All patients (n=29)

Malignancy-associated HLH (n=15)

Non malignancy-HLH (n=14)

p value

Mean age (± SD)

58.4 (±18.6)

61.1 (±18.5)

55.6 (±19.0)

0.47

Sex (M) n (%)

8 (53)

6 (43)

0.57

Etiologies of secondary HLH

T cell lymphoma (n=5)

Adult onset Still’s disease (n=2)

Diffuse large B cell lymphoma (n=3)

Psoriasis pustulosa (n=1)

Hodgkin lymphoma (n=2)

Rheumatic arthritis (n=1)

Centroblastic lymphoma (n=1)

SAPHO syndrome (n=1)

Myelodysplastic disorder (n=1)

Granulomatous disease (n=1)

Bladder cancer (n=1)

Unknown recurrent fever (n=1)

Breast cancer (n=1)

No known immunosuppression (n=6)

Liver cancer (n=1)

Signs and symptoms

Fever n (%)

27 (93)

13 (87)

14(100)

> 0.99

Fatigue n (%)

26 (90)

15 (100)

11 (78)

0.21

Lymphadenopathy n (%)

18 (62)

12 (80)

6 (43)

0.039

Splenomegaly n (%)

18 (62)

11 (73)

7 (50)

0.2

Hepatomegaly n (%)

12 (41)

6 (40)

6 (43)

0.88

Weight loss >10% n (%)

11(38)

9 (60)

2 (14)

0.012

Effusion n (%)

9 (31)

5 (33)

4 (29)

> 0.99

Triad cytopenia/SMG/fever n(%)

8 (28)

6 (40)

2 (14)

0.21

Anorexia n (%)

6 (20)

5(33)

1 (7)

0.18

Exanthema n (%)

5 (17)

2 (13)

3 (21)

0.65

Headache n (%)

4 (14)

0 (0)

4 (29)

0.042

Abdominal pain n (%)

3 (10)

1 (7)

2 (14)

0.58

Arthralgia n (%)

2 (7)

0 (0)

2 (14)

0.19