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My jamais vu in post allogeneic hematopoietic cell transplant: a review on secondary hemophagocytosis in adults

Bone Marrow Transplantation volume 55, pages 867–872 (2020)Cite this article

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Abstract

Post allogenic hematopoietic cell transplant (HCT) hemophagocytic lymphohistiocytosis (HLH) is an aggressive disease with unknown etiology. It has a poorly understood pathophysiology and poor outcome if untreated early. It’s a state of hypercytokinemia. There are many proposed diagnostic criteria for Post HCT HLH. It usually occurs early in the first 2–6 weeks after allogeneic HCT but can present late. The incidence is highest among cord blood transplant compared with other sources of stem cells with a higher incidence in HLA mismatch donors. Post HCT HLH has a marked low survival rate, when compared with Non-HLH post HCT patients and specifically poor outcome is associated in patients with liver dysfunction, graft failure, and those with endothelial complications. Steroid is the mainstay treatment which can be followed up by cyclosporine and etoposide though an optimal therapy is not known. Intravenous immunoglobulin (IVIg) has been tried in virus associated HLH. Second bone marrow transplant is a rescue procedure in patient with HLH due to graft failure, though a very careful selection of individual patients is mandatory. It has been recently found that etoposide based conditioning regimen may reduce HLH post HCT. A prospective study on post HCT HLH are needed to evaluate this unrecognized condition.

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Authors and Affiliations

  1. Allogeneic Blood and Marrow Transplant Program, Department of Medical Oncology and Hematology, Princess Margaret Cancer Centre, University Health Network, Toronto, ON, Canada

    Rehab M. Alblooshi, Fotios V. Michelis, Santhosh Thyagu, Dennis (Dong Hwan) Kim, Jeffrey H. Lipton & Auro Viswabandya

  2. Dubai Hospital, Dubai Health Authority, Dubai, United Arab Emirates

    Rehab M. Alblooshi

  3. Blood and Marrow Transplant Program, Division of Hematology, London Health Sciences Centre, London, ON, Canada

    Uday Deotare

  4. Faculty of Medicine Ain Shams University, Cairo, Egypt

    Ghada M. ElGohary

  5. King Khaled University Hospitals College of Medicine, Riyadh, Saudi Arabia

    Ghada M. ElGohary

Authors
  1. Rehab M. Alblooshi
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  2. Uday Deotare
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  8. Auro Viswabandya
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Contributions

RMA, UD, and AV were involved with the review concepts, analysis, interpretation, drafting and revision of the article, and the responsibility for the content of the paper. FVM, ST, DK, JHL, and GME made substantial contributions to data generation, revision of the article critically for important intellectual content, and provided final approval of the version published.

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Correspondence to Auro Viswabandya.

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Alblooshi, R.M., Deotare, U., Michelis, F.V. et al. My jamais vu in post allogeneic hematopoietic cell transplant: a review on secondary hemophagocytosis in adults. Bone Marrow Transplant 55, 867–872 (2020). https://doi.org/10.1038/s41409-019-0711-1

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  • DOI: https://doi.org/10.1038/s41409-019-0711-1

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