The DOI matches its Crossref record; 34 of the 34 references it cites were found in a registry or an index.
Elevated serum tryptase levels identify a subset of patients with a myeloproliferative variant of idiopathic hypereosinophilic syndrome associated with tissue fibrosis, poor prognosis, and imatinib responsiveness
Abstract
Since serum tryptase levels are elevated in some patients with myeloproliferative disorders, we examined their utility in identifying a subset of patients with hypereosinophilic syndrome (HES) and an underlying myeloproliferative disorder. Elevated serum tryptase levels (> 11.5 ng/mL) were present in 9 of 15 patients with HES and were associated with other markers of myeloproliferation, including elevated B12 levels and splenomegaly. Although bone marrow biopsies in these patients showed increased numbers of CD25+ mast cells and atypical spindle-shaped mast cells, patients with HES and elevated serum tryptase could be distinguished from patients with systemic mastocytosis and eosinophilia by their clinical manifestations, the absence of mast cell aggregates, the lack of a somatic KIT mutation, and the presence of the recently described fusion of the Fip1–like 1 (FIP1L1) gene to the platelet-derived growth factor receptor α gene (PDGFRA). Patients with HES and elevated serum tryptase were more likely to develop fibroproliferative end organ damage, and 3 of 9 died within 5 years of diagnosis in contrast to 0 of 6 patients with normal serum tryptase levels. All 6 patients with HES and elevated tryptase treated with imatinib demonstrated a clinical and hematologic response. In summary, elevated serum tryptase appears to be a sensitive marker of a myeloproliferative variant of HES that is characterized by tissue fibrosis, poor prognosis, and imatinib responsiveness.
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Self-citationThe hypereosinophilic syndrome: analysis of fourteen cases with review of the literature.
Chusid, M. J., Dale, D. C., West, B. C., & Wolff, S. M. (1975) · PubMed · Cited by 1,233
Diagnostic criteria and classification of mastocytosis: a consensus proposal
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Self-citationTHE HYPEREOSINOPHILIC SYNDROME: Analysis of Fourteen Cases With Review of The Literature
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The idiopathic hypereosinophilic syndrome
Weller, P., & Bubley, G. (1994) · Blood · doi:10.1182/blood.v83.10.2759.2759 · Cited by 929
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Self-citationMast cells as a source of multifunctional cytokines
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Abnormal Clones of T Cells Producing Interleukin-5 in Idiopathic Eosinophilia
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Cited by
Eosinophils: Biological Properties and Role in Health and Disease
HOGAN, S. P., ROSENBERG, H. F., MOQBEL, R., PHIPPS, S., FOSTER, P. S., LACY, P., KAY, A. B., & ROTHENBERG, M. E. (2008) · Clinical & Experimental Allergy · doi:10.1111/j.1365-2222.2008.02958.x · Cited by 834
Diagnostic Value of Tryptase in Anaphylaxis and Mastocytosis
Schwartz, L. B. (2006) · Immunology and Allergy Clinics of North America · doi:10.1016/j.iac.2006.05.010 · Cited by 477
Anaphylaxis During the Perioperative Period
Hepner, D. L., & Castells, M. C. (2003) · Anesthesia & Analgesia · doi:10.1213/01.ane.0000082993.84883.7d · Cited by 375
Approaches to the treatment of hypereosinophilic syndromes: A workshop summary report
Klion, A. D., Bochner, B. S., Gleich, G. J., Nutman, T. B., Rothenberg, M. E., Simon, H.-U., Wechsler, M. E., Weller, P. F., & the Hypereosinophilic Syndromes Working Group (2006) · Journal of Allergy and Clinical Immunology · doi:10.1016/j.jaci.2006.02.042 · Cited by 342
Self-citationPardanani, A., Brockman, S. R., Paternoster, S. F., Flynn, H. C., Ketterling, R. P., Lasho, T. L., Ho, C.-L., Li, C.-Y., Dewald, G. W., & Tefferi, A. (2004) · Blood · doi:10.1182/blood-2004-03-0787 · Cited by 309
Metadata
- Authors
- Klion, A. D., Noel, P., Akin, C., Law, M. A., Gilliland, D. G., Cools, J., Metcalfe, D. D., & Nutman, T. B.
- Venue
- Blood
- Publisher
- American Society of Hematology
- Published
- 2003-06-15
- Volume
- 101
- Issue
- 12
- Pages
- 4660-4666
- ISSN
- 0006-4971 (ISSN-L)
- 1528-0020
- OpenAlex
- W2064244980
- Cited by
- 386
- References
- 34
- FWCI
- 17.83
- Type
- journal article
- Language
- en
- Open access
- closed
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- Klion, A. D., Noel, P., Akin, C., Law, M. A., Gilliland, D. G., Cools, J., Metcalfe, D. D., & Nutman, T. B. (2003). Elevated serum tryptase levels identify a subset of patients with a myeloproliferative variant of idiopathic hypereosinophilic syndrome associated with tissue fibrosis, poor prognosis, and imatinib responsiveness. Blood. https://doi.org/10.1182/blood-2003-01-0006
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