Hemophagocytic lymphohistiocytosis is associated with deficiencies of cellular cytolysis but normal expression of transcripts relevant to killer-cell-induced apoptosis.

@article{Schneider2002HemophagocyticLI,
  title={Hemophagocytic lymphohistiocytosis is associated with deficiencies of cellular cytolysis but normal expression of transcripts relevant to killer-cell-induced apoptosis.},
  author={Elisabeth Marion Schneider and Ingrid Lorenz and Michaela M{\"u}ller-Rosenberger and Gerald Steinbach and Martina Kron and Gritta E. Janka-Schaub},
  journal={Blood},
  year={2002},
  volume={100 8},
  pages={2891-8}
}
In 65 patients with hemophagocytic lymphohistiocytosis (HLH), we found an as yet undescribed heterogeneity of defects in cellular cytotoxicity when assay conditions were modified by the incubation time, the presence of mitogen, or interleukin-2 (IL-2). The standard 4-hour natural killer (NK) test against K562 targets was negative in all patients. In patients deficient in type 1 (n = 21), type 2 (n = 5), and type 4 (n = 8) HLH, negative NK function could be reconstituted by mitogen, by IL-2, or… CONTINUE READING

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