Scientific Letters
Vol. 18 No. 1 (2026): Mediterranean Journal of Hematology and Infectious Diseases

Allogeneic Hematopoietic Stem Cell Transplantation in Hemophagocytic LymphohistiocytosisTwo Sides of the Locket: Trigger or Cure?

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Published: September 1, 2026
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Allogeneic hematopoietic stem cell transplantation (alloHSCT) represents a paradoxical role in hemophagocytic lymphohistiocytosis (HLH): curative in primary disease yet potentially triggering secondary HLH in the posttransplant setting. We report two adult cases illustrating this duality. The first case developed life-threatening secondary HLH on day +40 following haploidentical alloHSCT, presenting with extreme hyperferritinemia and procalcitonin elevation mimicking septic shock, successfully treated with dexamethasone, ruxolitinib, and plasma exchange. The second case underwent alloHSCT as definitive therapy for familial HLH harboring a UNC13D variant, achieving complete remission despite posttransplant EBV reactivation and lymphoproliferative disorder. These cases highlight the diagnostic challenges and therapeutic complexity of HLH within the transplant continuum.



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Citations

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Ethics Approval

allogeneic hematopoietic stem cell transplantation, Hemophagocytic Lymphohistiocytosis, Ruxolitinib, Endothelial Syndromes, Familial Hemophagocytic Lymphohistiocytosis, Inflammation

Supporting Agencies

No funding was received for this research

How to Cite



“Allogeneic Hematopoietic Stem Cell Transplantation in Hemophagocytic LymphohistiocytosisTwo Sides of the Locket: Trigger or Cure?” (2026) Mediterranean Journal of Hematology and Infectious Diseases, 18(1), p. e2026073. doi:10.4084/MJHID.2026.073.