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December 8, 2025BloodOpen Access

A novel spontaneous HLH mouse model by engrafting human HSCs into b-ndg MGMT3 mice

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Authors

RLRunxin LvCSChengzhang ShangJYJiangfeng Yuan

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Overview

Animal model demonstrates HLH features and elevated immune responses, suggesting new therapeutic avenues with etoposide and dexamethasone.

Key Points

  • To establish a new mouse model of hemophagocytic lymphohistiocytosis (HLH) to study human immune dysregulation and therapy effectiveness.
  • Generated B-NDG MGMT3 mice through genetic engineering and engrafted with human umbilical cord hematopoietic stem cells.
  • Monitored survival and analyzed peripheral blood through flow cytometry for immune cell quantification.
  • Conducted complete blood counts and tissue staining for histological evaluation of immune responses.
  • Mice exhibited significant mortality post-HSC engraftment despite weight gain.
  • Human CD45+ leukocytes achieved 70% chimerism with diverse immune cell types observed.
  • Complete blood counts indicated elevated leukocytes and reticulocytes while erythrocytes and hemoglobin were reduced.

Cite This Study

Lv et al. (2025) studied this question.

synapsesocial.com/papers/69362f4b4fa91c937236d75bhttps://doi.org/10.1182/blood-2025-1204
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Also Consider

Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context:

  1. 1Abstract 3390: Enhanced human immune system engraftment in a novel triple-engineered NCG-hIL6-mTSLP-mFlt3-KOmouse model2026
  2. 2Abstract 6977: B-NDG MHC I/II DKO mice plus: A refined humanized model for evaluating T cell engager efficacy and toxicity2026
  3. 3Abstract 112: The NSG-Flt3KO; Tg(hFLT3LG); hIL15 mouse strain improves engraftment of innate immune cells post-CD34+ humanization2024 · 1 citations
  4. 4Tamoxifen-inducible Pld3 Pld4 knockout mouse model as an animal model for secondary HLH and its treatment 20272025
  5. 5Pathological insights of human CD34+ hematopoietic stem cell-engrafted NSG mice: Implications for the safety assessment of cancer immunotherapy drugs2026 · 1 citations