Korean J Hematol.  2001 Nov;36(4):346-350.

Prolonged Remission Following Autologous Peripheral Blood Stem Cell Transplantation in an Infant with Familial Hemophagocytic Lymphohistiocytosis

• Kim JS
• Seo JH
• Hwang JY
• Kim YS
• Park CH
• Kim SJ
• Maeng KY
• Park BK

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.

Keyword

Familial hemophagocytic lymphohistiocytosis; Autologous peripheral blood stem cell transplantation

MeSH Terms

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