Table 2.
Characteristics of Polish patients with HLH
| n = 54 | sHLH n = 35 |
FHL n = 13 |
XLP1 and XLP2 n = 4 |
XMEN n = 1 | MKD n = 1 |
|
|---|---|---|---|---|---|---|
| Age at diagnosis (year) | ||||||
| Median | 2.87 | 5.56 | 0.24 | 3.75 | 17 | Started as intrauterine presentation |
| Average | 4.7 | 6.6 | 0.2 | 3.42 | ||
| Sd | 4.83 | 4.82 | 0.5 | 1.62 | ||
| Range | 0.01–17.85 | 0.47–17.85 | 0.01–0.82 | 1.25–4.93 | ||
| Sex (M/F) | 30/24 | 18/17 | 7/6 | 4/0 | 1/0 | 0/1 |
| Diagnostic criteria | ||||||
| Fever | 53/53 (100%) | 35/35(100%) | 12/12(100%) | 4/4(100%) | No | Yes |
| Splenomegaly | 51/53 (96%) | 33/35 (94%) | 12/12 (100%) | 4/4 (100%) | Yes | Yes |
| Bicytopenia | 51/53 (96%) | 33/35 (94%) | 12/12 (100%) | 4/4 (100%) | No | Yes |
| Triglycerides > 265 mg/dl | 38/51 (75%) | 25/33 (76%) | 9/12 (75%) | 2/4 (50%) | No | No |
| Fibrinogen < 150 mg/dl | 37/53 (70%) | 25/35 (71%) | 7/12 (58%) | 3/4 (75%) | No | No |
| Ferritin > 500 /dl | 50/53 (94%) | 33/35 (94%) | 12/12 (100%) | 3/4 (75%) | No | Yes |
| NK cell activity low | 28/40 (70%) | 19/26 (76%) | 8/11 (73%) | 0/2 (100%) | Not done | Yes |
| sCD25 > 2400 U/l | 6/6 (100%) | 0/0 | 4/4 (100%) | 1/1 (100%) | Not done | Yes |
| Hemophagocytosis | 39/52 (75%) | 29/34 (85%) | 7/12 (58%) | 1/4 (25%) | No | Yes |
| CNS involvement | 17/51 (33%) | 8/34 (24%) | 6/12 (50%) | 3/3 (100%) | No | No |
| Neurological symptoms | 8/17 | 6/8 | 2/6 | 0/3 | ||
| CSF pleocytosis | 13/17 | 5/8 | 5/6 | 3/3 | ||
| CSF proteinosis | 7/17 | 3/8 | 4/6 | 0/3 | ||
| MRI abnormalities | 7/15 | 4/8 | 2/6 | 1/1 | ||
CSF cerebrospinal fluid, MRI magnetic resonance imaging