Signs and symptoms of HLH
How does HLH present?
Hemophagocytic lymphohistiocytosis (HLH) is a heterogeneous syndrome with a range of nonspecific signs and symptoms. This can make diagnosis a challenge, as many symptoms can overlap with more common conditions like infections, malignancy, and rheumatologic diseases.1,2
There are also a variety of laboratory abnormalities associated with HLH that should increase your suspicion in cases of severe hyperinflammation.2
See below for more information about some of the commonly observed signs and symptoms of HLH.
References: 1. Jordan MB, Allen CE, Weitzman S, Filipovich AH, McClain KL. How I treat hemophagocytic lymphohistiocytosis. Blood. 2011;118(15):4041-4052. doi:10.1182/blood-2011-03-278127 2. George MR. Hemophagocytic lymphohistiocytosis: review of etiologies and management. J Blood Med. 2014;5:69-86. doi:10.2147/JBM.S46255 3. Price B, Lines J, Lewis D, Holland N. Haemophagocytic lymphohistiocytosis: a fulminant syndrome associated with multiorgan failure and high mortality that frequently masquerades as sepsis and shock. S Afr Med J. 2014;104(6):401-406. doi:10.7196/samj.7810 4. Marsh RA, Haddad E. How I treat primary haemophagocytic lymphohistiocytosis. Br J Haematol. 2018;182(2):185-199. doi:10.1111/bjh.15274 5. Jordan MB, Allen CE, Greenberg J, et al. Challenges in the diagnosis of hemophagocytic lymphohistiocytosis: recommendations from the North American Consortium for Histiocytosis (NACHO). Pediatr Blood Cancer. 2019;66(11):e27929. doi:10.1002/pbc.27929 6. Machowicz R, Janka G, Wiktor-Jedrzejczak W. Similar but not the same: differential diagnosis of HLH and sepsis. Crit Rev Oncol Hematol. 2017;114:1-12. doi:10.1016/j.critrevonc.2017.03.023 7. Valade S, Joly BS, Veyradier A, et al. Coagulation disorders in patients with severe hemophagocytic lymphohistiocytosis. PLoS One. 2021;16(8):e0251216. doi:10.1371/journal.pone.0251216
