Prolonged Remission Following Autologous Peripheral Blood Stem Cell Transplantation in an Infant with Familial Hemophagocytic Lymphohistiocytosis.
- Author:
Jeum Su KIM
1
;
Ji Hyoun SEO
;
Ji Young HWANG
;
Young Suk KIM
;
Chan Hoo PARK
;
Sun Joo KIM
;
Kook Young MAENG
;
Byung Kiu PARK
Author Information
1. Department of Pediatrics, College of Medicine, Gyeongsang National University, Korea.
- Publication Type:Case Report
- Keywords:
Familial hemophagocytic lymphohistiocytosis;
Autologous peripheral blood stem cell transplantation
- MeSH:
Bone Marrow Examination;
Bone Marrow Transplantation;
Diagnosis;
Drug Therapy;
Employment;
Erythrocytes;
Female;
Fever;
Histiocytes;
Humans;
Infant*;
Korea;
Life Expectancy;
Lymphocytes;
Lymphohistiocytosis, Hemophagocytic*;
Peripheral Blood Stem Cell Transplantation*;
Siblings
- From:Korean Journal of Hematology
2001;36(4):346-350
- CountryRepublic of Korea
- Language:Korean
-
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.
