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Prolonged Remission Following Autologous Peripheral Blood Stem Cell Transplantation in an Infant with Familial Hemophagocytic Lymphohistiocytosis / 대한혈액학회지
Korean Journal of Hematology ; : 346-350, 2001.
Artículo en Coreano | WPRIM | ID: wpr-720370
ABSTRACT
Familial hemophagocytic lymphohistiocytosis (FHL) is a fatal illness with a median life expectancy of 2 months if not treated. However, with the recent employment of allogeneic bone marrow transplantation in the treatment of FHL, even complete cure might be anticipated. We report here a case of prolonged remission following autologous peripheral blood stem cell transplantation (Auto-PBSCT) which was first attempted for FHL in Korea. A 4-month-old girl presented with high fever and a huge hepatosplenomegaly. Bone marrow examination revealed the proliferation of both non-malignant histiocytes engulfing red blood cells and lymphocytes. Her brother died at the age of 18 months showing quite similar manifestations. A diagnosis of FHL was made. Following 8 courses of chemotherapy with multiple drugs, Auto-PBSCT was performed. She has been well without any evidence of disease over 8 months of post- transplant period. This case illustrates that it might be worth trying Auto- PBSCT in the treatment of FHL.
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Texto completo: Disponible Índice: WPRIM (Pacífico Occidental) Asunto principal: Examen de la Médula Ósea / Linfocitos / Esperanza de Vida / Trasplante de Médula Ósea / Trasplante de Células Madre de Sangre Periférica / Hermanos / Diagnóstico / Quimioterapia / Empleo / Eritrocitos Tipo de estudio: Estudio diagnóstico Límite: Femenino / Humanos / Lactante País/Región como asunto: Asia Idioma: Coreano Revista: Korean Journal of Hematology Año: 2001 Tipo del documento: Artículo

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Texto completo: Disponible Índice: WPRIM (Pacífico Occidental) Asunto principal: Examen de la Médula Ósea / Linfocitos / Esperanza de Vida / Trasplante de Médula Ósea / Trasplante de Células Madre de Sangre Periférica / Hermanos / Diagnóstico / Quimioterapia / Empleo / Eritrocitos Tipo de estudio: Estudio diagnóstico Límite: Femenino / Humanos / Lactante País/Región como asunto: Asia Idioma: Coreano Revista: Korean Journal of Hematology Año: 2001 Tipo del documento: Artículo