The interferon-gamma pathway is selectively up-regulated in the liver of patients with secondary hemophagocytic lymphohistiocytosis
Fig 6
The activation of the IFNγ pathway is up-regulated in PBMCs of P1 collected during the active phase of sHLH.
A) Tyr(701)-phosphorylated STAT1 and total STAT1 protein levels were assessed by Western Blot analyses in PBMC lysates collected from P1 during the course of the disease (the day of liver biopsy collection was fixed as day 0). A sample collected 6 months before the sHLH episode, while the patient had inactive disease (ID), was also analyzed. pSTAT1/GAPDH and STAT1/GAPDH relative density values are shown above the blots. B) The Type II IFN score, the Type I IFN score and the inflammatory cytokine score obtained in PBMCs was calculated. C) CXCL9 mRNA levels in PBMCs and CXCL9 plasma levels have been measured. D) Ferritin and ALT/GPT levels during the course of the disease were reported. The area in grey represents the period in which the P1 was under treatment with glucocorticoids. Not available (NA).