Efficient management of secondary haemophagocytic lymphohistiocytosis with intravenous steroids and γ-immunoglobulin infusions

Table 1 Characteristics of the 80 adult patients with secondary haemophagocytic lymphohistiocytosis

Characteristic	n (%)
Clinical symptoms
Fever	75 (94)
Respiratory symptoms	24 (30)
Skin rash	20 (25)
Gastrointestinal symptoms	8 (10)
CNS symptoms1	7 (9)
Physical findings
Splenomegaly	47 (59)
Hepatomegaly	47 (59)
Lymphadenopathy	37 (46)
Laboratory findings
Elevated ferritin (≥ 500 ng/mL)	76 (95)
(≥ 10000 ng/mL)	20 (25)
Elevated inflammatory markers2	69 (86)
Complete blood count
Anaemia	58 (72)
Thrombocytopenia	57 (71)
Leucopoenia/neutropenia	38 (47)/9 (11)
Pancytopenia	24 (30)
Elevated LFTs	70 (87)
Elevated LDH	61 (76)
High triglycerides	55 (70)
Low fibrinogen levels	18 (22)
DIC	9 (11)

¹Disturbance of consciousness, headache/dizziness.

²C-reactive protein and erythrocyte sedimentation rate. CNS: Central nervous system; LFTs: Liver function tests; LDH: Lactate dehydrogenase; DIC: Disseminated intravascular coagulation.