show homogeneous, pink hyaline thickening
with associated luminal narrowing

2.Hyperplastic Arteriolosclerosis.
 vessels exhibit “onion-skin lesions,” characterized by
concentric, laminated thickening of the walls and luminal
narrowing
ARTERIOSCLEROSIS
 literally means "hardening of the arteries“

 it is a generic term reflecting arterial wall thickening and

loss of elasticity.

 Three patterns are recognized, with different clinical and

pathologic consequences
Arteriolosclerosis
 affects small arteries and arterioles, and may cause
downstream ischemic injury.
 Two type- hyaline and hyperplastic

Mönckeberg medial sclerosis

 is characterized by calcific deposits in muscular arteries
in persons typically older than age 50
Atherosclerosis
 is the most frequent and clinically important pattern
ATHEROSCLEROSIS
 Characterized by intimal lesions called atheromas (also
called atheromatous or atherosclerotic plaques), that
protrude into vascular lumina.

 Atheroma consists of a raised lesion with a soft,

yellow,core of lipid (mainly cholesterol and cholesterol
esters) covered by a firm, white fibrous cap.

20
 EPIDEMIOLOGY
 Common among most developed nations,

 causes far more morbidity and mortality (roughly half of

all deaths) in the Western world than any other disorder

 The prevalence and severity of atherosclerosis among

individuals are related to several risk factors.
RISK FACTORS
PATHOGENESIS
 The contemporary view of atherogenesis is expressed by
the response-to-injury hypothesis.

 This model views atherosclerosis as a chronic

inflammatory response of the arterial wall to endothelial
injury.
 The following are central tenets of the hypothesis:-
 Chronic endothelial injury with resultant endothelial
dysfunction, causing increased permeability, leukocyte
adhesion, and altered gene expression

 Causes include hypertension, hyperlipidemia, toxins

from cigarette smoke, homocysteine, and even infectious
agents
 Accumulation of lipoproteins (mainly LDL and its
oxidized forms) in the vessel wall.
- Lipoprotein abnormalities frequently found in
atherosclerosis are:-
 Increased LDL cholesterol level

 Decreased HDL cholesterol level

 Increased levels of the abnormal lipoprotein

Mechanisms by which hyperlipidemia contributes to
atherogenesis:-
 Hypercholesteromia may directly impair EC function
through increased production of oxygen free radicals
 Oxidized LDL

 Is ingested by macrophages through the scavenger

receptor forming foam cells
 Increases monocyte accumulation in lesions

 Stimulates release of growth factors & cytokines

 Is cytotoxic to ECs & SMCs

 Smooth Muscle Proliferation
 Factor release from activated platelets, macrophages, and
vascular wall cells, inducing SMC recruitment, either
from the media or from circulating precursors

 SMC proliferation and ECM production

31
 Enhanced lipid accumulation both extracellularly and
within cells (macrophages and SMCs)
 Formation of Fibro-fatty Atheroma
 CONSEQUENCES OF ATHEROSCLEROTIC
DISEASE
 Large elastic arteries (e.g., the aorta, carotid, and iliac
arteries) and large and medium-sized muscular arteries
(e.g., coronary and popliteal arteries) are the major
targets of atherosclerosis

 Myocardial infarction (heart attack), cerebral infarction

(stroke), aortic aneurysms, and peripheral vascular
disease (gangrene of the legs)
 Morphology
 Fatty streaks

 are the earliest lesions of atherosclerosis. They are

composed of lipid-filled foam cells.

 Beginning as multiple minute flat yellow spots, they

eventually coalesce into elongated streaks 1 cm or more
in length

 They are not significantly raised & don’t cause any

disturbance in blood flow.
 Atherosclerotic Plaque.
 An atheroma (derived from Greek word for gruel)
consists of a raised lesion with a soft, yellow, grumous
core of lipid (mainly cholesterol and cholesterol esters)
covered by a firm, white fibrous cap.

 superimposed thrombus over ulcerated plaques is red-

brown.

 Plaques vary from 0.3 to 1.5 cm in diameter

Major components of plaque
 Cells (SMC, macrophages and other WBC)

 ECM (collagen, elastin, and PGs)

 Lipid = Cholesterol (Intra/extracellular)

 (Often calcification)
 Atherosclerotic plaques are susceptible to the following
pathologic changes with clinical significance:-
Rupture, ulceration or erosion
 exposes the bloodstream to highly thrombogenic
substances and induces thrombus formation.

 Such thrombi can partially or completely occlude the

lumen and lead to downstream ischemia.
Hemorrhage into a plaque.
 Rupture of the overlying fibrous cap, or of the thin-walled
vessels in the areas of neovascularization, can cause intra-
plaque hemorrhage
 a contained hematoma may expand the plaque or induce
plaque rupture.

Atheroembolism.
 Plaque rupture can discharge atherosclerotic debris into the
bloodstream, producing microemboli.
 Aneurysm formation.
 Atherosclerosis-induced pressure or ischemic atrophy of
the underlying media

 Leads to loss of elastic tissue, causes weakness resulting

in aneurysmal dilation and potential rupture
ANEURYSMS AND DISSECTIONS
 An aneurysm is a localized abnormal dilation of a blood
vessel or the heart.

 When an aneurysm involves all three layers of the

arterial wall or the attenuated wall of the heart, it is
called a "true" aneurysm.

 Atherosclerotic, syphilitic, and congenital aneurysms,

and ventricular aneurysms that follow transmural
myocardial infarctions, are of this type.
 False aneurysm (also called pseudoaneurysm)
 is a breach in the vascular wall leading to an
extravascular hematoma that freely communicates with
the intravascular space ("pulsating hematoma").

 Examples include ventricular ruptures after myocardial

infarctions that are contained by a pericardial adhesion.
- Aneurysms are classified by macroscopic shape and size:-
 Saccular aneurysms are essentially spherical
outpouchings (involving only a portion of the vessel
wall) they vary from 5 to 20 cm in diameter and often
contain thrombi.

 Fusiform aneurysms involve diffuse, circumferential

dilation of a long vascular segment; they vary in
diameter (≤20 cm)
 Pathogenesis of Aneurysms
 The two most important causes aneurysms are
atherosclerosis and cystic medial degeneration of the
arterial media.

 Other causes that weaken vessel walls and lead to

aneurysms include
 trauma,
 congenital defects (e.g., berry aneurysms),
 infections (mycotic aneurysms),
 vasculitis
 AORTIC DISSECTION
 Aortic dissection occurs when blood splays apart the
laminar planes of the media to form a blood-filled
channel within the aortic wall

 this can be catastrophic if the dissection then ruptures

through the adventitia and hemorrhages into adjacent
spaces
- Aortic dissection occurs principally in two
epidemiologic groups
(1) Men aged 40 to 60 years, with antecedent
hypertension (more than 90% of cases of dissection)

(2) Younger patients with systemic or localized

abnormalities of connective tissue affecting the aorta
(e.g., Marfan syndrome)
Morphology
- In most cases, the intimal tear marking the point of
origin of the dissection is found in the ascending aorta,
usually within 10 cm of the aortic valve

- The dissection can extend along the aorta retrograde

toward the heart as well as distally, sometimes all the
way into the iliac and femoral arteries.
- Aortic dissections are classified into two types:-
 The more common (and dangerous) proximal lesions
(called type A dissections)
 involving both the ascending and descending aorta
(DeBakey type I) or the ascending aorta only (DeBakey
type II)

 Distal lesions not involving the ascending part and

usually beginning distal to the subclavian artery (called
type B dissections or DeBakey type III).
 Clinical Features
 The classic clinical symptoms of aortic dissection are the
sudden onset of excruciating pain, usually beginning in the
anterior chest, radiating to the back between the scapulae,
and moving downward as the dissection progresses

 the pain can be confused with that of myocardial infarction.

 The most common cause of death is rupture of the

dissection outward into the pericardial, pleural, or peritoneal
cavities
VASCULITIS
 general term for vessel wall inflammation

 The clinical features of the various vasculitides are

diverse and largely depend on the vascular bed affected

 Besides the findings referable to the specific tissue(s)

involved, the clinical manifestations typically include
constitutional signs and symptoms such as fever,
myalgias, arthralgias, and malaise
- The two most common pathogenic mechanisms of
vasculitis are
 immune-mediated inflammation and

 direct invasion of vascular walls by infectious pathogens.

- Physical and chemical injury, such as from irradiation,

mechanical trauma, and toxins, can also cause vasculitis.
Noninfectious Vasculitis
 The main immunologic mechanisms that initiate
noninfectious vasculitis are:-
(1) Immune complex deposition
Mechanism: Antibodies induced by the disease process
bind antigens. This interaction forms a complex that
deposits within the vessel wall. The immune complex
causes vasculitis through activation of complement.
(2) Antineutrophil cytoplasmic antibodies (ANCAs)
 Many patients with vasculitis have circulating antibodies
that react with neutrophil cytoplasmic antigens, so-called
antineutrophil cytoplasmic antibodies (ANCAs)
 Unknown for certain; however, one possible explanation
is that ANCAs cause degranulation of neutrophils.
 The degranulation of the neutrophils releases substances
that have toxic effects on vessels and surrounding
tissues.
 ANCAs were previously classified according to their
intracellular distribution, either cytoplasmic (c-ANCA)
or perinuclear (p-ANCA).

 c-ANCA are typical of Wegener granulomatosis

 p-ANCA are characteristic of microscopic polyangiitis

and Churg-Strauss syndrome
 (3) Anti-endothelial cell antibodies
 Antibodies to endothelial cells may predispose to certain
vasculitides, for example, Kawasaki disease
 Classification

 On the basis of
 Size and anatomic site of involved vessels
 Histologic characteristic of the lesions
 Clinical manifestations
GIANT-CELL (TEMPORAL) ARTERITIS
 the most common form of vasculitis among elderly
individuals

 It is a chronic, typically granulomatous inflammation of

large to small-sized arteries

 affects principally the arteries in the head especially the

temporal arteries but also the vertebral and ophthalmic
arteries.

 Ophthalmic arterial involvement can lead to permanent

blindness
PATHOGENESIS
 The cause is unknown

 Much evidence points to a T-cell mediated immune

response to an unknown, possibly vessel wall antigen.

 Evidences are
 characteristic granulomatous reaction,
 a correlation with certain HLA class II haplotypes,
 and a therapeutic response to steroids
MORPHOLOGY
 Gross : nodular thickening with reduction of the lumen +
thrombosis

 Histology –granulomatous inflammation in the inner half

of the media that leads to elastic lamina fragmentation
CLINICAL FEATURES
 Most common in older individuals(rare before 50yrs).

 Constitutional symptoms – fever, fatigue, wt loss

 Facial pain or headache – more intense along the course

of the superficial temporal artery.

 Ocular symptoms – diplopia to transient or complete

vision loss.
TAKAYASU ARTERITIS
 This is a granulomatous vasculitis of medium and larger
arteries

 characterized principally by ocular disturbances and

marked weakening of the pulses in the upper extremities
(hence its other name, "pulseless disease").

 transmural fibrous thickening of the aorta-particularly the

aortic arch and great vessels-with severe luminal
narrowing of the major branch vessels
 Females < 40yrs

 Cause and pathogenesis – unknown

 Immune mechanisms suspected.

MORPHOLOGY
 Gross: aortic arch, aortic branches, pulmonary arteries,
coronary, renal arteries.
 Irregular thickening of vessel wall with intimal
wrinkling with narrowing or obliteration of lumen.
 Histological changes range
 from adventitial mononuclear infiltrates with
perivascular cuffing of the vasa vasorum
 to intense mononuclear inflammation in the media
 to granulomatous inflammation, replete with giant
cells and patchy medial necrosis
Clinical Features
- Initial symptoms are usually nonspecific, including
fatigue, weight loss, and fever.
- With progression, vascular symptoms appear and
dominate the clinical picture.
- These include reduced blood pressure and weaker pulses
in the upper extremities relative to the lower extremities,
with coldness or numbness of the fingers;
- ocular disturbances, including visual defects, retinal
hemorrhages, and total blindness; and neurologic deficits
POLYARTERITIS NODOSA
 is a systemic vasculitis of small or medium-sized
muscular arteries (but not arterioles, capillaries, or
venules)

 typically involving renal and visceral vessels but sparing

the pulmonary circulation.

 the cause remains unknown in the majority of cases

 Morphology
 Classic PAN is characterized by segmental transmural
necrotizing inflammation of small to medium-sized
arteries.

 Vessels of the kidneys, heart, liver, and gastrointestinal

tract are involved in descending order of frequency

 During the acute phase there is transmural inflammation

of the arterial wall , frequently accompanied by fibrinoid
necrosis
 Later, the acute inflammatory infiltrate is replaced by
fibrous thickening of the vessel wall that can extend into
the adventitia.

 Characteristically, all stages of activity (from early to

late) coexist in different vessels or even within the same
vessel, suggesting ongoing and recurrent insults.
Clinical Course
 PAN is a disease primarily of young adults, but it can
occur at all ages.

 The course can vary from acute to chronic but is typically

episodic, with long symptom-free intervals.

 The most common manifestations are malaise, fever, and

weight loss

 hypertension, usually developing rapidly

 abdominal pain and melena

 diffuse muscular aches and pains;

 and peripheral neuritis, predominantly affecting motor

nerves.

 Renal (arterial) involvement is common and a major

cause of death.
KAWASAKI DISEASE
 acute febrile, usually self-limited illness of infancy and
childhood

 affecting large to medium-sized, and even small, vessels

 Its clinical significance stems primarily from a

predilection for coronary artery involvement;

 such coronary arteritis can cause aneurysms that rupture

or thrombose, resulting in acute myocardial infarctions
 Cause and pathogenesis – unknown
 Vasculitis thought to result by T-cell and macrophage
activation in response to unknown antigen
 Morphology.
 As with polyarteritis nodosa, lesions exhibit pronounced
inflammation affecting the entire thickness of the vessel
wall

 however, fibrinoid necrosis is usually less prominent

 healed lesions may have obstructive intimal thickening

 Clinical Features.
 Kawasaki disease is also known as mucocutaneous
lymph node syndrome, because it presents with
 conjunctival and oral erythema and erosion
 edema of the hands and feet
 erythema of the palms and soles
 desquamative rash and
 cervical lymph node enlargement.
 Approximately 20% of untreated patients develop
cardiovascular sequelae,
 ranging from asymptomatic coronary arteritis

 to coronary artery ectasia and aneurysm formation

 to giant coronary artery aneurysms (7–8 mm) with

rupture or thrombosis, myocardial infarction, and
sudden death.
MICROSCOPIC POLYANGIITIS
 necrotizing vasculitis that generally affects capillaries, as
well as arterioles and venules

 Typically involves the skin, mucous membranes, lungs,

brain, heart, GIT, kidney, muscles.

 Necrotizing glomerulonephrititis and pulmonary

capillaritis are common.
Pathogenesis
 In many cases – immunologic reactions to drugs,
bacteria, heterologous proteins and tumor antigens.

 this can either result in immune complex deposition or

may trigger secondary immune responses

 However, most lesions are pauci-immune (devoid of

immune complexes)
 Morphology.
 Microscopic polyangiitis is characterized by segmental
fibrinoid necrosis of the media with focal transmural
necrotizing lesions

 In some areas (typically post-capillary venules), only

infiltrating and fragmenting neutrophils are seen, giving
rise to the term leukocytoclastic vasculitis
 Clinical Features.
 Depending on the vascular bed involved, major clinical
features include
 hemoptysis
 hematuria, and proteinuria
 bowel pain or bleeding
 muscle pain or weakness;
 palpable cutaneous purpura
CHURG-STRAUSS SYNDROME
 small-vessel necrotizing vasculitis classically associated
with
 Asthma
 allergic rhinitis
 lung infiltrates
 peripheral hypereosinophilia
 and extravascular necrotizing granulomas

 Vascular lesions can be histologically similar to

polyarteritis nodosa or microscopic polyangiitis but are
also characteristically accompanied by granulomas and
eosinophils
 P-ANCAs are present in less than half the cases

 Cutaneous involvement (palpable purpura),

gastrointestinal tract bleeding, and renal disease
(primarily as focal and segmental glomerulosclerosis)
are the major associations
WEGENER GRANULOMATOSIS
- Wegener granulomatosis is a necrotizing vasculitis
characterized by a triad of:-
 Acute necrotizing granulomas of the upper respiratory
tract (ear, nose, sinuses, throat) or the lower respiratory
tract (lung).

 Necrotizing or granulomatous vasculitis affecting small to

medium-sized vessels (e.g., capillaries, venules,
arterioles, and arteries), most prominent in the lungs and
upper airways but affecting other sites as well.

 Renal disease in the form of focal necrotizing, often

95
crescentic, glomerulonephritis .
Pathogenesis
 Wegener granulomatosis probably represents some
form of cell-mediated hypersensitivity response,
possibly to an inhaled infectious or other environmental
agent.

 c-ANCAs are present in up to 95% of cases

 they are a useful marker of disease activity, and may

participate in disease pathogenesis
Clinical Features
 Males are affected more often than are females, at an
average age of about 40 years.
 Classical features include
 persistent pneumonitis with bilateral nodular and
cavitary infiltrates (95%),
 chronic sinusitis (90%),
 mucosal ulcerations of the nasopharynx (75%), and
 evidence of renal disease (80%).
 If untreated, the course of the disease is malignant; 80%
of patients die within 1 year.
BUERGER DISEASE
 Thromboangiitis obliterans (Buerger disease) is a
distinctive disease that often leads to vascular
insufficiency.

 It is characterized by segmental, thrombosing acute and

chronic inflammation of medium-sized and small arteries

 principally the tibial and radial arteries

 with occasional secondary extension into extremity veins

and nerves.
Pathogenesis
 Buerger disease is a condition that occurs almost
exclusively in heavy smokers of cigarettes, usually
beginning before age 35

 The strong relationship to cigarette smoking is thought to

involve direct toxicity to endothelium by some tobacco
products, or an idiosyncratic immune response to the
same agents
Clinical Features
 Chronic ulcerations of the toes, feet, or fingers may
appear, perhaps followed in time by frank gangrene.

 In contrast to the vascular insufficiency caused by

atherosclerosis, in Buerger disease the insufficiency
tends to be accompanied by severe pain, even at rest,
related undoubtedly to the neural involvement.
INFECTIOUS VASCULITIS
 Localized arteritis may be caused by the direct invasion of
infectious agents, usually bacteria or fungi, and in
particular Aspergillus and Mucor species.

 Vascular invasion can be part of a more general tissue

infection (e.g., bacterial pneumonia or adjacent to
abscesses),

 less commonly-it may arise from hematogenous spread of

bacteria during septicemia or embolization from infective
endocarditis.
RAYNAUD PHENOMENON
 Raynaud phenomenon results from an exaggerated
vasoconstriction of digital arteries and arterioles.

 These vascular changes induce paroxysmal pallor or

cyanosis of the digits of the hands or feet

 infrequently, the nose, earlobes, or lips can also be involved.

 Characteristically, the involved digits show color changes in

the sequence white-blue-red , correlating with
vasoconstriction, cyanosis & vasodilation.
 Raynaud phenomenon may be a primary disease entity
or be secondary to a variety of conditions.
 Primary Raynaud phenomenon (previously called
Raynaud disease) reflects an exaggeration of central and
local vasomotor responses to cold or emotion,
 prevalence in the general population ranges 3% to 5%
and a predilection for young women
 Secondary Raynaud phenomenon refers to vascular
insufficiency of the extremities in the context of arterial
disease caused by other entities including
 SLE,
 Scleroderma
 Buerger disease,
 or even atherosclerosis
VEINS AND LYMPHATICS
 Varicose veins and phlebothrombosis/thrombophlebitis
together account for at least 90% of clinical disease associated
with veins.
 Varicose Veins

 Varicose veins are abnormally dilated, tortuous veins produced

by prolonged increase in intraluminal pressure and loss of
vessel wall support.

 The superficial veins of the upper and lower legs are typically
involved
 When legs are dependent for long periods, venous
pressures in these sites can be markedly elevated (up to
10 times normal) and can lead to venous stasis and pedal
edema, even in essentially normal veins .
 Persons older than 50,obesity & women (due the
elevated venous pressure in lower legs caused by
pregnancy) are at increased risk.
 A familial tendency toward premature varicosities
results from imperfect venous wall development.
Clinical Course
 Varicose dilation renders the venous valves incompetent
and leads to stasis, congestion, edema, pain, and
thrombosis.
 The most disabling sequelae include persistent edema in
the extremity and secondary ischemic skin changes
including
 stasis dermatitis and ulcerations
 poor wound healing and
 superimposed infections can become chronic varicose
ulcer
Thrombophlebitis and Phlebothrombosis
- The deep leg veins account for more than 90% of cases of
thrombophlebitis and phlebothrombosis; the two terms
are largely interchangeable designations for venous
thrombosis and inflammation.
Lymphangitis and Lymphedema
 Bacterial infections may spread into and through the
lymphatics to create acute inflammatory involvement in
these channels (lymphangitis).

 The most common etiologic agents are the group A β-

hemolytic streptococci
 Clinically, lymphangitis is recognized by painful
subcutaneous red streaks that extend along the course of
lymphatics, with painful enlargement of the regional
lymph nodes (acute lymphadenitis).

 If the lymph nodes fail to block the spread of bacteria,

spillage into the venous system can initiate a bacteremia
or septicemia
- Occlusion of lymphatic drainage is followed by the
abnormal accumulation of interstitial fluid in the affected
part, called obstructive lymphedema.
TUMORS
 Tumors of the blood vessels and lymphatics range from
benign hemangiomas, to intermediate lesions that are
locally aggressive but infrequently metastatic, to
relatively rare, highly malignant angiosarcomas.

 Congenital or developmental malformations and reactive

vascular proliferations (e.g., bacillary angiomatosis) can
also present as tumor-like lesions.
BENIGN TUMORS AND TUMOR-LIKE
CONDITIONS
Hemangioma
 Hemangiomas are very common tumors characterized by
increased numbers of normal or abnormal vessels filled
with blood
 These lesions constitute 7% of all benign tumors of
infancy and childhood.
 Most are present from birth and expand along with the
growth of the child, but many of the capillary lesions
eventually regress sponta
 The majority are superficial lesions, often of the head or
neck, but they can occur internally, with nearly one-third
being found in the liver
 Microscopic morphology:
Two types-capillary and cavernous.
1. Capillary: Small, back to back, capillary-like vessels.
• Outcome: Tend to regress.
• Age: More common in children.
• Portion of body affected: Skin and subcutaneous tissues.
2. Cavernous: Large, blood-filled spaces.
• Outcome: Do not tend to regress.
• Age: More common in adults.
• Portion of body affected: Deeper structures (e.g., liver).
 Lymphangiomas
 Lymphangiomas are the benign lymphatic analogues of
blood vessel hemangiomas
 Simple (Capillary) Lymphangioma.

 These are composed of small lymphatic channels

predominantly occurring in the head, neck, and axillary
subcutaneous tissues.
 They are slightly elevated or sometimes pedunculated
lesions up to 1 to 2 cm in diameter.
 Histologically, lymphangiomas exhibit networks of
endothelium-lined spaces that can be distinguished from
capillary channels only by the absence of erythrocytes.
 Cavernous Lymphangioma (Cystic Hygroma).
 These lesions are typically found in the neck or axilla of
children, and rarely occur in the retroperitoneum;

 cavernous lymphangiomas of the neck are common in

Turner syndrome .

 Cavernous lymphangiomas can occasionally be

enormous (up to 15 cm in diameter) and may fill the
axilla or produce gross deformities about the neck
 INTERMEDIATE-GRADE (BORDERLINE)
TUMORS.
Kaposi Sarcoma
 Kaposi sarcoma (KS) used to be fairly common in
patients with acquired immunodeficiency syndrome
(AIDS) prior to the advent of effective antiretroviral
therapy.

 Its presence is used as a criterion for diagnosing AIDS.

Pathogenesis
 95% of KS lesions have subsequently been shown to be
KS- associated herpes virus (KSHV) (also known as
human herpesvirus-8 ) infected.

 KSHV is a member of the γ-herpesvirus subfamily; it is

transmitted sexually and by poorly understood nonsexual
routes.
 Four forms of the disease are recognized (based on
population demographics and risks), all of these share
the same underlying viral pathogenesis
Chronic KS (also called classic or European KS)
 Characteristically occurs in older men of Eastern
European (especially Ashkenazi Jews) or Mediterranean
descent .
 While chronic KS can be associated with an underlying
second malignancy or altered immunity, it is not
associated with human immunodeficiency virus (HIV).
 Chronic KS presents with multiple red to purple skin
plaques or nodules, usually in the distal lower
extremities
Lymphadenopathic KS (also called African or endemic KS
 particularly prevalent among South African Bantu
children
 Skin lesions are sparse, and patients present instead with
lymphadenopathy due to KS involvement;
 the tumor occasionally involves the viscera and is
extremely aggressive
Transplant-associated KS
 occurs in the setting of solid-organ transplantation with
its attendant long-term immunosuppression.
 It tends to be aggressive (even fatal) with nodal,
mucosal, and visceral involvement
AIDS-associated (epidemic) KS
 was originally found in a third of AIDS patients,
particularly male homosexuals
 AIDS-associated KS can involve lymph nodes or viscera
and disseminates widely early in the course of the
disease
 Morphology
 Gross - reddish purple patches, plaques, or nodules over
the skin or mucosa

 Microscopic – proliferation of plump spindle cells with

extravasated red blood cells
MALIGNANT TUMORS
Angiosarcoma
 malignant endothelial neoplasms with histology varying
from highly differentiated tumors that resemble
hemangiomas (hemangiosarcoma) to anaplastic lesions
difficult to distinguish from carcinomas or melanomas.
 Older adults are more commonly affected, with equal
gender predilections; they occur at any site but most
often involve skin, soft tissue, breast, and liver.
 Hepatic angiosarcomas are associated with carcinogens
such as polyvinyl chloride(PVC)