Cv Module - Pathology Veins And Lymphatics

Front 1

Define Varicose Veins

Back 1

Involves superficial veins of the extremities

Results from inc. intraluminal pressure and loss of vessel wall support

Front 2

Clinical features of varicose veins

Back 2

Incompetance of venous valves produces:

1. Venous stasis
2. pedal edema
3. stasis dermatitis
4. skin ulcerations
5. superficial venous thrombosis (rarely embolize)

Front 3

Risk factors for varicose veins include

Back 3

1.prolonged sitting or standing increases venous pressure
3. persons > 50 years old'
4. obesity
5. women

Front 4

Histology of varicose veins

Back 4

Histologic change:
1. Intima: loss of endothelial integrity
2. Media: damaed smooth muscle cells increases collagen content
3. Adventitia: destruction of vasa vasorum, fibrosis

Front 5

Morphology of varicose veins

Back 5

1. Thin walled veins, dilated, tortuous, fibrotic
2. abnormal venous valves (weak, genetic predisposition to weak venous wall)
3. May contain intraluminal thrombosis, risk of emoblization is low
3. Phlebosclerosis (elastic fragmentation, smooth muscle hypertrophy, fibrosis, calcification)

Front 6

Sites other than deep leg veins for varicose veins + complications at those sites

Back 6

1. esophageal varicosities
2. anorectal hemorrhoids

Complications:
1. bleeding
2. thrombosis
3. skin and mucosal ulcerations

Front 7

Define Thrombophlebitis and Phlebothrombosis

Back 7

Thrombophlebitis - inflammatiion of vein associated with thrombus formation (phlebothrombosis)

thrombophlebitis (+)--> phlebothrombosis

Largely present in deep leg veins (90%)

Front 8

Clinical predispositions to thrombophlebitis and phlebothrombosis

Back 8

1. cardiac failure (acute mi) > 60yo
2. Paraneoplastic syndrome associated with malignancies
--> mucin-producing adenocarcinoma of pancreas, colon, lung
--> Trousseau syndrome (migratory thrombophlebitis)
3. Pregnancy
4. Obesity
5. Postoperative states assoc. with immobilization
6. Prolonged bed rest
7. Genetic hypercoagulability syndromes

Front 9

Additional sites (10%) for development of thrombi

Back 9

Deep leg veins (90%)

1. Periprostatic venous plexus in males
2. Pelvic veins in females
3. Large veins in skull and dural sinuses
4. Abdominal vessels, related to infections

Front 10

Clinical Signs of Thrombophlebitis

Back 10

Early stages have few symptoms:
1. Edema
2. dusky cyanosis
3. dilitation of superficial veins
4. heat, tenderness, redness, pain

**Signs may be absent in bed-ridden patients

Front 11

Clinical Signs of phlebothrombosis

Back 11

HOMAS Sign of DVT: pain associated with sqeezing the calf muscles or forced dorsiflexion of the foot, pressure to the affected vein

Pulmonary embolism may be the initial maniphestation

Plegmasia alba dolens: (3rd trimester, immediate post-delivery) iliofemoral thrombosis in pregnant women

Associated with stasis of blood ad hypercoagulable state in pregnancy

Front 12

Pathogenesis of thrombophlebitis

Back 12

Thrombosis --> phlebitis (inflammation reaction in the veins) --> lymphatic blockage --> painful white leg

Front 13

Define Superior Vena Caval Syndrome

Back 13

SVC compression or invasion by tumors (lung, lymphoma)

Front 14

Clinical features of SVC Syndrome

Back 14

Dusky cyanosis

Prominent dilation of the veins of the head, neck and arms

Respiratory distress secondary to compression of pulmonary vessels

Front 15

Define Inferior Vena Caval Syndrome

Back 15

IVC Compression or invasion by
1. Neoplasms (hepatocellular carcinoma, renal cell carcinoma)
2. Thrombus from femoral or iliac vein

Front 16

Clinical findings of IVC Syndrome

Back 16

1. Leg Edema
2. Dilated superficial collateral veins of the lower abdomen
3. Renal vein obstruction produces massive proteinuria

Front 17

Etiology Lymphangitis

Back 17

Primary disorders of lymphatics are uncommon

Related to infections with group A beta-hemolytic Strep

Front 18

Clinical findings of Lymphangitis

Back 18

Produces:
1. abscesses
2. cellulitis
3. extension into perilymphatic tissues
4. painful sub-q red streaks in the distribution of the affected lymphatic channel
5. painful enlargement of associated lymph nodes

Complications:
1. septicemia in venous system
involved
2. Acute lymphadenitis

Front 19

Define Lymphedema

Back 19

Occlusion of lymphatic drainage with accumulation of interstitial fluid

Front 20

Lymphadema occurs with

Back 20

1. Obstruction of the lymphatic channel by tumor (+)--> chylous ascites, chylothorax, chylopericardium
2. Radical lymphdenectomy
3. Radiation
4. Filariasis (elephanitits)
5. Postinflammatory thrombosis and scarring

Front 21

Define Primary lymphadema

Back 21

Idiopathic ascending lymphadema spreads from feet/leg to trunk in young females and predisposes to infection

Front 22

Etiologies of Primary lymphadema

Back 22

1. Isolated primary condition (simple congential lymphedema)
2. Milroy Disease (heredofamilial congenital lymphedema)
3. Lymphedema praecox (premature) - in young females

Front 23

Pathogenesis of Primary lymphadema

Back 23

Lymphedema (+)--> lymphatic dilation/obstruction (+)--> increases interstitial fluid, sub-Q fibrosis and skin changes

Skin changes when advanced:
1. Peau d' orange appearance (obstruction of breast lymphatics)
2. ulceration

Front 24

Describe tumors of blood vessels, how are they detected?

Back 24

A tumor whose cells tend to form blood vessels or lymphatic vessels
OR a tumor made of BV or L

Vascular origin confirmed by immunohistochemistry (vWF, CD31, CD34)

Front 25

Distinguish between Benign and Malignant vascular tumors

Back 25

Benign - easily discerned BV or lymphatics line by normal endothelial cells
Examples:
Hemangioma
Pyogenic granuloma (Lobular Capillary Hemangioma)
Lymphngioma
Glomangioma
Vascular ectasias(telegectasia)
Bacillary angiomatosis
pyogenic granuloma

Intermediate grade tumors (hemangioendothelioma)

Malignant tumors - usually cellular with anaplasia, poorly formed vessels
Examples:
Angiosarcoma
Hemangiopericytoma
Kaposi's Sarcoma

Front 26

Hemangioma - who gets them and where are they located, clinical significance?

Back 26

Represents 7% of all benign tumors in infancy and childhood

Usually localized, but may be diffuse (angiomatosis)

Common locations - head, neck , liver

--most often of little clinical significance

Front 27

Hemangioma variant:

von Hipple-Lindau disease - hemangioma manifestations

Back 27

Hemangiomas in cerebellum, brain stem, eye

Angiomatous lesions or cystic neoplasms in pancreas, liver, other organs

Front 28

Hemangioma histological variant:

Capillary Hemangioma

Back 28

Tumor composed of closely packed aggregation of capillaries separated by scant CT

--conforms to caliber of normal capillary

Front 29

Capillary Hemangioma histological variant:

PYOGENIC GRANULOMA
(Lobular Capillary Hemangioma)

Back 29

--Polyploid growth with attached stalk
--Located on skin, gingiva, oral mucosa
--Induced by trauma

Histology:
--max size of 1-2 cm
--Edema, acute and chronic inflammation
--ulceration

Special variant:
GRANULOMA GRAVIDARUM
--gingiva of pregnant women
--regresses after delivery
--stimulated by estrogen

Front 30

Hemangioma histological variant:

Cavernous hemangioma

Back 30

Vascular tumor composed of dilated BV containing large amounts of blood

Occur in skin, viscera (liver, spleen, pancreas)

Not usually present at birth

Typical superficial lesion is dark red to deep blue

AKA: angioma cavernosum, strawberry mark, nevus

Front 31

Lymphangioma Variant:

Lymphangioma Circumscriptum (Capillary Lymphangioma)

Back 31

Lymphatic channels filled with exudate and not red blood cells

Subcutaneous lesions in axilla, head, neck region

Front 32

Lymphangioma Variant:

Cavernous Lymphangioma (Cystic Hygroma)

Back 32

1. Neck of children, axilla, rarely retroperitoneal
2. Size up to 15cm
3. Composed of increased numbers of dilated, cystic lymphatic spaces
4. NOT encapsulated
5. Occur in TURNER SYNDROME

Front 33

Describe Glomus tumor (Glomangioma)

Back 33

1. Benign, painful lesion
2. MC present under fingernails
3. Arise from modified smooth muscle cels of the glomus body

Front 34

Morphology of Glomus tumor (Glomangioma)

Back 34

Nodules:
--Small, >1cm
--slightly elevated
--red-blue
--firm

Consist of masses of glomus cells arranged around blood vessels

Resemble cavernous hemangiomas

Front 35

Define Vascular Ectasias

Back 35

Abnormal dilation of of preformed capillaries, venules, arterioles that create a small, focal lesion

Front 36

Examples of Vascular Ectasias

Back 36

1. Nevus flammeus (birthmark)
2. Spider telengiectasia
3. Hereditary hemorrhagic telangiectasia
(Osler-Weber-Rendu disease)
4. Sturge Weber Syndrome: includes port wine stain in the distribution of trigeminal nerve and may be associated with
--venous malformations involving the meninges
--mental retardation
--seizures
--hemiplegia

Front 37

Describe Spider telengiectasias

Back 37

Type of vascular ectasia:

Arterial spiders - pulsatile array of dilated sub-q arteries and arterioles about a central core

--Blanch with applied pressure
--Located on face, neck upper chest
--Occur in pregnant and cirrhotics

Front 38

Describe Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease)

Back 38

Type of vascular ectasia:

1. Autosomal dominant
2. Consists of dilated capillaries and veins
3. Located in skin, mucous membranes ( of oral cavity, lips, respiratory, GI, and urinary tracts)

Complications:
Prone to rupture (nosebleeds, hematuria, GI bleeds)

Front 39

Describe Bacillary Angiomatosis

Back 39

Opportunistic infection in immunocomprimised patients with AIDS

Vascular proliferations in skin, bone, brain, other organs

Tumor-like proliferation of capillaries with prominent epithelioid and endothelial cells with mitosis and nuclear atypia

Front 40

Etiology of Bacillary Angiomatosis

Back 40

Infections with gram negative organisms of the Bartonella group
--Bartonella Henslae (Cat Scratch Ds)
--B. quintana (Trench fever, soldiars WWII)

Front 41

Histology of Bacillary Angiomatosis

Back 41

Bacteria present as purplish granular material

Similar histologically to pyogenic granuloma, Kaposi sarcoma, angiosarcoma

Front 42

Intermediate-grade Vascular tumor

Describe Kaposi's Sarcoma

Back 42

1. Classic or European form
2. Lymphadenopathic (African or endemic Kaposi's)
3. Transplant-associated
4. AIDS-associated (epidemic)
5. Possible role - HSV 8 Virus

Front 43

Tumors of lymphatics

Back 43

capillary lymphangioma
cavernous lymphangioma (cystic hygroma)
lymphangiosarcoma (lyphedema-associated angiosarcoma)

Front 44

Malignant Vascular tumor

Describe Hemangiopericytoma

Back 44

Tumor composed of spindle cells with rich vascular network

Arise from pericytes

Occur in lower extremities or in retroperitoneum

Front 45

Intermediate-grade neoplasms

Back 45

Kaposi Sarcoma

Hemangioendothelioma

Front 46

Intermediate-grade neoplasms

Back 46

Kaposi Sarcoma

Hemangioendothelioma

Front 47

What are the etiologies and presentations of Kaposi Sarcoma subtypes?

Back 47

1. Classical or European form - in elderly men, indolent
2. Lymphadenopathic - endemic (African) - black African yound men and children
3. Transplant pts (immunocomp)
4. Epidemic - AIDS

Front 48

What are the etiologies and presentations of Kaposi Sarcoma subtypes?

Back 48

1. Classical or European form - in elderly men, indolent
2. Lymphadenopathic - endemic (African) - black African yound men and children
3. Transplant pts (immunocomp)
4. Epidemic - AIDS

Front 49

Intermediate-grade neoplasms

Back 49

Kaposi Sarcoma

Hemangioendothelioma

Front 50

Describe the pathogenesis and clinical course of Kaposi Sarcoma

Back 50

1. derived from primitive mesenchymal or endothelial cells
2. locally produced growth factors --> cellular overgrowth
3. relationship to HIV produces and HHV-8

Clinical course:
--classic form associated with long survival
--endemic and epidemic forms are aggressive and death occurs within 2-5 years

Front 51

Describe the pathogenesis and clinical course of Kaposi Sarcoma

Back 51

1. derived from primitive mesenchymal or endothelial cells
2. locally produced growth factors --> cellular overgrowth
3. relationship to HIV produces and HHV-8

Clinical course:
--classic form associated with long survival
--endemic and epidemic forms are aggressive and death occurs within 2-5 years

Front 52

Histopathology of Kaposi Sarcoma

Back 52

Patches, Plaques, Nodules

"EARLY": small vessels, normal endothelial cells, red cell extravasation, macs, hemosiderin, lymphs

"MID" dilated, irregularly shaped vessels, perivascular spindle-shaped cells, hemosiderin-laden macs, hyaline droplets, inflamm cells

"LATE" vessels indistinct, slit-like spaces with RBCs, increased spindle-shaped cells, hemorrhage, hemosiderin

Front 53

What are the etiologies and presentations of Kaposi Sarcoma subtypes?

Back 53

1. Classical or European form - in elderly men, indolent
2. Lymphadenopathic - endemic (African) - black African yound men and children
3. Transplant pts (immunocomp)
4. Epidemic - AIDS

Front 54

Malignant Vascular Tumor

Angiosarcoma

Back 54

Etiology: associated with chemical carcinogens (arsenicals, thorotrast, PVC)

Histopathology: increased numbers of spindle-shaped cells and poorly formed, somewhat inconspicuous vascular channels

Front 55

Describe the pathogenesis and clinical course of Kaposi Sarcoma

Back 55

1. derived from primitive mesenchymal or endothelial cells
2. locally produced growth factors --> cellular overgrowth
3. relationship to HIV produces and HHV-8

Clinical course:
--classic form associated with long survival
--endemic and epidemic forms are aggressive and death occurs within 2-5 years

Front 56

Histopathology of Kaposi Sarcoma

Back 56

Patches, Plaques, Nodules

"EARLY": small vessels, normal endothelial cells, red cell extravasation, macs, hemosiderin, lymphs

"MID" dilated, irregularly shaped vessels, perivascular spindle-shaped cells, hemosiderin-laden macs, hyaline droplets, inflamm cells

"LATE" vessels indistinct, slit-like spaces with RBCs, increased spindle-shaped cells, hemorrhage, hemosiderin

Front 57

Histopathology of Kaposi Sarcoma

Back 57

Patches, Plaques, Nodules

"EARLY": small vessels, normal endothelial cells, red cell extravasation, macs, hemosiderin, lymphs

"MID" dilated, irregularly shaped vessels, perivascular spindle-shaped cells, hemosiderin-laden macs, hyaline droplets, inflamm cells

"LATE" vessels indistinct, slit-like spaces with RBCs, increased spindle-shaped cells, hemorrhage, hemosiderin

Front 58

Malignant Vascular Tumor

Angiosarcoma

Back 58

Etiology: associated with chemical carcinogens (arsenicals, thorotrast, PVC)

Histopathology: increased numbers of spindle-shaped cells and poorly formed, somewhat inconspicuous vascular channels

Front 59

Malignant Vascular Tumor

Angiosarcoma

Back 59

Etiology: associated with chemical carcinogens (arsenicals, thorotrast, PVC)

Histopathology: increased numbers of spindle-shaped cells and poorly formed, somewhat inconspicuous vascular channels