Kaposi's Sarcoma

Kaposi's Sarcoma Human Herpes Virus 8 Dr.T.V.Rao MD

Moritz Kaposi (1837 - 1902), ●

Moritz Kaposi (Hungarian: Kaposi Mór) (b. 23 October 1837 in Kaposvár, Hungary; d. 6 March 1902 in Vienna, Austria) was an important Hungarian physician and dermatologist, discoverer of the skin tumor that received his name.

Moritz Kaposi, 1872 identifies as incurable condition The disease leads to death, and it does so within a short period of two to three years...The disease must, from our present experience, be considered from the onset not only as incurable but also as deadly.“ Moritz Kaposi ●

HIV infection Brings Kaposi’s Sarcoma in prominence ●

Kaposi's sarcoma (KS) occurs more often in men than in women and HIV-1associated KS has a high occurrence in homosexual men (over 30%). Most cultures of KS tumours yield cells with properties of hyperplasic (not malignant) endothelial cells under the control of several cytokines.

Identified as AIDS defining illness ●

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It became more widely known as one of the AIDS defining illnesses in the 1980s. The viral cause for this cancer was discovered in 1994. Identfied as latest Human tumour virus

Virology - KSV ●

KSHV is a herpes virus, and is a large doublestranded DNA virus with a protein covering that packages its nucleic acids, called the capsid, which is then surrounded by an amorphous protein layer called the tegument , and finally enclosed in a lipid envelope derived in part from the cell membrane.

Virology ●

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KSHV is a lymph tropic and more closely related to EBV and herpes virus saimiri. The KSHV genome (165 kbp) contain numerous genes related to cellular regulatory genes involved in cell proliferation, apoptosis, and host response contributing to pathogenesis.

Secretions transmit Infection ●

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KSHV appears to be shed in saliva independent of subjects immune status Viral DNA is detected in breast milk samples in African patients. HSHV 8 infects dividing B cells CD45+phase

Dr.T.V.Rao MD

Routes of Transmission ●

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Sexaully transmitted among men who have sex with men. Who have higher prevalence ( 3060%) Infections are common in Africa (>50%) with infections acquired early in life by nonsexual routes, through contact with oral secretions

KSHV in organ recipients ●

The virus are transmitted through organ transplantation, and places the recipients at risk of KSHV related diseases.

Prominent manifestations with KSHV infections ● ●

Causes Kaposi’s sarcoma Vascular tumors of mixed cellular composition and is involved in pathogenesis of body cavity based lymphomas occuring in AIDS patients and of multicentric Castleman”s disease.

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Types of Kaposi’s lesions Kaposi’s tumours are divided into three groups, based on appearance, with much overlap. 1 Nodular lesions are of varying size and thickness. They are purple and will at times have a halo of brown or yellow pigment around them. 2 Infiltrating lesions may be quite large, may be raised, or grow downward beneath the skin. 3 Lymphatic lesions can mimic other causes of swollen lymph nodes and may require a biopsy to rule out infection. ●

Kaposi’s sarcoma present with varied lesions ● ● ● ● ● ● ● ● ● ● ●

Localised nodular Locally aggressive Generalised lymphadenopathy Patch stage Localised plaques Infiltrative plaques Disseminated cutaneous and visceral disease Telangiectatic Keloidal Ecchymotic Lymphangioma-like / cavernous disease

Purplish lesions of Kaposi's sarcoma. Credit: NIH

Typical Lesions Of Kaposi's Sarcoma

KSHV infection involving Oral cavity

KSHV infection presenting as Dermal lesions

Clinically Important Disease ●

A relatively rare type of skin malignancy that tends to afflict elderly people or, especially, those with an abnormal immune system as in AIDS. Kaposi sarcoma is a highly vascular ("angioblastic") tumor of the skin characterized by soft purplish plaques and papules that form nodules which typically start on the feet and ankles and then slowly spread across the skin of the legs, hands and arms. In AIDS patients, these tumours can also develop internally and cause severe internal bleeding

Epidemic (AIDS-related) Kaposi sarcoma ●

The most common type of Kaposi sarcoma (KS) in the United States is epidemic or AIDS-related KS. This type of KS develops in people who are infected with HIV, the virus that causes AIDS. A person infected with HIV (that is, who is HIVpositive) does not necessarily have AIDS. The virus can be present in the body for a long time, typically many years, before causing major illness. The disease known as AIDS begins when the virus has seriously damaged the immune system,

Classic (Mediterranean) Kaposi sarcoma ●

Classic Kaposi sarcoma (KS) occurs in elderly people of Mediterranean, Eastern European, and Middle Eastern heritage. Classic KS is more common in men than in women. Patients typically have one or more lesions on the legs, ankles, or the soles of the feet. In comparison with other types of KS, the lesions in this type do not grow as quickly, and new lesions do not develop as often.

Endemic (African) Kaposi sarcoma ●

Endemic Kaposi sarcoma (KS) occurs in people living in Equatorial Africa and is sometimes called African KS. KS herpes virus infection is much more common in Africa than in other parts of the world, increasing the chance of KS developing.

Iatrogenic (transplant associated) Kaposi sarcoma ●

When Kaposi sarcoma (KS) develops in people whose immune systems have been suppressed after an organ transplant it is called iatrogenic, or transplant-associated, KS. Most transplant patients take drugs to keep the immune system from rejecting (attacking and killing) the new organ.

Frequency of Kaposi's sarcoma as reported to CDC ● ●

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Homosexual men — 20 to 30 percent Heterosexual injection drug users (IDU) — 3 percent Transfusion recipients — 3 percent Women or children — 3 percent Haemophiliacs —1 percent

Diagnosis Viral DNA can be detected in patient specimens using PCR assays. ● Viral culture is impractical ● Serology Antibodies to KSHV Indirect Immunofluorescence Western Blot ELISA methods ●

Epidemiology ● ● ●

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5 variants A – E B type predominantly in Africa Rates of seroconversion are higher risk groups for Kaposi’s sarcoma 30% of the Gay men are seropositive

Treatment ●

Several drugs were in trail or use Foscarnet. Gancilovir, Cidofovir With use of Above drugs KSHV replication is reduced.

Antiretroviral drugs in Kaposi’s Sarcoma ●

The rate of new Kaposi's sarcoma is markedly reduced in HIV- positive patients on effective Antiretroviral therapy

Created for Medical and Paramedical students in Developing World Dr.T.V.Rao MD Email [email protected]