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Differential effects of granulocyte-macrophage colony-stimulating factor
and granulocyte colony-stimulating factor in children with severe
congenital neutropenia
K Welte, C Zeidler, A Reiter, W Muller, E Odenwald, L Souza and H Riehm
Department of Pediatric Hematology and Oncology, Kinderklinik der
Medizinischen Hochschule Hannover, FRG.
Severe congenital neutropenia (SCN) is a disorder of myelopoiesis
characterized by severe neutropenia secondary to a maturational arrest at
the level of promyelocytes. We treated five patients with SCN with
recombinant human granulocyte-macrophage colony-stimulating factor
(rhGM-CSF) for 42 days and subsequently, between 1 and 3 months later, with
rhG-CSF for 142 days. The objective was to evaluate the safety and ability
of these factors to elicit a neutrophil response. rhGM-CSF was administered
at a dose of 3 to 30 micrograms/kg/d (30 to 60 minutes, intravenously). In
all patients, a specific, dose-dependent increase in the absolute
granulocyte counts was observed. However, in four patients this increase
was due to an increase in eosinophils, and in only one patient it was due
to an increase in the absolute neutrophil counts (ANC). Subsequently, all
patients received rhG-CSF at a dose of 3 to 15 micrograms/kg/d
subcutaneously. In contrast to rhGM-CSF treatment, all five patients
responded to rhG-CSF during the first 6 weeks of treatment with an increase
in the ANC to above 1,000/microL. The level of ANC could be maintained
during maintenance treatment. In one patient, the increase in ANC was
associated with an improvement of a severe pneumonitis caused by
Peptostreptococcus and resistant to antibiotic treatment. No severe
bacterial infections occurred in any of the patients during CSF treatment.
All patients tolerated rhGM-CSF and rhG-CSF treatment without severe side
effects. These results demonstrate the beneficial effect of rhG-CSF in SCN
patients.
Volume 75,
Issue 5,
pp. 1056-1063,
03/01/1990
Copyright © 1990 by The American Society of Hematology
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