“A Dangerous Black Box:” Idiopathic Hemophagocytic Lymphohistiocytosis in Adult Patients—A Case Report and Review of the Literature

Table 1

Case series and cohorts of HLH in adult patients. In some studies, more than a possible cause for HLH was identified.

Study	Number of patients	Malignancy	Hematological disease	Primary immunodeficiency	Infection	Autoimmune disease	Post-transplant	Other	Idiopathic	30-daymortality	Overall mortality
Ahn et al., 2010	30	4 (13.3%)	—	—	18 (60%)	1 (3.3)	—	—	7 (23.3%)	14 (46.7%)^+	NA
Tseng et al., 2010	96	61 (63.5%)	—	—	32 (33.3%)	3 (3.2%)	—	—	—	NA	60 (63%)
Shabbir et al., 2010	18	4 (22%)	1 (6%)	—	5 (28%)	2 (11%)	3 (16%)	—	3 (17%)	5 (27%)	12 (66.7%)
Park et al., 2012	23	Excluded	—	—	17 (74%)	—	—	—	6 (23%)	NA	17 (73.9%)
Kim et al., 2013	14	14 (42.8%)	—	—	14 (14.3%)	14 (14.3%)	—	—	14 (28.6%)	NA	5 (35.7%)
Parikh et al. 2014	62	32 (52%)			21 (34%)	5 (8%)	—	—	4 (6%)	27 (44%)	41 (66%)
Ramos et al., 2014^++	2197	1047 (47.66%)	—	—	1108 (50.4%)	276 (12.6%)	—	184 (8.4%)	81 (3.7%)	NA	1109 (41%)
Riviere et al., 2014	162	95 (59.9%)	—	—	40 (24.7%)	5 (3.1%)	—	-	14 (8.6%)	68 (42%)	33 (20%)
Barba et al., 2015	71	21 (30%)	—	—	20 (28%)	13 (13%)	—	—	18 (26%)	40 (41%)	68 (70%)
Li et al., 2015	85	23 (27.1%)	—	—	29 (34.1%)	6 (7.1%)	—	—	27 (31.8%)	NA	39 (65%)^+++
Schram et al., 2015	68	33 (49%)	—	—	22 (33%)	19 (28%)	—	—	15 (22%)	14 (21%)	46 (69%)
Oto et al., 2015	34	6 (17.6%)	—	—	4 (11.8%)	3 (8.8%)	—	4 (11.7%)	17 (50%)	NA	11 (32.4%)
Li et al., 2016	103	49 (47.6%)	—	—	24 (23.3)	14 (13.6%)	—	—	24 (23.3%)	NA	77 (74.8%)
Lim et al., 2016	264	170 (64.4%)	—	—	48 (18.2%)	15 (23.4%)	—	—	31 (11.7%)	NA	133 (50.37%)
Yuan et al., 2016	45	28 (62.2%)	—	—	10 (22.2%)	—	—	—	—	NA	NA
Zheng et al., 2016	43	31 (72%)	—	—	3 (7%)	3 (7%)	—	—	6 (14%)	NA	31 (72%)
Arslan et al., 2018	26	3 (11.5%)	—	—	9 (34.6%)	4(15.4%)	—	—	10 (38.5%)	NA	13 (50%)
Brito-Zeron et al., 2018	151	48 (31.8%)	—	—	32 (21.2%)	5 (35.1%)	8 (5.3%)	42 (27.8%)	42 (27.8%)	NA	80 (52.9%)
Kapoor et al., 2018	16	2 (13%); ^+infection 5/16 (31%)	—	—	5 (31%)	—	—	—	4 (25%)	6 (37%)	31(81%)
Miao et al., 2018	112	66 (58.9%)	—	—	23 (20.5%)	4 (3.6%)	—	—	19 (17%)	NA	NA
Qiaolei et al., 2018	174	—	92 (52.9%)	—	57 (32%); 33 (18.9%) ^+infection	6 (3.4%)	—	—	24 (13.8%)	NA	102 (58.6%)
Zhou et al., 2018	205	119 (58%)	—	—	83 (40.5%)	14 (6.8%)	—	—	14 (6.8%)	89 (43.3%)	NA
Kumar et al. 2019	12	—	—	—	4 (33.3%)	7 (58.3%)	—	—	1 (8.3%)	NA	NA
Jumic et al., 2019	41	16 (54%)	—	—	22 (41%)	8 (19%)	9 (22%)	—	—	NA	16 (39%)
Birndt et al., 2020	137	48 (35%)	—	—	61 (44.5%)	13 (9.5%)	—		15 (10.9%)	27 (20.6%)	67 (51%)
Diack et al., 2020	26	11 (42.3%)	—	1 (3.8%)	12 (46.1%)	6 (23%)	—	—	2 (7.6%)	NA	19 (73%)
Pandey et al., 2020	41	15 (37%)	—	—	31 (76%)	9 (22%)	—	—	—	NA	22 (54%)
Bichon et al., 2021	260	28 (11%)	205 (79%)	—	—	—	—	—	27 (10%)	NA	147 (75%)

⁺For 2 patients, the outcome is not available; ⁺⁺Meta-analysis, 24 studies published between Jan 1, 1974, and Sept 29, 2011, none of them separately listed in our table; ⁺⁺⁺39 deaths among 60 patients followed-up.