The Severe Chronic Neutropenia International Registry

The Severe Chronic Neutropenia International Registry (SCNIR) was established in 1994 to monitor the clinical course, treatment, and disease outcomes in patients with severe chronic neutropenia (SCN). The Registry has the largest collection of long-term data on patients with this condition in the world. Participation in the Registry benefits patients, their families and the physicians who treat them by providing the most up to date information to them on the natural history of SCN and its treatment options.

Patients qualify for the Registry if:

• ANC’s are under 500 per mm³ (0.5 X 10⁹/L) on at least three occasions in the three months prior to applying for the Registry (if currently treated, 3 ANC's under 500 prior to the start of G-CSF therapy) with the exception of patients with Shwachman-Diamond syndrome who are enrolled with higher ANC's or even no neutropenia at all.
• There is a history of recurrent infections.

Patients do not qualify for the Registry if:

• Their neutropenia is known to be drug-induced.
• The patient has any one of the following conditions:
  • Thrombocytopenia* (SDS and GSD1b patients are an exception to this exclusion)
  • Myelodysplastic Syndrome
  • Aplastic anaemia
  • HIV positive
• Known immune diseases such as rheumatoid arthritis
• The patient has had previous (within the past 5 years) chemotherapy for cancer.

*Shwachman-Diamond syndrome and Glycogen Storage Disease type 1b patients are an exception to this exclusion.

For patients qualifying for the Registry the following basic examinations are required:

• A bone marrow evaluation has been completed that confirms the diagnosis of SCN.
• A cytogenetic evaluation has been completed, if G-CSF treatment has been considered or initiated.
• The patient has signed a formal consent to allow the use of his/her anomymised data.

The objectives of the SCNIR are:

• Document the clinical course of SCN and monitor clinically significant changes e.g. primary treatment response over time and long-term safety.
• Study the incidence and/or outcome of the following previously identified adverse events: osteoporosis, splenomegaly, vasculitis, thrombocytopenia, cytogenetic abnormalities, myelodysplastic syndrome, and leukaemia.
• Evaluate growth and development in pediatric patients as well as hematologic parameters and pregnancy outcomes in all patients.
• Monitor for clinically significant changes in primary treatment response over time.
• Establish a physician network to increase the understanding of SCN.
• Establish a demographic database to allow for future research.
• Collect bone marrow samples of patients at different time points for future research.

The SCNIR consists of:

An Advisory Board of expert physicians/haematologists and consultants:

Dr. Blanche Alter National Cancer Institute Rockville, MD USA	Dr. Mary Ann Bonilla St. Joseph's Children's Hospital Paterson, NJ USA	Dr. Laurence Boxer University of Michigan Ann Arbor, MI USA	Dr. Bonnie Cham CancerCare Manitoba Winnipeg, MB, Canada
Dr. David C. Dale SCNIR Co-Director University of Washington Seattle, WA USA	Dr. Jean Donadieu Hospital Trousseau Paris, France	Carol J. Fier, RN, MSN Amgen, Inc Boulder, CO USA	Dr. Melvin Freedman Hospital for Sick Children Toronto, ON Canada
Dr. George Kannourakis University of Ballarat Ballarat, Victoria Australia	Dr. Sally Kinsey St James's University Hospital Leeds, West Yorkshire UK	Lee Reeves Patient Advocate Howell, MI USA	Prof. Karl Welte SCNIR Co-Director Medizinische Hochschule Hannover, Germany	Dr. Jerry Winkelstein Johns Hopkins Baltimore, MD USA

And a panel of European physicians/haematologists called the Local Liaison Physicians located throughout the European countries:

Dr. Tore Abrahamsen Norway	Dr. Phil Ancliff UK	Dr. Maurizio Aurico Italy	Dr. Yigal Barak Israel
Dr. Marie Bruin The Netherlands	Dr. Katharina Clodi Austria	Dr. Göran Elinder Sweden	Dr. Evaristo F. Feliu Spain
Dr. Mirjana Gotic Serbia	Dr. Tayfun Gungor Switzerland	Dr. Aydan Ikinciogullari Turkey	Dr. Krzysztof Kalwak Poland
Dr. Antonis Kattamis Greece	Dr. Andries Louwagie Belgium	Dr. Laszlo Marodi Hungary	Dr. Gundula Notheis Germany
Dr. Juan J. Ortega Aramburu Spain	Prof. Jan Palmblad Sweden	Dr. Helen Papadaki Greece	Dr. A.Y.N. Schouten-van Meeteren The Netherlands
Dr. Owen Smith Ireland	Dr. Adriana Teixeira Portugal	Dr. Geir Tjonnfjord Norway	Dr. Fabio Tucci Italy
Dr. Christiane Vermylen Belgium	Dr. Jaroslava Voglova Czech Republic	Dr. Sonja Zweegman The Netherlands