Prolonged Remission Following Autologous Peripheral Blood Stem Cell Transplantation in an Infant with Familial Hemophagocytic Lymphohistiocytosis / 대한혈액학회지
Korean Journal of Hematology
;
: 346-350, 2001.
Article
Dans Coréen
| 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.
Texte intégral:
Disponible
Indice:
WPRIM (Pacifique occidental)
Sujet Principal:
Myélogramme
/
Lymphocytes
/
Espérance de vie
/
Transplantation de moelle osseuse
/
Transplantation de cellules souches de sang périphérique
/
Fratrie
/
Diagnostic
/
Traitement médicamenteux
/
Emploi
/
Érythrocytes
Type d'étude:
Etude diagnostique
Limites du sujet:
Femelle
/
Humains
/
Bébé
Pays comme sujet:
Asie
langue:
Coréen
Texte intégral:
Korean Journal of Hematology
Année:
2001
Type:
Article
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