Table.2 Treatment and outcome of patients between 0 and 18 years of age diagnosed with HLH in FVL between 2012 and 2019

1

FHLH (LYST mutation)

EBV infection

5

X

No

No

Yes

2

FHLH (UNCD13 mutation)

EBV infection

3

X

Yes

Acute liver failure, CNS compromise

Yes

3

FHLH (XIAP mutation)

CMV infection

0.17

X

Yes

Severe dehydration secondary to diarrhea

Yes

4

Suspected FHLH, no genetic confirmation

0.75

X

Yes

CNS compromise

Yes

5

EBV infection (IgM)

3

X

No

No

Yes

6

EBV infection (Persistently elevated viral load)

13

X^a

No

Respiratory and renal failure

No

7

EBV infection (Elevated initial viral load)^b

13

No

No

Yes

8

EBV infection (Elevadted viral load)

8

X

No

No

Yes

9

EBV infection (Persistently Elevated viral load)

13

X

No

Respiratory failure

No

10

EBV Infection (elevated viral load) + JIA diagnosis

15

X

No

No

Yes

11

EBV Infection (biopsy of ganglion) + JIA diagnosis

9

X

Yes

No

Yes

12

Dengue (positive IgM) + EBV (Elevated viral load)

11

X

No

No

Yes

13

Dengue (positive IgM)

0.33

X

No

No

Yes

14

Dengue (positive IgM)

11

X

No

No

Yes

15

CMV (positive viral load) + EBV (Positive viral load) + JIA diagnosis

6

X

No

No

Yes

16

Abdominal Mycobacterium tuberculosis infection

15

X

No

Respiratory, renal and hepatic failure

Yes

17

SLE

14

X

No

No

Yes

18

Langerhans Histiocytosis + Pseudomona aeruginosa and Klebsiella pneumoniae Bacteremia

1

X

No

No

Yes

19

Suspected Autoimmunity, diagnosis pending

11

X

No

No

Yes

20

Unknown

1

X^c

No

Respiratory, renal and hepatic failure

No

21

Unknown

16

X

No

No

Yes