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Severe neutropenia in RD is typically unresponsive to recombinant human granulocyte colony-stimulating factor (rhG-CSF). To delineate lineage-specific transcriptional responses and cellular heterogeneity induced by rhG-CSF, we integrated single-cell RNA sequencing from seven samples, including two RD patients (with and without rhG-CSF) and three pediatric controls. We identified a moderate increase in hematopoietic stem and progenitor cells (HSPCs) and common myeloid progenitor\/granulocyte\u2013monocyte progenitor fractions following rhG-CSF. Notably, B cell fractions increased after rhG-CSF, accompanied by enhanced maturation from common lymphoid progenitors to precursor B cells. In parallel, genes involved in B cell differentiation were markedly upregulated compared with untreated cells. It also modulated the HSPC compartment by markedly suppressing interferon-\u03b3 (IFN\u03b3) signaling pathways. RhG-CSF administration before HSCT may benefit patients with RD by modestly increasing neutrophils and supporting infection control, while suppressing IFN\u03b3 signaling in HSPCs and potentially promoting B cell differentiation.<\/jats:p>","DOI":"10.70962\/jhi.20250232","type":"journal-article","created":{"date-parts":[[2026,3,7]],"date-time":"2026-03-07T07:21:41Z","timestamp":1772868101000},"update-policy":"https:\/\/doi.org\/10.70962\/jhi.crossmarkpolicy","source":"Crossref","is-referenced-by-count":1,"title":["Response to recombinant human granulocyte colony-stimulating factor in reticular dysgenesis"],"prefix":"10.70962","volume":"2","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0364-5886","authenticated-orcid":true,"given":"Manabu","family":"Wakamatsu","sequence":"first","affiliation":[{"id":[{"id":"https:\/\/ror.org\/04chrp450","id-type":"ROR","asserted-by":"publisher"}],"name":"Nagoya University Graduate School of Medicine 1 Department of Pediatrics, , 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