Familial Hemophagocytic Lymphohistiocytosis Secondary to PRF1 Mutation

Table 1

An overview of relevant laboratory parameters during the clinical course of the patient.

Parameters	Reference range	Admission Day 0	Day 5	Day 1 of HLH-04 protocol	Day 5 of HLH-04 protocol	Day 10 of HLH-04 protocol	Day 15 of HLH-04 protocol	Day 20 of HLH-04 protocol	Day of discharge
Hemoglobin (g/-dL)	13.0–18.0	8.7	7.8	10.8	10.2	9.7	8.2	8.5	9.5
Neutrophils (10^9xL)	2.0–7.5	0.8	0.99	0.75	0.92	1.02	0.79	0.42	3.44
Lymphocytes (10^9/L)	1.5–4.0	1.61	1.07	1.44	2.3	0.48	0.41	0.84	2.16
Platelet count (10^9/L)	150–450	63	54	41	87	68	69	221	273
Ferritin (μg/L)	11–172	1420	1912	948	1821	1612	976	881	371
Fibrinogen (g/L)	2.0–4.0	—	1.5	1.6	0.9	1.2	1.4	1.6	1.9
Triglyceride (mmol/L)	0.5–2.20	3.13	—	—	—	—	—	—	—
Direct bilirubin (μmol/L)	1.7–6.7	20	80	—	—	—	—	—	34
ALT (IU/L)	9–24	502	707	611	323	303	257	274	128
AST (IU/L)	14–35	354	608	390	189	113	79	89	63
ALP (IU/L)	59–164	994	885	967	631	413	305	309	253
GGT (IU/L)	7–21	250	219	569	424	535	647	782	1025
sIL-2r (U/ml)	158–623	—	—	—	15900	—	—	—	—