Haemophagocytic lymphohistiocytosis following allogeneic hematopoietic cell transplantation from mismatched unrelated donors associated with low CD34 and CD3 cell counts in the graft

Gower, N. D.; Coiteux, Valérie; Srour, Micha; Magro, Léonardo; Chauvet, Paul; Terriou, Louis; Ballot, Caroline; Yakoub-Agha, Ibrahim; Beauvais, David

doi:10.1038/s41409-022-01585-x

Cited by 1 publication

(1 citation statement)

References 13 publications

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“…Secondary HLH has also been reported in a small number of patients following allogeneic HCT [ 19 , 116 , 117 , 118 ], with an estimated incidence of 1% post-allogeneic HCT (0.15% for autologous HCT) [ 119 ]. Gower et al reported findings consistent with HLH in 6.8% of recipients of 9/10 HLA-mismatched unrelated donors, with all HLH cases in recipients of low CD34 + count bone marrow grafts and none with peripheral blood stem cell grafts [ 120 ]. HLH was also previously reported to occur with relatively high incidence in adults receiving umbilical cord blood grafts, again with a finding that low CD34 + stem cell dose was a significant risk factor [ 121 ].…”

Section: Special Populations and Treatment-related Hlhmentioning

confidence: 99%

Diagnosis and Management of Adult Malignancy-Associated Hemophagocytic Lymphohistiocytosis

Lee

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2023

Cancers

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Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of severe, dysregulated inflammation driven by the inability of T cells to clear an antigenic target. When associated with malignancy (mHLH), the HLH syndrome is typically associated with extremely poor survival. Here, we review the diagnosis of secondary HLH (sHLH) syndromes in adults, with emphasis on the appropriate workup and treatment of mHLH. At present, the management of HLH in adults, including most forms of mHLH, is based on the use of corticosteroids and etoposide following the HLH-94 regimen. In some cases, this therapeutic approach may be cohesively incorporated into malignancy-directed therapy, while in other cases, the decision about whether to treat HLH prior to initiating other therapies may be more complicated. Recent studies exploring the efficacy of other agents in HLH, in particular ruxolitinib, offer hope for better outcomes in the management of mHLH. Considerations for the management of lymphoma-associated mHLH, as well as other forms of mHLH and immunotherapy treatment-related HLH, are discussed.

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