Erschienen in:
01.01.2021 | Original Article
Characteristics and predictors of post-transplant-associated hemophagocytic lymphohistiocytosis in adults
verfasst von:
Norimichi Hattori, Misuzu Sato, Yuka Uesugi, Ayaka Nakata, Yohei Sasaki, Shotaro Shimada, Megumi Watanuki, Shun Fujiwara, Yukiko Kawaguchi, Nana Arai, Yui Uto, Tomoharu Matsui, Kouji Yanagisawa, Sachiko Tahara, H Phillip Koeffler, Keiichi Iezumi, Tsuyoshi Nakamaki
Erschienen in:
International Journal of Hematology
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Ausgabe 5/2021
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Abstract
Hemophagocytic lymphohistiocytosis (HLH) is an uncontrolled hyperinflammatory disorder driven by an overactive immune system that results in high mortality. Post-transplant-associated hemophagocytic lymphohistiocytosis (PT-HLH) is a type of secondary HLH that occurs following allogeneic hematopoietic stem cell transplantation (allo-HSCT). The clinical features of PT-HLH remain unclear and diagnostic and prognostic tools have not yet been established. Here, we retrospectively evaluated the clinical manifestations and outcomes of PT-HLH in 94 patients who underwent allo-HSCT. According to our PT-HLH criteria (hyperferritinemia and increased macrophage count in bone marrow), PT-HLH occurred in 12 patients (12.8%). The PT-HLH patients showed splenomegaly (P = .001), a higher risk of engraftment failure (P = .013), and an increased percentage of macrophages and hemophagocytes in bone marrow aspirates (P = .0009 and P = .0006, respectively). Moreover, univariate and multivariate analyses revealed that the survival rate was lower in PT-HLH patients than non-PT-HLH patients (P = .0017 and P = .034, respectively). This study defines the clinical features of PT-HLH and PT-HLH criteria that could be useful tools for diagnosing PT-HLH.