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Blood, Vol. 92 No. 4 (August 15), 1998:
pp. 1097-1103
RAPID COMMUNICATION
Defective Expression of Granulocyte-Macrophage Colony-Stimulating
Factor/Interleukin-3/Interleukin-5 Receptor Common  Chain in
Children With Acute Myeloid Leukemia Associated With Respiratory
Failure
Uta Dirksen,
Uwe Hattenhorst,
Peter Schneider,
Horst Schroten,
Ulrich Göbel,
Alfred Böcking,
Klaus-Michael Müller,
Richard Murray, and
Stefan Burdach
From the Department of Pediatric Hematology/Oncology and the
Department of General Pediatrics and Pulmonology, Children's Hospital
Medical Center and the Institute for Cytopathology and the Department
of Internal Medicine Hematology/Oncology, and the Center for BioMedical
Research (BMFZ), Heinrich-Heine-University, Düsseldorf, Germany;
Children's Hospital Medical Center, Martin-Luther University, Halle,
Germany; The Institute for Pathology, Ruhr University, Bochum,
Germany; and The DNAX Research Institute, Palo Alto, CA.
Deficiency of the granulocyte-macrophage colony-stimulating factor
(GM-CSF)/interleukin-3 (IL-3)/IL-5 receptors common  chain (c) is
a cause of fatal respiratory failure. c deficiency manifests as
pulmonary alveolar proteinosis (PAP). PAP has heterogenous etiologies
that may be genetic or aquired. Some cases of PAP have been reported to
be associated with hematologic malignancies such as acute myeloid
leukemia (AML). In mice, the PAP phenotype was generated
by targeted deletion of the gene for c and can be treated by
transplantation of wild-type bone marrow into c  / mice. Thus,
our findings in c / mice provide evidence for a causal relationship between the lung disease and the hematopoietic system. We
describe here expression defects of c or c plus GM-CSF receptor  chain (GM-CSFR ) in 3 pediatric patients with AML and PAP
symptoms. All of the patients' leukemic cells failed to express normal
levels of c. The leukemic cells of patients no. 2 and 3 additionally lacked the expression of GM-CSFR , as shown by flow cytometry. Strikingly reduced or absent function of c was demonstrated in clonogenic progenitor assays with absent colony-forming unit (CFU) growth after GM-CSF or IL-3 stimulation. The response to growth factors
acting via a growth factor receptor distinct from the GM-CSF/IL-3/IL-5
system (recombinant human granulocyte colony-stimulating factor
[rhG-CSF]) was normal. After antileukemic treatment, the pulmonary symptoms resolved and c or c plus GM-CSFR
expression was normal. Our findings provide evidence that a defect in
the expression of c or c plus GM-CSFR on AML blasts can be
associated with respiratory failure in patients with AML.
© 1998 by The American Society of Hematology.
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