Frontiers in Oncology (Oct 2022)

Hemophagocytic lymphohistiocytosis as an etiology of bone marrow failure

  • Jonathan Paolino,
  • Nancy Berliner,
  • Barbara Degar

DOI
https://doi.org/10.3389/fonc.2022.1016318
Journal volume & issue
Vol. 12

Abstract

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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.

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