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Hemophagocytic lymphohistiocytosis as an etiology of bone marrow failure

Jonathan Paolino1, Nancy Berliner2, Barbara Degar1

  • 1Department of Pediatric Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, United States.

Frontiers in Oncology
|November 17, 2022
PubMed

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of multiorgan system dysfunction that is caused by hypercytokinemia and persistent activation of cytotoxic T lymphocytes and macrophages. A nearly ubiquitous finding and a diagnostic criterion of HLH is the presence of cytopenias in ≥ 2 cell lines. The mechanism of cytopenias in HLH is multifactorial but appears to be predominantly driven by suppression of hematopoiesis by pro-inflammatory cytokines and, to some extent, by consumptive hemophagocytosis. Recognition of cytopenias as a manifestation of HLH is an important consideration for patients with bone marrow failure of unclear etiology.

Keywords:
bone marrow failure (BMF)cytopeniafamilial hemophagocytic lymphohistiocytosis (FHL)hemophagocytic lymphohistiocytosishypercytokinaemiamacrophage activation syndrome (MAS)

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