IRCT20150125020786N3 IRCT 2018-07-08 Pouyesh Darou biopharmaceutical Co. Follow-up of Severe Congenital Neutropenia Patients Receiving PegGCSF (PD lasta); Study of Early and Late Drug Complications Follow-up of Severe Congenital Neutropenia Patients Receiving PegGCSF (PD lasta); Study of Early and Late Drug Complications 2018-07-23 anticipated 10 Complete https://irct.ir/trial/18379 interventional Randomization: N/A, Blinding: Not blinded, Placebo: Not used, Assignment: Single, Purpose: Treatment. 1-2 Congenital Neutropenia. PegGCSF produced by Pouyesh Darou Prescribed weekly or every 2 weeks and the dose will be adjusted based on response to drug. No - There is not a plan to make this available Justification or reason for not sharing IPD is Patients recruitment will be before January 1st 2019.

public Marzieh Maddah

4th Floor, Building No. 3, Children's Hospital Medical Center, Keshavarz Blvd., Qarib Street

Tehran Iran (Islamic Republic of) 1419733151 +98 21 6693 5855 marzieh.maddah@gmail.com Immunology, Asthma and Allergy Research Institute scientific Mostafa Moin

4th Floor, Building No. 3, Children's Hospital Medical Center, Qarib Street

Tehran Iran (Islamic Republic of) 1419733151 +98 21 6693 5855 mmoin@tums.ac.ir Immunology, Asthma and Allergy Research Institute Iran (Islamic Republic of) Adult patients and children with severe congenital neutropenia over 12 years Severe Congenital Neutropenia Patients Myeloid maturation arrest in bone marrow Already successfully treated with Filgrastim Negative mutation in GCSF receptor Normal spleen and liver in abdominal sonography Consent to Participate in study and sign inform consent Cooperative patient for close follow-up 12 years no limit Both Secondary neutropenia due to chemotherapy or other diseases Life-threatening complications such as anaphylaxis or splenic rupture due to Filgrastim Uncooperative patient for close follow-up Severe Spleenomegaly patients with other chronic diseases such as heart or hepatic disease or ... D70 Neutropenia Treatment - Drugs PegGCSF produced by Pouyesh Darou Prescribed weekly or every 2 weeks and the dose will be adjusted based on response to drug Absolute Neutrophil Count. Timepoint: first day of study , 7th day, 14th day, 21st day, 28th day, 35th day, 42nd day, 49th day, 56th day, 3th day, 70th day, 77th day, 84th day, 91st day then every month until reciving the drug. Method of measurement: Blood Sample. Infection. Timepoint: Monthly. Method of measurement: Clinical questionnare. Hospitalization rate. Timepoint: Monthly. Method of measurement: Clinical questionnare. Immunology, Asthma and Allergy research Institute Pouyesh Darou biopharmaceutical Co. Immunology, Asthma and Allergy research Institute Approved 2014-01-04 Ethics committee of Immunology , Asthma and Allergy Research Institiute 4th Floor, Building No. 3, Tehran - Children's Hospital Medical Center, Keshavarz Blvd. Qarib Street Tehran Tehran Iran (Islamic Republic of)