ABSTRACT
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
Subject(s)
Humans , Female , Histiocytosis/drug therapyABSTRACT
Las histocitosis infantiles son un grupo de enfermedades diversas y poco frecuentes, de etiología desconocida y de difícil diagnóstico clínico. Los estudios anatomopatológicos han permitido su clasificación y enfoque terapéutico. En casi todos los procesos que agrupa la histiocitosis, la boca puede presentar lesiones con una frecuencia de hasta el 70 por ciento, afecta los tejidos duros y blandos y a veces es el odontólogo el primero en observar al paciente. El objetivo de esta comunicación es realizar el diagnóstico diferencial de lesiones de histiocitosis, analizar su evolución con y sin tratamiento y orientar sobre su pronóstico. Sobre 13 niños de 1 a 14 años se siguió la evolución de las lesiones, se realizó el diagnóstico histopatológico y el tratamiento dentro de un equipo multidisciplinario
Subject(s)
Humans , Male , Female , Infant , Child, Preschool , Adolescent , Dental Care for Children/methods , Diagnosis, Differential , Histiocytosis, Langerhans-Cell/diagnosis , Histiocytosis, Non-Langerhans-Cell/diagnosis , Histiocytic Sarcoma/diagnosis , Histiocytosis/classification , Histiocytosis/diagnosis , Histiocytosis/therapy , Gingival Diseases/etiology , Eosinophilic Granuloma/diagnosis , Histiocytosis, Langerhans-Cell/diagnosis , Histiocytosis/drug therapy , Histiocytosis/radiotherapy , Mouth Diseases/etiology , Patient Care Team , Periodontal Diseases/etiology , Recurrence/prevention & controlABSTRACT
This is a report of a case, 7 1/2 year-old-boy having chronic febrile and recurrent crops of painful subcutaneous nodules on lower extremities, which had previously been diagnosed as Weber-Christian disease, which progressed to have cytophagic histiocytic activity in the skin, bone marrow with abnormal liver function and hemorrhagic diathesis. He was subsequently treated with corticosteroid without good response. After he was diagnosed as having cytophagic histiocytic panniculitis, cyclosporin A was administered intravenously in an initial dosage of 1 mg/kg/day and in oral maintenance dose of 10 mg/kg/day with a successful response and the patient completely recovered within 6 months with mild hypertension as an adverse effect.