|
|
 Previous Article | Table of Contents | Next Article 
Blood, 1970, Vol. 36, No. 5, pp. 576-585.
© 1970 American Society of Hematology, Inc.
Congenital Agranulocytosis: Prolonged Survival and
Terminal Acute Leukemia
PRISCILLA A. GILMAN 1,
DUDLEY P. JACKSON 1, and
HARRIET G. GUILD 1
1 Departments of Medicine and Pediatrics of The Johns Hopkins University
School of Medicine and Hospital, Baltimore, Md.
Three unrelated, Caucasian patients with
a disorder resembling infantile genetic
agranulocytosis have been studied. There
was no history of consanguinity. Parents and siblings were normal. Onset
occurred before 3 weeks of age and persisted throughout life. All had severe
neutropenia, myeloid arrest pattern of
the bone marrow, eosinophilia, monocytosis and hyperglobulinemia. Other congenital anomalies were not present. Their
clinical courses were characterized by recurrent, severe bacterial infections particularly of the skin, mouth and lungs
but without bacteremia. The infections
responded to, but were not prevented by
antibiotics, which were administered almost continuously. Fungal infections did
not occur and viral infections were not
severe. Splenectomy and corticosteroids
were without apparent benefit. Relative,
though transient, neutrophilia occurred
during a few episodes of infection.
Chromosome analysis was normal in two
of the patients. In one, maturation of
neutrophilic leukocytes was not observed
in cultures of bone marrow to which
normal serum or cysteine was added.
One boy is living at 14 10/12 years of
age, and one died at 13 years of disseminated infection. A unique occurrence
was the development of terminal acute
leukemia (monocytic) in the third patient,
a girl at the age of 14 years.
Submitted on April 1, 1970
Revised on May 20, 1970
Accepted on May 27, 1970
 CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati What's this?
This article has been cited by other articles:
|
|
|
|
 
M. Germeshausen, M. Ballmaier, and K. Welte
Incidence of CSF3R mutations in severe congenital neutropenia and relevance for leukemogenesis: results of a long-term survey
Blood,
January 1, 2007;
109(1):
93 - 99.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
P. S. Rosenberg, B. P. Alter, A. A. Bolyard, M. A. Bonilla, L. A. Boxer, B. Cham, C. Fier, M. Freedman, G. Kannourakis, S. Kinsey, et al.
The incidence of leukemia and mortality from sepsis in patients with severe congenital neutropenia receiving long-term G-CSF therapy
Blood,
June 15, 2006;
107(12):
4628 - 4635.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
R Lakshman and A Finn
Neutrophil disorders and their management
J. Clin. Pathol.,
January 1, 2001;
54(1):
7 - 19.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. Jeha, K. W. Chan, A. G. Aprikyan, W. K. Hoots, S. Culbert, H. Zietz, D. C. Dale, and M. Albitar
Spontaneous remission of granulocyte colony-stimulating factor-associated leukemia in a child with severe congenital neutropenia
Blood,
November 15, 2000;
96(10):
3647 - 3649.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. H. Freedman, M. A. Bonilla, C. Fier, A. A. Bolyard, D. Scarlata, L. A. Boxer, S. Brown, B. Cham, G. Kannourakis, S. E. Kinsey, et al.
Myelodysplasia syndrome and acute myeloid leukemia in patients with congenital neutropenia receiving G-CSF therapy
Blood,
July 15, 2000;
96(2):
429 - 436.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
C. Zeidler, K. Welte, Y. Barak, F. Barriga, A. A. Bolyard, L. Boxer, G. Cornu, M. J. Cowan, D. C. Dale, T. Flood, et al.
Stem cell transplantation in patients with severe congenital neutropenia without evidence of leukemic transformation
Blood,
February 15, 2000;
95(4):
1195 - 1198.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
P. Anderlini, M. Korbling, D. Dale, A. Gratwohl, N. Schmitz, D. Stroncek, C. Howe, S. Leitman, M. Horowitz, E. Gluckman, et al.
Allogeneic Blood Stem Cell Transplantation: Considerations for Donors
Blood,
August 1, 1997;
90(3):
903 - 908.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. Korbling
Peripheral Blood Stem Cells: A Novel Source for Allogeneic Transplantation
Oncologist,
April 1, 1997;
2(2):
104 - 104.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. H. Freedman
Congenital Marrow Failure Syndromes and Malignant Hematopoietic Transformation
Oncologist,
December 1, 1996;
1(6):
354 - 360.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
F. Dong, R. K. Brynes, N. Tidow, K. Welte, B. Lowenberg, and I. P. Touw
Mutations in the Gene for the Granulocyte Colony-Stimulating-Factor Receptor in Patients with Acute Myeloid Leukemia Preceded by Severe Congenital Neutropenia
N. Engl. J. Med.,
August 24, 1995;
333(8):
487 - 493.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. Kato, K. Tsuji, Y. Tsunematsu, R. Koide, and J. Utsumi
Familial Leukemia: HLA System and Leukemia Predisposition in a Family
Arch Pediatr Adolesc Med,
July 1, 1983;
137(7):
641 - 644.
[Abstract]
[PDF]
|
|
|
|
|
