Glossary of CV module - Pathology Veins and Lymphatics

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Define Varicose Veins
Involves superficial veins of the extremities

Results from inc. intraluminal pressure and loss of vessel wall support
Clinical features of varicose veins
Incompetance of venous valves produces:

1. Venous stasis
2. pedal edema
3. stasis dermatitis
4. skin ulcerations
5. superficial venous thrombosis (rarely embolize)
Risk factors for varicose veins include
1.prolonged sitting or standing increases venous pressure
3. persons > 50 years old'
4. obesity
5. women
Histology of varicose veins
Histologic change:
1. Intima: loss of endothelial integrity
2. Media: damaed smooth muscle cells increases collagen content
3. Adventitia: destruction of vasa vasorum, fibrosis
Morphology of varicose veins
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)
Sites other than deep leg veins for varicose veins + complications at those sites
1. esophageal varicosities
2. anorectal hemorrhoids

1. bleeding
2. thrombosis
3. skin and mucosal ulcerations
Define Thrombophlebitis and Phlebothrombosis
Thrombophlebitis - inflammatiion of vein associated with thrombus formation (phlebothrombosis)

thrombophlebitis (+)--> phlebothrombosis

Largely present in deep leg veins (90%)
Clinical predispositions to thrombophlebitis and phlebothrombosis
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
Additional sites (10%) for development of thrombi
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
Clinical Signs of Thrombophlebitis
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
Clinical Signs of phlebothrombosis
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
Pathogenesis of thrombophlebitis
Thrombosis --> phlebitis (inflammation reaction in the veins) --> lymphatic blockage --> painful white leg
Define Superior Vena Caval Syndrome
SVC compression or invasion by tumors (lung, lymphoma)
Clinical features of SVC Syndrome
Dusky cyanosis

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

Respiratory distress secondary to compression of pulmonary vessels
Define Inferior Vena Caval Syndrome
IVC Compression or invasion by
1. Neoplasms (hepatocellular carcinoma, renal cell carcinoma)
2. Thrombus from femoral or iliac vein
Clinical findings of IVC Syndrome
1. Leg Edema
2. Dilated superficial collateral veins of the lower abdomen
3. Renal vein obstruction produces massive proteinuria
Etiology Lymphangitis
Primary disorders of lymphatics are uncommon

Related to infections with group A beta-hemolytic Strep
Clinical findings of Lymphangitis
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

1. septicemia in venous system
2. Acute lymphadenitis
Define Lymphedema
Occlusion of lymphatic drainage with accumulation of interstitial fluid
Lymphadema occurs with
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
Define Primary lymphadema
Idiopathic ascending lymphadema spreads from feet/leg to trunk in young females and predisposes to infection
Etiologies of Primary lymphadema
1. Isolated primary condition (simple congential lymphedema)
2. Milroy Disease (heredofamilial congenital lymphedema)
3. Lymphedema praecox (premature) - in young females
Pathogenesis of Primary lymphadema
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
Describe tumors of blood vessels, how are they detected?
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)
Distinguish between Benign and Malignant vascular tumors
Benign - easily discerned BV or lymphatics line by normal endothelial cells
Pyogenic granuloma (Lobular Capillary Hemangioma)
Vascular ectasias(telegectasia)
Bacillary angiomatosis
pyogenic granuloma

Intermediate grade tumors (hemangioendothelioma)

Malignant tumors - usually cellular with anaplasia, poorly formed vessels
Kaposi's Sarcoma
Hemangioma - who gets them and where are they located, clinical significance?
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
Hemangioma variant:

von Hipple-Lindau disease - hemangioma manifestations
Hemangiomas in cerebellum, brain stem, eye

Angiomatous lesions or cystic neoplasms in pancreas, liver, other organs
Hemangioma histological variant:

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

--conforms to caliber of normal capillary
Capillary Hemangioma histological variant:

(Lobular Capillary Hemangioma)
--Polyploid growth with attached stalk
--Located on skin, gingiva, oral mucosa
--Induced by trauma

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

Special variant:
--gingiva of pregnant women
--regresses after delivery
--stimulated by estrogen
Hemangioma histological variant:

Cavernous hemangioma
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
Lymphangioma Variant:

Lymphangioma Circumscriptum (Capillary Lymphangioma)
Lymphatic channels filled with exudate and not red blood cells

Subcutaneous lesions in axilla, head, neck region
Lymphangioma Variant:

Cavernous Lymphangioma (Cystic Hygroma)
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
Describe Glomus tumor (Glomangioma)
1. Benign, painful lesion
2. MC present under fingernails
3. Arise from modified smooth muscle cels of the glomus body
Morphology of Glomus tumor (Glomangioma)
--Small, >1cm
--slightly elevated

Consist of masses of glomus cells arranged around blood vessels

Resemble cavernous hemangiomas
Define Vascular Ectasias
Abnormal dilation of of preformed capillaries, venules, arterioles that create a small, focal lesion
Examples of Vascular Ectasias
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
Describe Spider telengiectasias
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
Describe Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease)
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)

Prone to rupture (nosebleeds, hematuria, GI bleeds)
Describe Bacillary Angiomatosis
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
Etiology of Bacillary Angiomatosis
Infections with gram negative organisms of the Bartonella group
--Bartonella Henslae (Cat Scratch Ds)
--B. quintana (Trench fever, soldiars WWII)
Histology of Bacillary Angiomatosis
Bacteria present as purplish granular material

Similar histologically to pyogenic granuloma, Kaposi sarcoma, angiosarcoma
Intermediate-grade Vascular tumor

Describe Kaposi's Sarcoma
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
Tumors of lymphatics
capillary lymphangioma
cavernous lymphangioma (cystic hygroma)
lymphangiosarcoma (lyphedema-associated angiosarcoma)
Malignant Vascular tumor

Describe Hemangiopericytoma
Tumor composed of spindle cells with rich vascular network

Arise from pericytes

Occur in lower extremities or in retroperitoneum
Intermediate-grade neoplasms
Kaposi Sarcoma

What are the etiologies and presentations of Kaposi Sarcoma subtypes?
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
Describe the pathogenesis and clinical course of Kaposi Sarcoma
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
Histopathology of Kaposi Sarcoma
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
Malignant Vascular Tumor

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

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

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