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Late relapse of primary hemophagocytic lymphohistiocytosis after hematopoietic stem cell transplantation: a consequence of low-level chimerism from a carrier donor?

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References

  1. Janka GE, Lehmberg K. Hemophagocytic lymphohistiocytosis: pathogenesis and treatment. Hematology Am Soc Hematol Educ Program. 2013;2013:605–11.

    Article  PubMed  Google Scholar 

  2. Marsh RA, Vaughn G, Kim MO, Li D, Jodele S, Joshi S, et al. Reduced-intensity conditioning significantly improves survival of patients with hemophagocytic lymphohistiocytosis undergoing allogeneic hematopoietic cell transplantation. Blood. 2010;116:5824–31.

    Article  CAS  PubMed  Google Scholar 

  3. Hartz B, Marsh R, Rao K, Henter JI, Jordan M, Filipovich L, et al. The minimum required level of donor chimerism in hereditary hemophagocytic lymphohistiocytosis. Blood. 2016;127:3281–90.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  4. Ouachee-Chardin M, Elie C, de Saint Basile G, et al. Hematopoietic stem cell transplantation in hemophagocytic lymphohistiocytosis: a single- center report of 48 patients. Pediatrics. 2006;117:e743–50.

    Article  PubMed  Google Scholar 

  5. Voskoboinik I, Thia M-C, Fletcher J, Ciccone A, Browne K, Smyth MJ, et al. Calcium-dependent plasma membrane binding and cell lysis by perforin are mediated through its C2 domain. A critical role for aspartate residues 429, 435, 483 and 485 but not 491. J Biol Chem. 2005;280:8426–34.

    Article  CAS  PubMed  Google Scholar 

  6. House IG, Thia K, Brennan AJ, Tothill R, Dobrovic A, Yeh WZ, et al. Heterozygosity for the common perforin mutation, p.A91V, impairs the cytotoxicity of primary natural killer cells from healthy individuals. Immunol Cell Biol. 2015;93:575–80.

    Article  CAS  PubMed  Google Scholar 

  7. van Montfrans JM, Rudd E, van de Corput L, Henter JI, Nikkels P, Wulffraat N, et al. Fatal hemophagocytic lymphohistiocytosis in X-linked chronic granulomatous disease associated with a perforin gene variant. Pediatr Blood Cancer. 2009;52:527–9.

    Article  PubMed  Google Scholar 

  8. Vastert SJ, van Wijk R, D’Urbano LE, de Vooght KMK, de Jager W, Ravelli A, et al. Mutations in the perforin gene can be linked to macrophage activation syndrome in patients with systemic onset juvenile idiopathic arthritis. Rheumatology. 2010;49:441–9.

    Article  CAS  PubMed  Google Scholar 

  9. Ghosh S, Carmo M, Calero-Garcia M, Ricciardelli I, Bustamante Ogando JC, Blundell MP, et al. T-cell gene therapy for perforin deficiency corrects cytotoxicity defects and prevents hemophagocytic lymphohistiocytosis manifestations. J Allergy Clin Immunol. 2018;142:904–913.e3.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

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Correspondence to Andrew R. Gennery.

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Verrinder, A.E., Marsden, J.L.S., Slatter, M.A. et al. Late relapse of primary hemophagocytic lymphohistiocytosis after hematopoietic stem cell transplantation: a consequence of low-level chimerism from a carrier donor?. Immunol Res 67, 261–264 (2019). https://doi.org/10.1007/s12026-019-09085-9

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  • DOI: https://doi.org/10.1007/s12026-019-09085-9

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