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

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

HLH Molecular diagnosis		At least 5 of the following 8 diagnostic criteria
FHL genes	PRF1, UNC13D, STX11, STXBP2	1. Fever	2. Splenomegaly
FHL genes	PRF1, UNC13D, STX11, STXBP2	3. Cytopenias (≥2/3 lineages)^a	4. High triglyceride/low fibrinogen levels^b
Non-FHL genes	RAB27A, LYST, AP3B1, SH2D1A, XIAP	5. Hemophagocytosis	6. Low/absent NK-cell activity
Non-FHL genes	RAB27A, LYST, AP3B1, SH2D1A, XIAP	7. Ferritin ≥500 ng/mL	8. Soluble IL-2 receptor ≥2400 U/mL

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
^aHb <9 g/dL, platelets <100 K/mcL, neutrophils <1.0 K/mcL. ^bTriglycerides ≥265 mg/dL, fibrinogen ≤150 mg/dL

ISSN: 1752-1947