Pathology 9 &10: Peripheral Venous and Lymphatic disease Flashcards

1
Q

Vericose Veins

A

Abnormally dilated, tortuous veins

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2
Q

Causes of Vericose Veins (2)

A

Prolonged, increased intraluminal pressure

Loss of vessel wall support (vein walls are much more weak than arterial walls)

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3
Q

Which veins typically become vericose ?

A

Superficial veins

Upper and lower leg

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4
Q

Is there a familial link to Varicose veins ?

A

You know it ! Due to defective development of vein walls

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5
Q

The most troublesome complications from varicose veins are persistent edema in the extremity and Ischemic skin changes. What are some of theses skin changes ?

A

Stasis dermatitis

Cutaneous ulceration

These contribute to poor wound healing , infections –> osteomyelitis if infection gets into bone, This can lead to amputation of the limb

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6
Q

What causes esophageal varices to form ?

A

Portal Vein HTN ( Cirrhotic liver)

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7
Q

With cirrhosis and subsequent portal vein hypternsion, where else might you see varices ?

A
Rectum (hemorrhoids) 
Periumbilical veins (caput medusae)
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8
Q

What is the most dangerous association with esophageal varicoses ?

A

RUPTURE –> massive upper GI bleeds

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9
Q

What non-cirrhotic conditions can lead to hemorrhoids ?

A

Prolonged pelvic vascular congestion
Pregnancy
Chronic constipation
Increased intra-abdominal pressure

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10
Q

Thrombophelbitis/Phlebothrombitis

A

Venous thrombosis and inflammation

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11
Q

What are some state that can potentiate Venous Thrombosis ?

A

cardiac failure, neoplasia, pregnancy, obesity, post-op state, prolonged bed rest/immobilization

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12
Q

Where do most cases of thrombophlebitis occur in the body ?

A

Deep leg veins

Other sites:

Periprostatic venous plexus (males)
Pelvic venous plexus (females)
Large veins in the skull and the dural sinuses (infection or inflammation)
Portal vein thrombosis
Peritoneal infections, appendicitis, salpingitis
and pelvic abscesses

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13
Q

Explain how carcinoma can lead to thrombophlebitis

A

Elaboration of pro-coagulant factors by the tumor cells

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14
Q

Migratory thrombophlebitis (thrombophlebitis migrans) exhibits will exhibit “trousseaus sign” or clotting in many areas of the body w/o order. What is this indicative of

A

Cancer (adenocarcinoma)

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15
Q

What is the most serious complication of DVT (phlebothrombosis of the deep leg veins) ?

A

Pulmonary embolism

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16
Q

What is often the first sign of Thrombophlebitis ?

A

PE ! Sadly, many don’t realize they have a DVT until it is too late.

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17
Q

Lymphangitis (define)

A

Acute inflammation in lymphatic channels

Dilated and filled with inflammatory infiltrate
Composed of neutrophils and histiocytes
Extends through the vessel wall
Perilymphatic tissues
Cellulitis or focal abscesses (severe cases

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18
Q

Most common agent of lymphangitis ?

A

Group A β-hemolytic streptococci

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19
Q

Clinical features of lymphangitis

A

Painful subcutaneous red streaks
Extend along the course of lymphatics
Painful enlargement of regional lymph nodes
If lymph nodes fail to block bacteria
Drainage into venous circulation
Bacteremia or septicemia

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20
Q

Primary Lymphedema

A

Isolated congenital defect (simple congenital lymphedema)

Often due to agenesis or hypoplasia or lymph tissue

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21
Q

Famililal Milroy disease

A

Heredofamilial congenital lymphedema

Lymphatic agenesis or hypoplasia

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22
Q

Secondary Lymphedema

A

Occlusion of lymphatic vessels
Abnormal accumulation of interstitial fluid

Caused by:
Malignancy (efacement of lymphnode and
destruction of lymph tissue)

 Post surgical
 Post irradiation fibrosis 

 Post inflamm scarring

 Obstruction due to filiarisis
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23
Q

Persistent edema seen in lymphedema leads to subcutaneous interstitial fibrous tissue and induration. Induration is often referred to by its strange appearance, what is it ?

A

Peau d’orange (orange peel)

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24
Q

In lymphedema, lymphatics may rupture leading to milky collections of lymph in potential spaces. What is a major lymph structure that could rupture leading to this ?

A

Thoracic duct

Places where lymph will accumulate by name:
Chylous ascites (peritoneum)
Chylothorax (pleural cavity)
Chylopericardium (pericardium

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25
Q

A common cause of lymph rupture is ….

A

malignant invasion.

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26
Q

What kind of tumors are primarily found in the aorta,plum art and vena cava ?

A

Connective Tissue Sarcoma’s

Extremely rare

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27
Q

Endothelium derived neoplasms include

A

Hemangioma, Lymphangioma and Angiosarcoma

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28
Q

Tumors arising from cells that support vessels include

A

Glomus tumor and hemangiopericytoma

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29
Q

Hemangioma

A

Benign and extremely common tumors compose of increased # of normal or abnormal vessels filled w/ blood.

Rare turn malignant

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30
Q

Where do you find most hemangiomas

A

head and neck (superficial)

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31
Q

Where can you find internal hemangiomas

A

The liver (1/3 of all)

32
Q

Capillary hemangioma

A

Most common
Composed of a proliferation of benign capillaries

Found in:

Skin and subcutaneous tissues

Mucous membranes of the oral cavities and lips

Liver, spleen, and kidneys

33
Q

Physical description of capillary hemangioma

A

Bright red to blue
Millimeters to several centimeters in diameter
Flat or elevated

34
Q

Histologic description of Capillary Hemangioma

A

Unencapsulated aggregates of closely packed, thin-walled capillaries
Blood-filled and lined by flattened endothelium
Scant connective tissue stroma separates vessels
Lumens may be partially or completely thrombosed

35
Q

Describe “strawberry type” capillary hemangioma

A

AKA Juvenile hemangioma of the skin of newborns
Extremely common
May be multiple
Grows rapidly in the first few months
Fades at 1 to 3 years of age
Completely regresses by age 7 (75% to 90% of
cases)

36
Q

Carvenous Hemangioma

A
Large(compared to the capillary hemangioma), dilated vascular channels
Less well circumscribed 
Involve deep structures
Brain hemangiomas--most problematic
      Pressure symptoms or rupture
37
Q

Cavernous Hemangiomas are a component of what disease

A

Von Hippel Lindau disease (Cerebellum,brain stem and retina)

38
Q

Physical description of Cavernous Hemangioma

A

Red-blue, soft, spongy masses
1 to 2 cm in diameter
Sharply defined
Composed of large, cavernous blood-filled vascular spaces
Separated by a modest connective tissue
stroma

39
Q

In Cavernous hemangioma, intravascular thrombosis is associated with …

A

dystrophic calcification

40
Q

Pyogenic Granuloma

A

Essentially it is a capillary hemangioma which has ulcerated (acute inflammation )

Rapidly growing pedunculated red nodule
Skin, gingival or oral mucosa
Bleeds easily; often ulcerated

Due to proliferating capillaries –> extensive edema

Shows acute and chronic inflammatory infiltrate

41
Q

Lymphangiomas

A

Benign proliferations of lymph tissue

42
Q

Simple lymphangioma

A

Small lymphatic channels in the head, ncke and axial sub cue tissues.

Elevated or pedunculated (1/2/ cm in diameter)

No erythrocytes

43
Q

Cavernous lymphangioma

A

Neck or axila of children
Large (15 cm in diameter) –> gross deformity of the neck

Massively dialated, seperated by intervening connective tissue stroma

44
Q

Cavernous lymphangioma is associated with what genetic disease ?

A

Turner syndrome

45
Q

GLomus tumor

A

Benign, exquisitely painful tumors

Arises from modified smooth muscle cells of the glomus body (Specialized arteriovenous structure involved in thermoregulation).

Located anywher eon the skin but often found under the nails on digits

46
Q

Physical description of GLomus tumor

A

Round, slightly elevated, red-blue

Firm nodules (<1 cm in diameter)

Aggregates, nests, and masses of specialized glomus cells
Branching vascular channels

47
Q

Vascualar ectasias

A

Group of abnormally prominent capillaries, venules and arterioles
Creates a focal red lesion usually in skin or
mucous membranes

48
Q

Nevus flammeus

A

“Birthmark”
Most common form of ectasia
Head or neck; light pink to deep purple
Vascular dilation of blood vessels in dermis

49
Q

Port Wine Stain

A

Special form of nevus flammeus; grows with child
Thickens skin surface

Distributed over Trigeminal Nerve

50
Q

What disease is Port Wine Stain often associated with ?

A

Sturge-Weber syndrome
encephalotrigeminal angiomatosis
Rare congenital disorder
Associations
Venous angiomatous masses in cortical
leptomeninges
Ipsilateral facial port wine nevi
Mental retardation, seizures, hemiplegia

51
Q

Spider Telangiectasia

A

Non neoplastic lesion that blanches when pressure is applied to it

Focal network of subcutaneous small arteries or arterioles
Arranged in a radial fashion around a central core

52
Q

Who mostly gets Spider Telangiectasia ?

A

Pregnant women or patients with cirrhosis
Increased estrogen levels ! Estrogen not broken
down in liver due to cirrhosis

53
Q

Hereditary Hemorrhagic Telangiectasia (Osler-Weber-Rendu Disease)

A

Dont confuse with Sturge Weber (associated with port wine stain)

Autosomal dominant disorder
Multiple aneurysmal telangiectasias
    Skin and oral mucous membranes
    Respiratory, GI, urinary tracts
Present from birth
Lesions rupture (occasional)
    Serious epistaxis, GI bleed, or hematuria
54
Q

Bacillary Angiomatis

A

Vascular proliferation due to opportunistic infections in immunocompromised patients

Involve skin bone brain others.

Due to gran negative bacilli in the Bartonella family.

55
Q

Physical description of a Bacillary Angiomatosis

A

Skin lesions
Red papules and nodules
Rounded subcutaneous masses
Capillary proliferation with epithelioid endothelial cells
Nuclear atypia and mitoses
Lesions contain neutrophils, nuclear dust, and bacteria

56
Q

Diagnosis of Bacillar Angiomatis

A

PCR !

57
Q

Treatment of Bacillary Angiomatis

A

Macrolide antibiotics

58
Q

Kaposi Sarcoma (Forms )

A

Intermediate Grade Neoplasm

Common in AIDS patients

Chronic KS
    AKA classic or European KS
Lymphadenopathic KS
     AKA African or endemic KS
Transplant-associated KS
AIDS-associated (epidemic) KS
59
Q

Chronic Kaposi Sarcoma

A

Can see with underlying malignancy; not HIV
Multiple red to purple skin plaques or nodules
Distal lower extremities
Increase in size and number; spread proximally
Locally persistent; asymptomatic
Remain localized to skin and subcutaneous tissue

60
Q

Who will you most likely see Chronic Kaposi in ?

A

Older men of Eastern European (Ashkenazi Jews) or Mediterranean descent

61
Q

Lymphadenopathic KS

A

Same geographic distribution as Burkitt lymphoma
Prevalent among South African Bantu children
Not associated with HIV
Skin lesions are sparse
Lymphadenopathy
Extremely aggressive

62
Q

Transplant-associated KS

A
Solid-organ transplantation
    Long-term immunosuppression
Aggressive (even fatal)
Nodal, mucosal, and visceral involvement
Cutaneous lesions may be absent

occasionally these regress, may lower immunosupressives

63
Q

AIDS-associated (epidemic) KS

A
Originally found in 1/3rd of AIDS patients
Male homosexuals
Regimens of antiretroviral therapy
Incidence is less than 1% 
Lymph nodes or viscera
Disseminates widely early in disease
64
Q

What cuases Kaposi sarcoma ?

A

Human herpesvirus-8 (HHV-8)

Sexually transmitted or via saliva

65
Q

Kaposi: Patch Stage

A

Red to purple macules
Confined to the distal lower extremities
Dilated irregular endothelial cell-lined vascular spaces
Interspersed lymphocytes, plasma cells, and macrophages

66
Q

Kaposi: Plaque Stage

A

Over time, spread proximally
Larger, violaceous (more purple), raised
Dermal accumulations of dilated, jagged vascular channels
Lined and surrounded by plump spindle cells
Extravasated red cells, hemosiderin-laden macrophages, and other mononuclear inflammatory cells

67
Q

Kaposi: Nodule Stage

A
Distinctly neoplastic
Sheets of plump, proliferating spindle cells
Dermis or subcutaneous tissues
Small vessels and slit-like spaces
Contain red cells
More marked hemorrhage, hemosiderin pigment, and mononuclear inflammation
Mitotic figures are common
Heralds nodal and visceral involvement
African and AIDS-associated variants
68
Q

Hemangioendothelioma

A

Intermediate Grade Neoplasm wide spectrum

69
Q

Epithelioid hemangioendothelioma

A

Vascular tumor of adults
Around medium-sized and large veins
Plump and cuboidal tumor cells
Inconspicuous well-defined vascular channels

70
Q

Angiosarcomas

A

Group of malignant endothelial neoplasms
Anaplastic –> Revert back to stem cell like structure.

More often affects older adults

Skin soft tissue breast an liver

71
Q

What are angiosarcomas associated with (chemicals) ?

A

Associated with carcinogen exposures

Arsenic (arsenical pesticides)
Thorotrast
 Radioactive contrast agent formerly used for
  radiologic imaging
Polyvinyl chloride (PVC)
72
Q

Describe angiosarcoma and association with lymphedema

A

Several years after radical mastectomy
Lymph node resection no lymph drainage. Will
be in ipsilateral upper extremity.

Ipsilateral upper extremity

Tumor arises from lymphatic vessels (lymphangiosarcoma)

73
Q

Physical description of Angiosarcoma

A

Small, sharply demarcated, asymptomatic, red nodules
Large, fleshy masses of red-tan to gray-white tissue
Margins blend with surrounding structures
Central areas of necrosis and hemorrhage

74
Q

Histologic representation of angiosarcoma

A

Plump, anaplastic endothelial cells producing vascular channels
Wildly undifferentiated tumors
Solid spindle cell appearance and without
definite blood vessels
CD31 or von Willebrand factor

Locally Invasive, metastasize readily

Aggressive tumors 5 year survival rates approaching 30%

75
Q

Hemangiopericytoma

A

Rare neoplasm

Derived from pericytes-myofibroblast-like cells
Arranged around capillaries and venues

Slowly enlarging, painless masses

76
Q

Where are hemangiopericytomas most common ?

A

Most common
Lower extremities (thigh)
Retroperitoneum

77
Q

Histologic description of Hemangiopericytoma

A

Numerous branching capillary channels
Gaping sinusoidal spaces enclosed within nests of spindle-shaped to round cells
Recur after excision
About half will metastasize
Hematogenously to lungs, bone, or liver