dra. monique kowalski schmitz dr. levi h. jales neto dr. fernando h. c. souza dr. diogo s. domiciano...
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Dra. Monique Kowalski Schmitz Dra. Monique Kowalski Schmitz Dr. Levi H. Jales NetoDr. Levi H. Jales Neto
Dr. Fernando H. C. SouzaDr. Fernando H. C. Souza
Dr. Diogo S. DomicianoDr. Diogo S. DomicianoDr. Samuel K. ShinjoDr. Samuel K. Shinjo
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SÍNDROME HEMOFAGOCÍTICA
Macrofágos
Histiócitos
Eritrócitos Leucócitos Plaquetas Precursores
HEMOFAGOCITOSE
LES
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SÍNDROME HEMOFAGOCÍTICA
Relativamente freqüentes LES Doença de Still do adulto AIJ Doença de Castleman
Raras ou excepcionais Doença de Kikuschi Eclerose sistêmica Artrite reumatóide Dermatomiosite DMTC Sd. Sjögren Doença de Kawasaki Poliarterite nodosa Sarcoidose
Larroche & Mouthon, Autoimmun Rev, 2004Tristano, Med Sci Monit, 2008
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AINHs
Metotrexato
Sulfassalazina
Sais de ouro
Anti-TNF (etarnecept, infliximabe)
Tristano, Med Sci Monit, 2008
SÍNDROME HEMOFAGOCÍTICA
Drogas
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LES
Infecção Medicação
Triggers
Células T ativadas
Complexo imune mediado Auto-Ac
Histiócito
Ativação de monócitose macrófagos
Sensibilização e fagocitose
IL1, IL2IL6, IL8
IL18, IFN M-CSF
(+)
Tristano, Med Sci Monit, 2008Reis, Acta Reumatol Port, 2008
Hagiwara et al, Mod Rheumatol, 2006
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Larroche & Mouthon, Autoimmun Rev, 2004
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CRITÉRIOS
Critérios clínicos e laboratoriaisCritérios clínicos e laboratoriais FebreFebre (>7dias / 38,5 C)(>7dias / 38,5 C) EsplenomegaliaEsplenomegalia CitopeniasCitopenias ≥≥ 2 linhagens 2 linhagens
Hb Hb <9 g/dL<9 g/dL
Neutrófilos Neutrófilos < 1000 cels/dL< 1000 cels/dL
Plaquetas Plaquetas <100 mil cels/dL<100 mil cels/dL
FerritinaFerritina ≥≥3 DP3 DP ouou DHLDHL ≥≥3 DP3 DP
Critério histopatológicoCritério histopatológico HemofagocitoseHemofagocitose ( MO, líquor ou linfonodos)( MO, líquor ou linfonodos)
Imashuku , Int J Hematol, 1997Imashuku , Int J Hematol, 1997
1. Imashuku1. Imashuku
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CRITÉRIOS
CitopeniasCitopenias ≥ ≥2 de 3 linhagens, 2 de 3 linhagens, não causada por MO aplásica ou displásicanão causada por MO aplásica ou displásica
Histiocitose hemofagocíticaHistiocitose hemofagocítica (MO ou outros sistema reticulo-endoteliais)(MO ou outros sistema reticulo-endoteliais)
Fase aguda de doença auto-imuneFase aguda de doença auto-imune
Exclusão de outras causas Exclusão de outras causas (vírus ou neoplasias)(vírus ou neoplasias)
2. Kumakura
Kumakura, Intern Med, 2005
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TRATAMENTO
Corticosteróides IVIG Ciclosporina A Plasmaférese Ciclofosfamida Rituximabe Etoposide (EBV) Anti-TNF*
Tristano, Med Sci Monit, 2008
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Revisão sistêmica de casos publicados - MEDLINE database
9 pacientes
< 18 anos
Screening para infecções ( - )
A
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Febre Todos
Linfadenopatia 5/8 *
Alteração do SNC 3/8 *
Esplenomegalia 2/8 *
Hepatomegalia 2/8 *
Complicação hemorrágica 1/8 *
Acometimento cardíaco, pulmonar e/ou renal 4/9
* NAPringe et al, Lupus, 2007
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FAN 9/9
Anti-DNA 7/9
C3 7/9
Pringe et al, Lupus, 2007
MO 9/9Hemofagocitose
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Todos: Corticoesteróides
Outros:
2 Óbitos
Nenhuma recidivaPringe et al, Lupus, 2007
Imunoglobulina Ciclosporina CFF Etoposide Plasmaférese
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SD HEMOFAGOCÍTICA
LES Juvenil AIJ Linfadenopatia
Falência (múltiplos órgãos) ↓Absoluta (Leuco e Plaq)
Hepatomegalia
↓Relativa (Leuco e Plaq)
Pringe et al, Lupus, 2007
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Ferritina > 10.000 ug/LFase aguda da síndrome
Indicador de melhor prognóstico
FERRITINA
Pringe et al. Lupus, 2007
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Mulher, 28a, LES há 2 anos Flare da doença Febre, rash, úlceras orais, bicitopenia, hiperferritinemia, ↑transaminases MO: hemofagocitose Sorologias ( - )
Gamaglobulina (400mg/kg/d) 5 dias
Metilprednisolona(1g) 3 dias
CFF(1g)
B
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Hemofagocitose no sangue periférico
Biópsia invasiva
Risco PlaquetopeniaCoagulopatiaCIVD
C
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1º caso sucessoLES há 6 anosRefratária
MetilprednisolonaPlasmaféreseVincristinaEtoposide
Infliximabe5mg/kg/dia, 2x
SHF
D
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Quin & Yang, Clin Rheumatol, 2007
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Tristano, Med Sci Monit, 2008
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FebreHepatoesplenomegaliaPancitopenia↑Ferritina e DHLHemofagocitose
Sd hemofagocítica
?Infecções ( - )Neoplasias ( - )Medicamento ( - )Reumatológica
CASO CLÍNICO
Metilprednisolona
IVIG
Ciclofosfamida FAN, Anti-Sm, Anti-DNA Serosite, Renal, Hemorragia alveolar
LES
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