|
|
 Previous Article | Table of Contents | Next Article 
Blood, Vol. 92 No. 8 (October 15), 1998:
pp. 2629-2640
REVIEW ARTICLE
Cyclical Neutropenia and Other Periodic Hematological Disorders: A
Review of Mechanisms and Mathematical Models
Caroline Haurie,
David C. Dale, and
Michael C. Mackey
From the Departments of Physiology, Physics, and Mathematics, Center
for Nonlinear Dynamics in Physiology and Medicine, McGill University,
Montreal, Quebec, Canada; and the Division of Hematology, Department of
Medicine, University of Washington, Seattle, WA.
Although all blood cells are derived from hematopoietic stem cells,
the regulation of this production system is only partially understood.
Negative feedback control mediated by erythropoietin and thrombopoietin
regulates erythrocyte and platelet production, respectively, but the
regulation of leukocyte levels is less well understood. The local
regulatory mechanisms within the hematopoietic stem cells are also not
well characterized at this point. Because of their dynamic character,
cyclical neutropenia and other periodic hematological disorders offer a
rare opportunity to more fully understand the nature of these
regulatory processes. We review the salient clinical and laboratory
features of cyclical neutropenia (and the less common disorders
periodic chronic myelogenous leukemia, periodic auto-immune hemolytic
anemia, polycythemia vera, aplastic anemia, and cyclical
thrombocytopenia) and the insight into these diseases afforded by
mathematical modeling. We argue that the available evidence indicates
that the locus of the defect in most of these dynamic diseases is at
the stem cell level (auto-immune hemolytic anemia and cyclical
thrombocytopenia seem to be the exceptions). Abnormal responses to
growth factors or accelerated cell loss through apoptosis may play an
important role in the genesis of these disorders.
© 1998 by The American Society of Hematology.
 CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati What's this?
This article has been cited by other articles:
|
|
|
|
 
S. J. Salipante, M. E. B. Rojas, B. Korkmaz, Z. Duan, J. Wechsler, K. F. Benson, R. E. Person, H. L. Grimes, and M. S. Horwitz
Contributions to Neutropenia from PFAAP5 (N4BP2L2), a Novel Protein Mediating Transcriptional Repressor Cooperation between Gfi1 and Neutrophil Elastase
Mol. Cell. Biol.,
August 15, 2009;
29(16):
4394 - 4405.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
D. S. Grenda, M. Murakami, J. Ghatak, J. Xia, L. A. Boxer, D. Dale, M. C. Dinauer, and D. C. Link
Mutations of the ELA2 gene found in patients with severe congenital neutropenia induce the unfolded protein response and cellular apoptosis
Blood,
December 15, 2007;
110(13):
4179 - 4187.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. S. Horwitz, Z. Duan, B. Korkmaz, H.-H. Lee, M. E. Mealiffe, and S. J. Salipante
Neutrophil elastase in cyclic and severe congenital neutropenia
Blood,
March 1, 2007;
109(5):
1817 - 1824.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
C. J. Gore, W. G. Hopkins, and C. M. Burge
Errors of measurement for blood volume parameters: a meta-analysis
J Appl Physiol,
November 1, 2005;
99(5):
1745 - 1758.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
C. Bellanne-Chantelot, S. Clauin, T. Leblanc, B. Cassinat, F. Rodrigues-Lima, S. Beaufils, C. Vaury, M. Barkaoui, O. Fenneteau, M. Maier-Redelsperger, et al.
Mutations in the ELA2 gene correlate with more severe expression of neutropenia: a study of 81 patients from the French Neutropenia Register
Blood,
June 1, 2004;
103(11):
4119 - 4125.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. S. Lim and K. S.J. Elenitoba-Johnson
The Molecular Pathology of Primary Immunodeficiencies
J. Mol. Diagn.,
May 1, 2004;
6(2):
59 - 83.
[Full Text]
[PDF]
|
|
|
|
|
|
|
F. E. Ouriaghli, E. Sloand, L. Mainwaring, H. Fujiwara, K. Keyvanfar, J. J. Melenhorst, K. Rezvani, G. Sconocchia, S. Solomon, N. Hensel, et al.
Clonal dominance of chronic myelogenous leukemia is associated with diminished sensitivity to the antiproliferative effects of neutrophil elastase
Blood,
November 15, 2003;
102(10):
3786 - 3792.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
T. Andrews and K. E. Sullivan
Infections in Patients with Inherited Defects in Phagocytic Function
Clin. Microbiol. Rev.,
October 1, 2003;
16(4):
597 - 621.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
D. S. Grenda, S. E. Johnson, J. R. Mayer, M. L. McLemore, K. F. Benson, M. Horwitz, and D. C. Link
Mice expressing a neutrophil elastase mutation derived from patients with severe congenital neutropenia have normal granulopoiesis
Blood,
October 16, 2002;
100(9):
3221 - 3228.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
W. C. Zamboni, D. Z. D'Argenio, C. F. Stewart, T. MacVittie, B. J. Delauter, A. M. Farese, D. M. Potter, N. M. Kubat, D. Tubergen, and M. J. Egorin
Pharmacodynamic Model of Topotecan-induced Time Course of Neutropenia
Clin. Cancer Res.,
August 1, 2001;
7(8):
2301 - 2308.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. A. G. Aprikyan, W. C. Liles, E. Rodger, M. Jonas, E. Y. Chi, and D. C. Dale
Impaired survival of bone marrow hematopoietic progenitor cells in cyclic neutropenia
Blood,
January 1, 2001;
97(1):
147 - 153.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. A. Lekstrom-Himes and J. I. Gallin
Immunodeficiency Diseases Caused by Defects in Phagocytes
N. Engl. J. Med.,
December 7, 2000;
343(23):
1703 - 1714.
[Full Text]
[PDF]
|
|
|
|
|
|
|
D. C. Dale, R. E. Person, A. A. Bolyard, A. G. Aprikyan, C. Bos, M. A. Bonilla, L. A. Boxer, G. Kannourakis, C. Zeidler, K. Welte, et al.
Mutations in the gene encoding neutrophil elastase in congenital and cyclic neutropenia
Blood,
October 1, 2000;
96(7):
2317 - 2322.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Tefferi, M. A. Elliott, P. C. Kao, S. Yoon, I. El-Hemaidi, and T. C. Pearson
Hydroxyurea-induced marked oscillations of platelet counts in patients with polycythemia vera
Blood,
August 15, 2000;
96(4):
1582 - 1584.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
H. Tagaya, T. Kunisada, H. Yamazaki, T. Yamane, T. Tokuhisa, E. F. Wagner, T. Sudo, L. D. Shultz, and S.-I. Hayashi
Intramedullary and extramedullary B lymphopoiesis in osteopetrotic mice
Blood,
June 1, 2000;
95(11):
3363 - 3370.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. C. Dinauer, J. A. Lekstrom-Himes, and D. C. Dale
Inherited Neutrophil Disorders: Molecular Basis and New Therapies
Hematology,
January 1, 2000;
2000(1):
303 - 318.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Morley;, C. Haurie, M. C. Mackey, and D. C. Dale
Periodic Hematological Disorders
Blood,
June 1, 1999;
93(11):
4023 - 4024.
[Full Text]
[PDF]
|
|
|
|
|
