Research Studies on Chronic Neutropenia

Description of Work

Research Studies on Chronic Neutropenia are a group of studies concerned with the  consequences and treatment of diseases causing severe chronic neutropenia (SCN). This is a condition that predisposes patients, particularly children, to the risk of severe bacterial and fungal infections. Neutrophils are the critical cells defending the body from infections by organism of the surfaces of the body. When the level of these cells in the blood drops to about 10 to 20 percent of the normal level, about 500/cmm, infections are likely to occur. Many of the conditions causing severe chronic neutropenia are quite rare. Through the collaborative efforts of physicians and patients all over the world, this research program has successfully studied the diseases causing severe chronic neutropenia, performing basic research studies, investigating new treatments, and  educating physicians, nurses, other health care workers, patients and their families.

Principal Investigator:
David C. Dale, MD

Investigators:
W. Conrad Liles, MD, PhD
Steven M. Stein, MD
Andranik (Andrew) Aprikyan, PhD
Audrey Anna Bolyard, RN, BS
Elin Rodger, MT, BS

Research Projects

A brief summary of each of the research projects under Research Studies on Chronic Neutropenia appears below, along with web site links whenever available.

The Severe Chronic Neutropenia International Registry (SCNIR)

David C. Dale, Principal Investigator
Grant Title: Severe Chronic Neutropenia International Registry
Funding: National Institutes of Health (NIH); Gift from Amgen Foundation
Website: http://depts.washington.edu/registry/

The Severe Chronic Neutropenia International Registry (SCNIR) is an international disease registry seeking to establish a worldwide database of treatment and disease-related outcomes for persons diagnosed with severe chronic neutropenia (SCN). The information collected will lead to improved medical care and an increased understanding of the long term clinical course of patients with SCN. The SCNIR also serves as a focus for future research, and to educate patients, their physicians, and other caregivers about neutropenia and currently available treatments.

The SCNIR is a unit of the University of Washington, Department of Medicine, General Internal Medicine, and is directed by an advisory board of physicians who treat SCN patients.

The Severe Chronic Neutropenia Tissue Bank (Repository)

David C. Dale, Principal Investigator
Grant Title: Severe Chronic Neutropenia Tissue Bank (Repository)
Funding: National Institutes of Health (NIH); Gift from Amgen Foundation; American Cancer Society

The SCN Repository collects and maintains a bank of blood and bone marrow specimens, on both neutropenic and non-neutropenic subjects, for research on the genetic, molecular, and cellular mechanisms that cause SCN. Part of this research involves studying the genotype/phenotype relationships of mutations in the neutrophil elastase gene, ELA2, which is currently the only known cause for ELA2-related neutropenias.

The SCN Tissue Bank (Repository) is a unit of the University of Washington, Department of Medicine, General Internal Medicine.

The Neutropenia Family Studies

David C. Dale, Principal Investigator
Grant Title: Neutropenia Family Studies
Funding: National Institutes of Health (NIH); National Institutes for Human Genome Research, NIH; Gift from Amgen Foundation

The Neutropenia Family Studies project investigates families to track their hereditary patterns of neutropenia. Some, but not all, varieties of neutropenia may be due to heritable factors. This study helps to define the genetic and molecular bases for the various diseases causing SCN.

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Last reviewed: April 20, 2004 - Last updated: April 20, 2004 (mb)