Posttransplant Hemophagocytic Lymphohistiocytosis in Pediatric Liver Transplant Recipients

Abstract

Hemophagocytic lymphohistiocytosis (HLH) refers to the life-threatening syndrome of pathologic immune activation, with a characteristic constellation of clinical findings comprising prolonged fever, splenomegaly, cytopenias, elevated inflammatory markers, and hemophagocytosis (HP). ( 1) HLH has been reported to occur after solid organ transplantation. (2) However, data are scarce regarding the incidence, characteristics, and long-term outcomes of HLH in the post-liver transplantation (LT) setting, with only 5 pediatric and 18 adult cases reported in the literature. (3)(4)(5)(6)(7) In this report, we describe the clinical, laboratory, and pathologic characteristics as well as the treatment approach, mortality rate, and long-term outcomes in posttransplant HLH.

Hemophagocytic lymphohistiocytosis occurring after liver transplantation: A case series and review of the literature

“…There do not appear to be clear differences in presentation between HLH presenting in the context of LT or without LT. Fever, hepatosplenomegaly, cytopenias, elevated liver enzymes, and high LDH are common to both transplant and non-transplant related HLH. 19,20 The most frequent infectious trigger for HLH, in both post-LT and non-LT cases, is viral infection. 1 Herpesviruses such as EBV, CMV, and HHV-8 account for up to 62% of cases (Table 2).…”

“…The overall mortality rate was only 25%, which is lower than in previous reports. 19 Finally, while patients with HLH can present with secondary ALF, LT is contraindicated in this setting as this will not reverse the underlying systemic inflammation and HLH will progress despite LT. 28 In conclusion, post-LT HLH is a rare condition associated with high mortality. While most reported cases have occurred early following LT, there is a risk for its development in patients who are many years removed from transplant, so increased vigilance is necessary.…”

Graft-versus-host disease after liver transplantation is associated with bone marrow failure, hemophagocytosis, and DNMT3A mutations

“…4 The present study highlights the pathophysiological importance of hemophagocytosis in the bone marrow in GVHD after solid organ transplantation. While hemophagocytic lymphohistiocytosis (HLH) has been described after kidney transplant, 36,37 and linked to viral infections (e.g., EBV, cytomegalovirus) after LT in case reports, [38][39][40][41] the association of HLH features with BMF and GVHD has not been reported after LT. Importantly, we found increased numbers of hemophagocytes in the bone marrow aspirate slides of all but one evaluable patient, and we suggest that hemophagocytosis may be a reliable feature of GVHD after LT and might even prove to be an early indicator.…”