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Hemophagocytic syndrome after hematopoietic stem cell transplantation: a prospective observational study

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Abstract

The aim of this prospective observational study was to evaluate the incidence of hemophagocytic syndrome (HPS) after hematopoietic stem cell transplantation (HSCT). Between July 2006 and December 2007, all patients who received a HSCT in our institution were included in this study. All the following criteria were needed for the diagnosis of HPS: sustained fever over 7 days; cytopenia (neutropenia and/or thrombocytopenia); presence of more than 3% mature macrophages in bone marrow; hyperferritinaemia (>1,000 ng/mL). During this study, 171 patients received a HSCT (68 allogeneic and 103 autologous). The median age was 32 years (3–62). We observed six cases of HPS (6/68; 8.8%) after allogeneic stem cell transplantation (ASCT): one case of EBV-related HPS, two cases of CMV-related HPS, and three cases with no evidence of bacterial, fungal or viral infections. We observed only one case of CMV-related HPS (1/103; 0.9%) after autologous stem cell transplantation. Four patients died despite aggressive supportive care. To our knowledge, this is the first prospective observational study conducted with the aim to evaluate the incidence of HPS after HSCT. This study provides a relatively high incidence of HPS after ASCT. When sustained fever with progressive cytopenia and hyperferritinaemia are observed, HPS should be suspected, and bone marrow aspirate considered. The rapid diagnosis of HPS and the early initiation of an appropriate treatment are essential for patient management.

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References

  1. Henter JI, Elinder G, Ost A. Diagnostic guidelines for hemophagocytic lymphohistiocytosis. The FHL Study Group of the Histiocyte Society. Semin Oncol. 1991;18:29–33.

    CAS  PubMed  Google Scholar 

  2. Larroche C, Mouthon L. Pathogenesis of hemophagocytic syndrome (HPS). Autoimmun Rev. 2004;3:69–75. doi:10.1016/S1568-9972(03)00091-0.

    Article  CAS  PubMed  Google Scholar 

  3. Stepp SE, Dufourcq-Lagelouse R, Le Deist R, et al. Perforin gene defects in familial hemophagocytic lymphohistiocytosis. Science. 1999;286:1957–9. doi:10.1126/science.286.5446.1957.

    Article  CAS  PubMed  Google Scholar 

  4. Janka G, Imashuku S, Elinder G, et al. Infection and malignancy-associated hemophagocytic syndromes. Secondary hemophagocytic lymphohistiocytosis. Hematol Oncol Clin North Am. 1998;12:435–44. doi:10.1016/S0889-8588(05)70521-9.

    Article  CAS  PubMed  Google Scholar 

  5. Imashuku S. Differential diagnosis of hemophagocytic syndrome, underlying disorders and selection of the most effective treatment. Int J Hematol. 1997;66:135–51. doi:10.1016/S0925-5710(97)00584-7.

    Article  CAS  PubMed  Google Scholar 

  6. Marr KA, Seidel K, Slavin MA, et al. Prolonged fluconazole prophylaxis is associated with persistent protection against candidiasis-related death in allogeneic marrow transplant recipients: long-term follow-up, of a randomized, placebo-controlled trial. Blood. 2000;96:2055–61.

    CAS  PubMed  Google Scholar 

  7. Sato M, Matsushima T, Takada S, et al. Fulminant CMV-associated, haemophagocytic syndrome following unrelated bone marrow transplantation. Bone Marrow Transplant. 1998;22:1219–22. doi:10.1038/sj.bmt.1701501.

    Article  CAS  PubMed  Google Scholar 

  8. Ishikawa J, Maeda T, Miyazaki T, et al. Early onset of hemophagocytic syndrome following allogeneic bone marrow transplantation. Int J Hematol. 2000;72:243–6.

    CAS  PubMed  Google Scholar 

  9. Abe Y, Choi I, Hara K, et al. Hemophagocytic syndrome: a rare complication of allogeneic nonmyeloablative hematopoietic stem cell transplantation. Bone Marrow Transplant. 2002;29:799–801. doi:10.1038/sj.bmt.1703554.

    Article  CAS  PubMed  Google Scholar 

  10. Iyama S, Matsunaga T, Fujimi A, et al. Successful treatment with oral ribavirin of adenovirus-associated hemophagocytic syndrome in a stem cell transplantation recipient. Rinsho Ketsueki. 2005;46:363–7.

    PubMed  Google Scholar 

  11. Abdelkefi A, Ben Othman T, Torjman L, et al. Plasmodium falciparum causing hemophagocytic syndrome after allogeneic blood stem cell transplantation. Hematol J. 2004;5:449–50. doi:10.1038/sj.thj.6200531.

    Article  PubMed  Google Scholar 

  12. Reardon DA, Roskos R, Hanson Ca, et al. Virus-associated hemophagocytic syndrome following bone marrow transplantation. Am J Pediatr Hematol Oncol. 1991;13:305–9. doi:10.1097/00043426-199123000-00010.

    Article  CAS  PubMed  Google Scholar 

  13. Tsuda H. Hemophagocytic syndrome in children and adults. Int J Hematol. 1997;65:215–26. doi:10.1016/S0925-5710(96)00560-9.

    Article  CAS  PubMed  Google Scholar 

  14. Henter JI, Horne A, Aricó M, et al. HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007;48:124–31. doi:10.1002/pbc.21039.

    Article  PubMed  Google Scholar 

  15. Spitzer TS. Engraftment syndrome following hematopoietic stem cell transplantation. Bone Marrow Transplant. 2001;27:893–8. doi:10.1038/sj.bmt.1703015.

    Article  CAS  PubMed  Google Scholar 

  16. Tanaka T, Matsubara H, Adachi S, Chang H, Fujino H, Higashi Y, et al. Second transplantation from HLA 2-locimismatched mother for graft failure due to hemophagocytic syndrome after cord blood transplantation. Int J Hematol. 2004;80:467–9. doi:10.1532/IJH97.04063.

    Article  PubMed  Google Scholar 

  17. Sreedharan A, Bowyer S, Wallace CA, et al. Macrophage activation syndrome and other systemic inflammatory conditions after BMT. Bone Marrow Transplant. 2006;37:629–34. doi:10.1038/sj.bmt.1705305.

    Article  CAS  PubMed  Google Scholar 

  18. Allen CE, Yu X, Kozinetz CA, MuClain KL. Highly elevated ferritin levels and the diagnosis of hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2008;50:1227–35. doi:10.1002/pbc.21423.

    Article  PubMed  Google Scholar 

  19. Esumi N, Ikushima S, Hibi S, Todo S, Imashuku S. High serum ferritin level as a marker of malignant histiocytosis and virus-associated hemophagocytic syndrome. Cancer. 1988;61:2071–6. doi:10.1002/1097-0142(19880515)61:10<2071::AID-CNCR2820611023>3.0.CO;2-6.

    Article  CAS  PubMed  Google Scholar 

  20. Emmenegger U, Reimers A, Frey U, et al. Reactive macrophage activation syndrome: a simple screening strategy and its potential in early treatment initiation. Swiss Med Wkly. 2002;132:230–6.

    CAS  PubMed  Google Scholar 

  21. Schaer DJ, Schleiffenbaum B, Kurrer M, et al. Soluble hemoglobin-haptoglobin scavenger receptor CD163 as a lineage-specific marker in the reactive hemophagocytic syndrome. Eur J Haematol. 2005;74:6–10. doi:10.1111/j.1600-0609.2004.00318.x.

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Centre National de Greffe de Moelle Osseuse, Rue Jebel Lakhdar, 1006, Bab Saadoun, Tunis, Tunisia

    Abderrahman Abdelkefi, Wassim Ben Jamil, Lamia Torjman, Saloua Ladeb, Habib Ksouri, Amel Lakhal, Assia Ben Hassen, Abdeladhim Ben Abdeladhim & Tarek Ben Othman

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  1. Abderrahman Abdelkefi
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  2. Wassim Ben Jamil
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  3. Lamia Torjman
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  4. Saloua Ladeb
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  5. Habib Ksouri
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  6. Amel Lakhal
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  7. Assia Ben Hassen
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  8. Abdeladhim Ben Abdeladhim
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  9. Tarek Ben Othman
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Correspondence to Abderrahman Abdelkefi.

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Abdelkefi, A., Jamil, W.B., Torjman, L. et al. Hemophagocytic syndrome after hematopoietic stem cell transplantation: a prospective observational study. Int J Hematol 89, 368–373 (2009). https://doi.org/10.1007/s12185-009-0261-1

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  • DOI: https://doi.org/10.1007/s12185-009-0261-1

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