| Co-Investigator(Kenkyū-buntansha) |
YASUI Kozo Shinshu Univ. School Med., Dept. Pediatrics, Instructor, 医学部, 助手 (90200493)
KAWAI Hiroshi Shinshu Univ. School Med., Dept. Pediatrics, Assistant Prof., 医学部, 講師 (70126671)
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| Budget Amount *help |
¥2,700,000 (Direct Cost: ¥2,700,000)
Fiscal Year 1989: ¥700,000 (Direct Cost: ¥700,000)
Fiscal Year 1988: ¥2,000,000 (Direct Cost: ¥2,000,000)
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| Research Abstract |
The present study was conducted to establish hemopoietic cytokine therapy for chronic neutropenias of childhood. Mechanisms for neutropenia were first analyzed to make better protocols of hemopoietic cytokine therapy. Then, a hemopoigtic cytokine (M-CSF or G-CSF) was given based on the protocol to 35 patients with primary neutropenia, 1 with glycogen storage disease type Ib, and 7 with aplastic anemia.
1. Mechanisms for neutropenia
(1) There was no decrease in the number of neutrophil progenitor cells (GM-CFU) in primary neutropenia and glycogen storage disease type Ib, which indicated probable favorable clinical effects of the cytokines by their activity to promote proliferation of the progenitor cells in these disorders. In aplastic anemia, GM-CFU were not detectable or present in decreased numbers. This finding indicated possible favorable clinical effects of hemopoietic cytokine therapy in some patients with aplastic anemia.
(2) Anti-neutrophil antibody was detectable in chronic benig
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n neutropenia of infancy. It was expected that an increase in neutrophil numbers by hemopoietic cytokines may decrease the antibody and result in earlier improvement of the neutropenia.
2. Hemopoietic cytokine therapy and its clinical effects
(1) M-CSF (6 X 10^6 U/m^2/day was administered by intravenous drip infusion for consecutive 7 days. G-CSF (50 - 400mug/m^2/day) was administered subcutaneously (intravenously in a patient) for consecutive 14 days or more. The patient with glycogen storage disease type Ib was placed on the maintenance therapy thereafter.
(2) Primary neutropenia: Twenty-eight patients were given M-CSF, and sufficient data were obtained from 19 of them; there was an increase in neutrophil counts in 10 (52.6%) of the 19 patients. Five patients received G-CSF, and the neutrophil counts increased in all of them. It was noteworthy that there was an apparent increase ( >1,000/muL) in neutrophil counts, and intractable infections were successfully treated by the therapy in two of congenital neutropenia patients.
(3) Glycogen storage disease type Ib,(6 yr, male): The G-CSF administration yielded a recovery from the neutropenia and persistent infections. His neutrophil counts are being maintained at levels of > 1,000/muL by twice/week of maintenance administration of G-CSF (60 mug/m^2/day), and infection episodes have been apparently decreased.
(4,) Aplastic anemia: There was an increase in neutrophil counts in four of 7 patients who were treated with G-CSF. One patient complained bone pain during the therapy, but there were no side effects in the others. Less
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