Faizana Amod SIDA E SARCOMA DE KAPOSI EM CRIANÇAS EM ÀFRICA.

Faizana AmodFaizana Amod

SIDA E SARCOMA DE KAPOSI EM CRIANÇAS

EM ÀFRICA

SARCOMA DE KAPOSI SARCOMA DE KAPOSI (SK)(SK)

Resultado da expressão não controlada do Herpesvirus Humano-8(HHV8) nas células endoteliais, normalmente secundário a imunodepressão

Kaposi Sarcoma: The African HIV Epidemic’s Partner in Crime, by Alan Davidson, FCPaed, MPhi, Kaposi Sarcoma: The African HIV Epidemic’s Partner in Crime, by Alan Davidson, FCPaed, MPhi, Pediatr Blood Cancer 2010;54:657–658lPediatr Blood Cancer 2010;54:657–658lKaposi Sarcoma: The African HIV Epidemic’s Partner in Crime, by Alan Davidson, FCPaed, MPhi, Kaposi Sarcoma: The African HIV Epidemic’s Partner in Crime, by Alan Davidson, FCPaed, MPhi, Pediatr Blood Cancer 2010;54:657–658lPediatr Blood Cancer 2010;54:657–658l

SARCOMA DE KAPOSISARCOMA DE KAPOSI

O SK é endémico na África Sub-Sahariana onde a seroprevalência do HHV8 ~ 20-70%

A prevalência do HHV8 em África subiu dramaticamente (30-50x) com a epidemia do HIV

Kaposi Sarcoma–Associated Herpesvirus (KSHV),Seroprevalence in Population-Based Samples of African Children:Evidence for At Least 2 Patterns, of KSHV Transmission

Kaposi Sarcoma–Associated Herpesvirus (KSHV),Seroprevalence in Population-Based Samples of African Children:Evidence for At Least 2 Patterns, of KSHV Transmission

VAZ et al,VAZ et al, Treatment of Kaposi’s sarcoma in HIV-1-infected Mozambican Children with Treatment of Kaposi’s sarcoma in HIV-1-infected Mozambican Children with Antiretroviral Drugs and ChemotherapyAntiretroviral Drugs and Chemotherapy; ; PIDJ, in pressPIDJ, in pressVAZ et al,VAZ et al, Treatment of Kaposi’s sarcoma in HIV-1-infected Mozambican Children with Treatment of Kaposi’s sarcoma in HIV-1-infected Mozambican Children with Antiretroviral Drugs and ChemotherapyAntiretroviral Drugs and Chemotherapy; ; PIDJ, in pressPIDJ, in press

SARCOMA DE KAPOSI-SARCOMA DE KAPOSI-HHV8HHV8

Em Moçambique Prevalência do HHV8 51.1 % Em Moçambique Prevalência do HHV8 51.1 %Ceffa. S et al,Seroprevalence of HHV8 in a cohort of HIV-negative and HIV-positive patients in Mozambique. 2007 Nov-Dec;19(6):519-23. Ceffa. S et al,Seroprevalence of HHV8 in a cohort of HIV-negative and HIV-positive patients in Mozambique. 2007 Nov-Dec;19(6):519-23.

Prevalencia HHV8-Prevalencia HHV8-CriancasCriancas

A. Sul – 7.5-9% 2anos

Uganda -10%2anos -30% 8 anos

Lisa M. Et al,Kaposi Sarcoma–Associated Herpesvirus (KSHV),Seroprevalence in Population-Based Samples of African Children:Evidence for At Least 2 Patterns, of KSHV Transmission

Lisa M. Et al,Kaposi Sarcoma–Associated Herpesvirus (KSHV),Seroprevalence in Population-Based Samples of African Children:Evidence for At Least 2 Patterns, of KSHV Transmission

SARCOMA DE KAPOSISARCOMA DE KAPOSI

50%

13%9%

6%3%

19%

BURKIT RETINO KAPOSI WILMS LEUCEMIA OUTROS

Sinfield et al.Spectrum and Presentation of Sinfield et al.Spectrum and Presentation of Pediatric Malignancies in the HIV Era: Pediatric Malignancies in the HIV Era: Experience From Blantyre, Malawi, 1998–Experience From Blantyre, Malawi, 1998–2003, Pediatr Blood Cancer 2007;48:515–5202003, Pediatr Blood Cancer 2007;48:515–520

Sinfield et al.Spectrum and Presentation of Sinfield et al.Spectrum and Presentation of Pediatric Malignancies in the HIV Era: Pediatric Malignancies in the HIV Era: Experience From Blantyre, Malawi, 1998–Experience From Blantyre, Malawi, 1998–2003, Pediatr Blood Cancer 2007;48:515–5202003, Pediatr Blood Cancer 2007;48:515–520

Num

ber

of

Case

s

C. Carrilho,Serviço de Anatomia Patológica –HCM Janeiro 2005- Dezembro 2006C. Carrilho,Serviço de Anatomia Patológica –HCM Janeiro 2005- Dezembro 2006

1998-20031998-2003 2005-20062005-2006

SARCOMA DE KAPOSISARCOMA DE KAPOSI

5159

4841

66

86

24

8

4

24

19

0

10

20

30

40

50

60

70

80

90

100

1998 1999 2000 2001 2002 2003

0

5

10

15

20

25

30

L.BURKIT S.KAPOSI

Sinfield et al.Spectrum and Presentation of Pediatric Malignancies in the, HIV Era: Experience From Sinfield et al.Spectrum and Presentation of Pediatric Malignancies in the, HIV Era: Experience From Blantyre, Malawi, 1998–2003, Pediatr Blood Cancer 2007;48:515–520Blantyre, Malawi, 1998–2003, Pediatr Blood Cancer 2007;48:515–520Sinfield et al.Spectrum and Presentation of Pediatric Malignancies in the, HIV Era: Experience From Sinfield et al.Spectrum and Presentation of Pediatric Malignancies in the, HIV Era: Experience From Blantyre, Malawi, 1998–2003, Pediatr Blood Cancer 2007;48:515–520Blantyre, Malawi, 1998–2003, Pediatr Blood Cancer 2007;48:515–520

SARCOMA DE KAPOSISARCOMA DE KAPOSI

Incidência de SK aumentou 10x mais em países como Malawi, Uganda e na África Sub- Sahariana em comparação com modesto aumento do L. Burkitt

SK é uma das principais neoplasias na idade Pediátrica

Kaposi Sarcoma: The African HIV Epidemic’s Partner in Crime, by Alan Davidson, FCPaed, MPhi, Kaposi Sarcoma: The African HIV Epidemic’s Partner in Crime, by Alan Davidson, FCPaed, MPhi, Pediatr Blood Cancer 2010;54:657–658lPediatr Blood Cancer 2010;54:657–658lKaposi Sarcoma: The African HIV Epidemic’s Partner in Crime, by Alan Davidson, FCPaed, MPhi, Kaposi Sarcoma: The African HIV Epidemic’s Partner in Crime, by Alan Davidson, FCPaed, MPhi, Pediatr Blood Cancer 2010;54:657–658lPediatr Blood Cancer 2010;54:657–658l

SK-HOSPITAL CENTRAL SK-HOSPITAL CENTRAL DE MAPUTO (HCM)DE MAPUTO (HCM)

32 crianças estudadas ( SIDA + SK )

Dec. 2003- Jan,2008

2-16 anos de idade (média de 8.3 anos)

♂ (2.4:1)VAZ et al,VAZ et al, Treatment of Kaposi’s sarcoma in HIV-1-infected Mozambican Children with Treatment of Kaposi’s sarcoma in HIV-1-infected Mozambican Children with Antiretroviral Drugs and ChemotherapyAntiretroviral Drugs and Chemotherapy; ; PIDJ, in pressPIDJ, in pressVAZ et al,VAZ et al, Treatment of Kaposi’s sarcoma in HIV-1-infected Mozambican Children with Treatment of Kaposi’s sarcoma in HIV-1-infected Mozambican Children with Antiretroviral Drugs and ChemotherapyAntiretroviral Drugs and Chemotherapy; ; PIDJ, in pressPIDJ, in press

SK-FORMAS DE SK-FORMAS DE APRESENTAÇÃO-HCMAPRESENTAÇÃO-HCM

VAZ et al,VAZ et al, Treatment of Kaposi’s sarcoma in HIV-1-infected Mozambican Children with Treatment of Kaposi’s sarcoma in HIV-1-infected Mozambican Children with Antiretroviral Drugs and ChemotherapyAntiretroviral Drugs and Chemotherapy; ; PIDJ, in pressPIDJ, in pressVAZ et al,VAZ et al, Treatment of Kaposi’s sarcoma in HIV-1-infected Mozambican Children with Treatment of Kaposi’s sarcoma in HIV-1-infected Mozambican Children with Antiretroviral Drugs and ChemotherapyAntiretroviral Drugs and Chemotherapy; ; PIDJ, in pressPIDJ, in press

18%

36%46%

4%

0%

5%

10%

15%

20%

25%

30%

35%

40%

45%

50%

LN PELE MISTO VISCERAL

SK -TRATAMENTO - HCMSK -TRATAMENTO - HCM

VAZ et al,VAZ et al, Treatment of Kaposi’s sarcoma in HIV-1-infected Mozambican Children with Treatment of Kaposi’s sarcoma in HIV-1-infected Mozambican Children with Antiretroviral Drugs and ChemotherapyAntiretroviral Drugs and Chemotherapy; ; PIDJ, in pressPIDJ, in pressVAZ et al,VAZ et al, Treatment of Kaposi’s sarcoma in HIV-1-infected Mozambican Children with Treatment of Kaposi’s sarcoma in HIV-1-infected Mozambican Children with Antiretroviral Drugs and ChemotherapyAntiretroviral Drugs and Chemotherapy; ; PIDJ, in pressPIDJ, in press

SK-UGANDASK-UGANDA

73 crianças estudados (SIDA+SK) Out. 2004- Jun. 2007

2-18 anos Idade media - 10.1 anos

♂ (1.04:1)

Gantt et al.,Clinical Presentation and Outcome of Epidemic Kaposi Sarcoma iUgandan Children, Gantt et al.,Clinical Presentation and Outcome of Epidemic Kaposi Sarcoma iUgandan Children, Pediatr Blood Cancer 2010;54:670–674Pediatr Blood Cancer 2010;54:670–674Gantt et al.,Clinical Presentation and Outcome of Epidemic Kaposi Sarcoma iUgandan Children, Gantt et al.,Clinical Presentation and Outcome of Epidemic Kaposi Sarcoma iUgandan Children, Pediatr Blood Cancer 2010;54:670–674Pediatr Blood Cancer 2010;54:670–674

SK-FORMAS DE SK-FORMAS DE APRESENTAÇÃO-UGANDAAPRESENTAÇÃO-UGANDA

60% 48%

21%12%

LN PELE C.ORAL VISCERAL

Gantt et al.,Clinical Presentation and Outcome of Epidemic Kaposi Sarcoma iUgandan Children, Pediatr Gantt et al.,Clinical Presentation and Outcome of Epidemic Kaposi Sarcoma iUgandan Children, Pediatr Blood Cancer 2010;54:670–674Blood Cancer 2010;54:670–674Gantt et al.,Clinical Presentation and Outcome of Epidemic Kaposi Sarcoma iUgandan Children, Pediatr Gantt et al.,Clinical Presentation and Outcome of Epidemic Kaposi Sarcoma iUgandan Children, Pediatr Blood Cancer 2010;54:670–674Blood Cancer 2010;54:670–674

LN 6.5 anos

Pele 11.5 anos

Visceral e cavidade oral sem relação com a idade

SK-FORMAS DE SK-FORMAS DE APRESENTAÇÃO-UGANDAAPRESENTAÇÃO-UGANDA

Gantt et al.,Clinical Presentation and Outcome of Epidemic Kaposi Sarcoma iUgandan Children, Pediatr Gantt et al.,Clinical Presentation and Outcome of Epidemic Kaposi Sarcoma iUgandan Children, Pediatr Blood Cancer 2010;54:670–674Blood Cancer 2010;54:670–674Gantt et al.,Clinical Presentation and Outcome of Epidemic Kaposi Sarcoma iUgandan Children, Pediatr Gantt et al.,Clinical Presentation and Outcome of Epidemic Kaposi Sarcoma iUgandan Children, Pediatr Blood Cancer 2010;54:670–674Blood Cancer 2010;54:670–674

SK -TRATAMENTO SK -TRATAMENTO UGANDAUGANDA

QT SEM TARV 10

QT COM TARV 26TARV 15SEM

TRATAMENTO22

TOTAL 73

Gantt et al.,Clinical Presentation and Outcome of Epidemic Kaposi Sarcoma iUgandan Children, Pediatr Gantt et al.,Clinical Presentation and Outcome of Epidemic Kaposi Sarcoma iUgandan Children, Pediatr Blood Cancer 2010;54:670–674Blood Cancer 2010;54:670–674Gantt et al.,Clinical Presentation and Outcome of Epidemic Kaposi Sarcoma iUgandan Children, Pediatr Gantt et al.,Clinical Presentation and Outcome of Epidemic Kaposi Sarcoma iUgandan Children, Pediatr Blood Cancer 2010;54:670–674Blood Cancer 2010;54:670–674

COMPLT.

PARCIAL

S/ RESP

DESCON.

QT S/TARV

1 5 0 4

QT+TARV

17 6 1 2

TARV 2 0 0 13

TOTAL 20 11 1 41

SK -TRATAMENTO SK -TRATAMENTO UGANDAUGANDA

Gantt et al.,Clinical Presentation and Outcome of Epidemic Kaposi Sarcoma iUgandan Children, Pediatr Gantt et al.,Clinical Presentation and Outcome of Epidemic Kaposi Sarcoma iUgandan Children, Pediatr Blood Cancer 2010;54:670–674Blood Cancer 2010;54:670–674Gantt et al.,Clinical Presentation and Outcome of Epidemic Kaposi Sarcoma iUgandan Children, Pediatr Gantt et al.,Clinical Presentation and Outcome of Epidemic Kaposi Sarcoma iUgandan Children, Pediatr Blood Cancer 2010;54:670–674Blood Cancer 2010;54:670–674

SK –ÁFRICA DO SULSK –ÁFRICA DO SUL

70 crianças estudadas (SK+SIDA) Jan. 1998- Dec. 2009

0-14 anosIdade média ao diagnóstico 6 anos

♂ (1.59:1)

Stefan et al.,Kaposi Sarcoma in South African Children, Pediatr Blood Cancer-2010Stefan et al.,Kaposi Sarcoma in South African Children, Pediatr Blood Cancer-2010

SK-FORMAS DE APRESENTAÇÃO-ÁFRICA DO SUL

30%

57%

25%

38%

10%

LN PELE C.ORAL VISCERAL S/INF.

Stefan et al.,Kaposi Sarcoma in South African Children, Pediatr Blood Cancer-2010Stefan et al.,Kaposi Sarcoma in South African Children, Pediatr Blood Cancer-2010Stefan et al.,Kaposi Sarcoma in South African Children, Pediatr Blood Cancer-2010Stefan et al.,Kaposi Sarcoma in South African Children, Pediatr Blood Cancer-2010

SK-TRATAMENTOÁFRICA DO SUL

TARV

TARVAntes Diagnostico

SK

14

TARVApós Diagnóstico

SK

35

SEM TARV 16

S/Informação 5

QTERAPIAQT 52

SEM QT 8

S/Informação 10

Stefan et al.,Kaposi Sarcoma in South African Children, Pediatr Blood Cancer-2010Stefan et al.,Kaposi Sarcoma in South African Children, Pediatr Blood Cancer-2010Stefan et al.,Kaposi Sarcoma in South African Children, Pediatr Blood Cancer-2010Stefan et al.,Kaposi Sarcoma in South African Children, Pediatr Blood Cancer-2010

SK- TRATAMENTOSK- TRATAMENTOÁFRICA DO SULÁFRICA DO SUL

SOBREVIVÊNCIA ÓBITOQT+TAR

V28 (40%) 17

QT S/ TARV

0 15

TOTAL 28 32

Stefan et al.,Kaposi Sarcoma in South African Children, Pediatr Blood Cancer-2010Stefan et al.,Kaposi Sarcoma in South African Children, Pediatr Blood Cancer-2010Stefan et al.,Kaposi Sarcoma in South African Children, Pediatr Blood Cancer-2010Stefan et al.,Kaposi Sarcoma in South African Children, Pediatr Blood Cancer-2010

DISCUSSÃO

Idade

Sexo mais frequente - ♂

DISCUSSÃODISCUSSÃOFORMAS DE FORMAS DE APRESENTAÇÃOAPRESENTAÇÃO Estudo de A. Sul e HCM forma

cutânea mais frequente

A forma visceral 2º mais frequente na A. Sul

DISCUSSÃO DISCUSSÃO RESPOSTA AO RESPOSTA AO TRATAMENTOTRATAMENTO

Sobrevivência melhor em pacientes que fizeram QT e TARV

EPIDEMIOLOGIA/EPIDEMIOLOGIA/PATOGENIAPATOGENIA

Evolução do SK na era do TARV

Estudos moleculares para avaliar os mecanismos de aparecimento da doença

Sindrome de reconstituição imunitária (SRIS) no SK

TRATAMENTO E TRATAMENTO E RESPOSTARESPOSTA

Ensaios clínicos para estabelecer cuidados padronizados

Qual o melhor regime TARV? QT e TARV ? Qual QT ? Que

sequencia ? Melhor tratamento de acordo com

as diferentes apresentações

OBRIGADA