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DEC-2014-CSBRP

CVS - MiscellaneousCSBR.Prasad, MD.,

DEC-2014-CSBRP

Raynaud’s phenomenon (RP)

• Paroxysmal exaggerated vasoconstriction of digital arteries and arterioles

• Results in cyanosis and pallor• Sometimes may affect: Nose, ear and lips• Types:

– Primary RP • Response to cold / emotional stress• CF: Atrophic changes may develop in the course of time

– Secondary RP• SLE, Scleroderma, Buerger’s disease

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Raynaud’s phenomenon

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Buerger’s diseaseThromboangiitis obliterans - TAO

• Def: It’s segmental, thrombosing arteritis affecting small & medium sized vessels of acute or chronic nature

• Men <40yrs• Smokers• Arteries: Tibial, femoral, peroneal & popleteal• Upper limb involvement – 70%• More common in Asians than in Western countries

DEC-2014-CSBRP

Buerger’s diseaseThromboangiitis obliterans - TAO

Morphology:• Thrombosis• Initially: Neutrophil exudate in all the layers –

microabscesses• Later: lymphocytes and macrophages,

occasionally giant cells• Musculature of the media and elastic laminae

are preserved

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Buerger’s diseaseThromboangiitis obliterans - TAO

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Buerger’s diseaseThromboangiitis obliterans - TAO

Clinical features:• Intermittent claudication

– In the instep– Less commonly in the calf– Rarely in the upper limbs

• Later: pain comes on at night and is relieved by walking / hanging the leg over the side of bed

• Pulse may be absent in the small vessels like Dorsalis pedis and Radial

• Raynaud’s phenomenon– 50% of patients develop this when the hands are exposed to

cold• Migratory superficial thrombophlebitis• Gangrene

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Buerger’s diseaseThromboangiitis obliterans - TAO

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Buerger’s diseaseThromboangiitis obliterans - TAO

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Varicose veins

• Abnormally dilated tortuous veins • Due to:

– Prolonged increased pressure within– Loss of vessel wall support

• Site: Superficial vessels of lower limbs are affected• Women more commonly affected• Obesity increases the risk• Familial tendencies are seen in some• CF: Edema, Stasis dermatitis, Ulceration, ThombosisNOTE: Embolism is not a feature of thrombosis of

superficial veins. It’s commonly associated with DVT

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Stasis dermatitis

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Varicose veins

• Other sites for varocosities:– Esophageal varices (due to PHT)– Hemorrhoides (due to PHT, Pregnancy,

Constipation)– Varicocele

• Complication: Bleeding

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Esophageal varices in PHT

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Portal hypertension (PHT)

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Varococele

What is the importance of

ACUTE left sided varicocele ?

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Thrombophlebitis & Phlebothrombosis

• Interchangeable terms• Components:

– Venous thrombosis and – Inflammation

• Sites:– Deep veins of legs– Periprostatic plexus in male– Pelvic venous plexus in female– Large veins of skull & dural sinuses

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Thrombophlebitis & Phlebothrombosis

Causes:• Peritoneal infections

– Appendicitis– Salpingitis

• PLT hyperactivity– PCV– Myelofibrosis

• Prolonged immobilization• Systemic hypercoagulability

– Adenocarcinoma• Complications of DVT

– Pulmonary embolism

Trousseau’s sign(Migratory thrombophlebitis)

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Known for:Trousseau sign of malignancyTrousseau sign of latent tetanyTrousseau-Lallemand bodies

"use new drugs quickly, while they still work"

During his later years Trousseau developed pancreatic cancer. Coincidentally, he previously described Trousseau sign of malignancy

and developed a similar finding in himself. This cancer limited his activities and eventually proved fatal.

Armand Trousseau(1801-1867)

Case: Identify the abnormality. Give your differentials.

Marcos Duarte Siosaki, M.D., and Ana Tarsila Souza, M.D.N Engl J Med 2013; 368:e7February 7, 2013DOI: 10.1056/NEJMicm1204740

Case: Identify the abnormality. Give your differentials.

Marcos Duarte Siosaki, M.D., and Ana Tarsila Souza, M.D.N Engl J Med 2013; 368:e7February 7, 2013DOI: 10.1056/NEJMicm1204740

Virchow's node or

Troisier's node

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Dr.Troisier The French pathologist Charles Emile Troisier noted in 1889 that other abdominal cancers, too,

could spread to the Virchow’s node

Cf

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Tumors of blood vessels

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Hemangiomas

• Very common tumors• Increased number of blood vessels filled with

blood• Many of the capillary lesions seen in infancy

may regress spontaneously as the child grows (by the age of 7years)

• Some hemangiomas involve large portions of the body – Angiomatosis

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Capilalry Hemangioma

• Sites: Skin, subcutis, oral cavity, liver, spleen, kidney• Strawberry angioma – new bornsMorphology:• Bright red to blue• Closely packed thin walled blood vessels• Lined by flat endothelium• Scant connective tissue• Hemorrhages, thrombosis, hemosiderin laden MØ

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Capillary angioma

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Capillary angioma - Strawberry angioma

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Capillary Angioma

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Cavernous angioma

• Large dilated vascular channels• Less well circumscribed• Frequently involve deep structures• May be a part of VHL, Muffucci syndromesMorphology:• Red blue soft spongy masses• Large intercommunicating vascular channels• Modest connective tissue stroma• Thrombosis and calcification

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Cavernous angioma

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Muffucci syndromeCavernous angiomas + Enchondromas

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Cavernous angioma

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Granuloma pyogenicum(Pregnancy tumor)

• A form of capillary angioma• Rapidly growing• Pedunculated red nodule• Skin, gingiva, oral mucosa• “Bleeds on touch”• Capillaries arranged in lobular fashion• Finger like epidermal projections at the

margins

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Granuloma pyogenicum(Pregnancy tumor)

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Granuloma pyogenicum

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Granuloma pyogenicum(Pregnancy tumor)

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Hemangiomas - gist

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Bacillary AngiomatosisBacillary angiomatosis is a vascular proliferation in immunocompromised

hostsCaused by:• Bartonella henselae• Bartonella quintana

Morphology:• Skin lesions are red papules and nodules, or rounded subcutaneous masses• Histologically:

– Capillary proliferation with prominent epithelioid endothelial cells – Nuclear atypia and mitoses– Stromal neutrophils, nuclear dust, and the causal bacteria

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Bacillary Angiomatosis

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Kaposi sarcoma (KS)

Forms of KS:• Chronic KS (Classic)• Lymphadenopathic KS• Transplant associated KS• AIDS associated KSPathogenesis:• HHV8 (KSHV)• Latent infection in endothelial cells > [p53 inhibitors +

Cyclin-D equivalent + viral G protein which induces VEGF] > Cellular proliferation

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Kaposi sarcoma (KS)Morphology:• Patches / plaques / nodules• Red / purple / violaceous• Dermal accumulations of spindle cells with slit like

spaces with extravasated RBCs• Hemosiderin laden macrophages• Mitotic figures are common in nodular form

Nodular forms often heralds LN and visceral involvementIFN-α anti-VEGF are effective

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Kaposi sarcoma

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Kaposi sarcoma – Nodular form

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Kaposi sarcoma

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Kaposi sarcoma

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Kaposi sarcoma

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Angiosarcoma• Malignant endothelial neoplasm • Primarily affects older adults• Tumor may occur at any site

– Skin, soft tissue, breast, and liver• Liver: Thorostrat, Vinyl chloride

Morphology:• Skin: Asymptomatic red papules or nodules• Plump, atypical endothelial cells forming vascular Channels • Wildly undifferentiated tumors with a solid spindled appearance and

no discernible blood vessels• IHC: CD31 or von Willebrand factor

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Angiosarcoma

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Angiosarcoma

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Angiosarcoma

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Epithelioid angiosarcoma

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E N D

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