Table 2 : Proposed HLH diagnostic criteria, 2009.


1. Molecular diagnosis of hemophagocytic lymphohistiocytosis
    (HLH) or X-linked lymphoproliferative syndrome (XLP).
-

2. Or at least 3 of 4:  

a. Fever +
b. Splenomegaly +
c. Cytopenias (minimum 2 cell lines reduced) +
d. Hepatitis -

3. And at least 1 of 4:  

a. Hemophagocytosis +
b.↑Ferritin +
c.↑sIL2Rα (age based) --
d. Absent or very decreased NK function --

4. Other results supportive of HLH diagnosis:  

a. Hypertriglyceridemia +
b. Hypofibrinogenemia +
c. Hyponatremia +

Vrotsos et al.Journal of Cancer Therapeutics and Research  2014 3:7DOI : 10.7243/2049-7962-3-7