Prolonged Remission Following Autologous Peripheral Blood Stem Cell Transplantation in an Infant with Familial Hemophagocytic Lymphohistiocytosis / 대한혈액학회지
Korean Journal of Hematology
;
: 346-350, 2001.
Artigo
em 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.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Exame de Medula Óssea
/
Linfócitos
/
Expectativa de Vida
/
Transplante de Medula Óssea
/
Transplante de Células-Tronco de Sangue Periférico
/
Irmãos
/
Diagnóstico
/
Tratamento Farmacológico
/
Emprego
/
Eritrócitos
Tipo de estudo:
Estudo diagnóstico
Limite:
Feminino
/
Humanos
/
Lactente
País/Região como assunto:
Ásia
Idioma:
Coreano
Revista:
Korean Journal of Hematology
Ano de publicação:
2001
Tipo de documento:
Artigo
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