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Table 1 Hemophagocytic lymphohistiocytosis (HLH) 2004 diagnostic criteria (Adapted with permission from John Wiley and Sons Ltd. from Brisse et al. [3] and Henter et al. [5]). Diagnostic criteria 1–5 represent the original 1991 diagnostic criteria; the HLH 2004 revision adds criteria 6–8. The diagnosis of primary HLH can be made either on a molecular or clinical basis

From: Secondary hemophagocytic lymphohistiocytosis in the setting of metastatic renal cell carcinoma: a case report

HLH Molecular diagnosis At least 5 of the following 8 diagnostic criteria
FHL genes PRF1, UNC13D, STX11, STXBP2 1. Fever 2. Splenomegaly
3. Cytopenias (≥2/3 lineages)a 4. High triglyceride/low fibrinogen levelsb
Non-FHL genes RAB27A, LYST, AP3B1, SH2D1A, XIAP 5. Hemophagocytosis 6. Low/absent NK-cell activity
7. Ferritin ≥500 ng/mL 8. Soluble IL-2 receptor ≥2400 U/mL
  1. FHL Familial hemophagocytic lymphohistocytosis, NK Natural Killer, and IL Interleukin. non-FHL gene mutations occur in Gricselli syndrome, Chediak-Higashi syndrome, Hermansky-Pudlak syndrome type 2, and X-linked lymphoproliferative disease
  2. aHb <9 g/dL, platelets <100 K/mcL, neutrophils <1.0 K/mcL. bTriglycerides ≥265 mg/dL, fibrinogen ≤150 mg/dL