Successful treatment of adult-onset still disease caused by pulmonary infection-associated hemophagocytic lymphohistiocytosis: A case report

Table 1 Laboratory data upon hospital admission

Laboratory parameter (measure)	Reference range	Before admission	Upon admission	During treatment	After treatment
WBC (109/L)	4-10	34.8	49.82	2.82	7.86
Neutrophil count (109/L)	2-7	-	121	1.9	4.2
NE (%)	50-70	96.89	88.0	68.1	53.3
Hb (g/L)	120-160	127	121	98	106
PLT (109/L)	100-300	329	374	144	309
CRP (mg/L)	0-8	183	204	83.3	1.9
ESR (mm/L)	0-15	58	66	24	5
PCT (ng/mL)	0-0.49	-	21.94	0.32	-
Fibrinogen (g/L)	2.0-4.0	-	4.87	-	2.06
SF (ng/mL)	30-400	-	42703	> 2000	764.4
TG	0.57-1.7	-	1.43	2.6	1.34
LDH (U/L)	114-240	-	458	637	151
ALT (U/L)	0-40	-	71	126	23
AST (U/L)	0-40	-	56	82	13
NK cells (%)	9.2-21.5	-	20.9	6.4	15.9
ANA	Negative	-	Negative	-	-
RF (IU/mL)	Negative	-	Negative	-	Negative

WBC: White blood cell; NE: Neutrophil percentage; Hb: Hemoglobin; PLT: Platelet; CRP: C-reactive protein; ESR: Erythrocyte sedimentation rate; PCT: Procalcitonin; SF: Serum ferritin; TG: Triglyceride; LDH: Lactate dehydrogenase; ALT: Alanine aminotransferase; AST: Aspartate aminotransferase; NK: Natural killer; ANA: Antinuclear antibody; RF: Rheumatoid factor.

Table 2 Diagnostic criteria for hemophagocytic lymphohistiocytosis: HLH-2004 - revised diagnostic guidelines for HLH10

Diagnosis of HLH can be established if one of the two criteria below are met
1 A molecular diagnosis consistent with HLH (i.e. reported mutations found in either PRF1 or MUNC13-4), or
2 Diagnostic criteria for HLH are fulfilled (i.e. at least five of the eight criteria listed below are present)
(A) Persistent fever
(B) Splenomegaly
(C) Cytopenia (affecting ≥ 2 of 3 lineages in the peripheral blood)
(i) Hemoglobin < 90g/L (in infants < 4 wk: < 100g/L)
(ii) Platelets < 100 × 109/L
(iii) Neutrophils < 1.0 × 109/L
(D) Hypertriglyceremia and/or hypofibrinogenemia
(i) Fasting triglycerides ≥ 3.0 mmol/L (i.e. ≥ 265 mg/dL)
(ii) Fibrinogen ≤ 1.5g/L
(E) Hemophagocytosis in bone marrow, spleen or lymph nodes, no evidence of malignancy
(F) Serum ferritin ≥ 500 µg/L (i.e. 500 ng/mL)
(G) Low or absent natural killer cell activity (according to local laboratory reference)
(H) Increased serum sIL2Rα (according to local laboratory reference)

HLH: Hemophagocytic lymphohistiocytosis.