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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

Mouse model demonstrates HLH features like cytopenias in vivo, highlighting critical immune dysregulation mechanisms.

Key Points

  • Mortality occurred by Week 6 post-HSC engraftment, despite stable weight gain among the mice.
  • At Week 12, human leukocytes reached 70% chimerism—specifically, T/B/NK cells and macrophages.
  • Engrafted HSCs resulted in high leukocyte levels and decreased erythrocytes, hemoglobin, and platelets compared to controls.
  • Model recapitulates HLH pathophysiology with tissue infiltration of CD3+ T cells and CD68+ macrophages, indicating severe immune dysregulation.

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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