[Familial lymphohistiocytosis. Evolution of management apropos of 3 cases]. / La lymphohistiocytose familiale. Evolution de la prise en charge à propos de trois cas.
J Med Liban
; 48(2): 100-3, 2000.
Article
en Fr
| MEDLINE
| ID: mdl-11028159
Familial lymphohistiocytosis is a rare rapidly lethal genetic disease. It is characterized by an uncontrolled activation of T lymphocytes and macrophages, with multiple organ infiltration, beginning with fever and unexplained coagulopathy. Recently, one of the genes implicated in 50% of families at risk was identified (locus FHL1, chromosome 10, region q21-22). Based on data suggesting an essential role of T lymphocytes in the genesis of familial lymphohistiocytosis, the treatment has recently evolved from a chemotherapy including Etoposide (VP16) and corticosteroids, sometimes efficient but toxic, to an almost always efficient and slightly toxic immunosuppressive treatment. These two treatments achieved a remission somewhat lasting with no definite cure. In fact, all patients relapsed in the central nervous system and died. Bone marrow transplantation (BMT) is the only curative treatment. However only 20% of patients benefit from an HLA identical BMT. Recent improvements in HLA non-identical BMT offer an acceptable alternative to the other 80% of patients. In this review, we present three cases illustrating the evolution and optimization in the management of infants with familial lymphohistiocytosis.
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Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Cromosomas Humanos Par 10
/
Histiocitosis de Células no Langerhans
/
Proteínas de Homeodominio
Tipo de estudio:
Diagnostic_studies
/
Prognostic_studies
Límite:
Female
/
Humans
/
Infant
Idioma:
Fr
Revista:
J Med Liban
Año:
2000
Tipo del documento:
Article
País de afiliación:
Líbano
Pais de publicación:
Líbano