A neonate with recurrent infection was diagnosed with Kostmann syndrome. Treatment is:
Severe congenital neutropenia (SCN) consists of a group of inherited diseases that are characterized by severely impaired neutrophil counts [ Diagnosis of SCN requires examination of the bone marrow. Subcutaneous injection of the cytokine granulocyte colony-stimulating factor (G-CSF) usually improves neutrophil development and thus prevents infection in most SCN diseases. SCN with ELA2 mutation are refractory to G-CSF and may require curative treatment allogeneic hematopoietic stem cell transplantation (HSCT). Ref: Dale D.C. (2010). Chapter 65. Neutropenia and Neutrophilia. In J.T. Prchal, K. Kaushansky, M.A. Lichtman, T.J. Kipps, U. Seligsohn (Eds), Williams Hematology, 8e.
G-CSF and HSCT
GM-CSF
Antithymocyte globulin+cyclosporine
Antithymocyte globulin+cyclosporine +GM-CSF