Table 2.
Patient characteristics in history, clinical findings, and laboratory parameters before start of therapy in all patients
| Patient subgroup | All patients (N = 249) | (1) Familial patients (n = 60) | (2) Dead < 8 wk (n = 35) | (3) HSCT (n = 124) | (4) Off therapy, no HSCT (n = 49) |
|---|---|---|---|---|---|
| General | |||||
| Mean/median age, mo (range) | 24/8 (0-184) | 16/2 (0-145) (↓*) | 23/4 (0-137) | 15/4 (0-150) (↓*) | 51/24 (2-184) (↑*) |
| Male sex, % | 137, 55 | 35, 58 | 20, 57 | 73, 59 | 19, 39 (↓†) |
| Familial disease, % | 60/247, 24 | 60, 100 | 5/34, 15 | 44/123, 36 (↑*) | 0 (↓*) |
| Consanguinity, % | 50/240, 21 | 18/56, 32 (↑†) | 12/34, 35 (↑†) | 26/119, 22 | 3/48, 6 (↓‡) |
| Infectious history, % | 109/244, 45 | 19/58, 33 (↓†) | 15, 43 | 47/120, 39 | 25/48, 52 |
| Clinical parameters | |||||
| Hepatomegaly, % | 237, 95 | 2, 97 | 34, 97 | 119, 96 | 43, 88 (↓‡) |
| Lymphadenopathy, % | 80/244, 33 | 12, 20 (↓†) | 10/34, 29 | 36/122, 30 | 22, 45 (↑†) |
| Edema, % | 97/243, 40 | 24/59, 41 | 21/34, 62 (↑‡) | 36/121, 30 (↓‡) | 21, 43 |
| Jaundice, % | 84/244, 34 | 21/58, 36 | 20/34, 59 (↑‡) | 38/121, 31 | 14, 29 |
| Rash, % | 77/246, 31 | 20/59, 34 | 9/34, 26 | 40/122, 33 | 19, 39 |
| Neurological symptoms, % | 80/245, 33 | 23, 38 | 10/33, 30 | 43, 35 | 8, 16 (↓‡) |
| MRI or CT of brain pathological, % | 43/135, 32 | 5/26, 19 | 6/10, 60 (↑†) | 22/75, 29 | 6/28, 21 |
| Laboratory findings | |||||
| ↑ Ferritin, % | 198/212, 93 | 41/48, 85 (↓†) | 26/29, 90 | 96/102, 94 | 44/47, 94 |
| ↑ LDH, % | 182/209, 87 | 41/48, 85 | 29/31, 94 | 81/100, 81 (↓†) | 42/44, 95 |
| ↑ SGOT, % | 164/216, 76 | 37/53, 70 | 25/30, 71 | 80/107, 75 | 38/45, 84 |
| ↑ SGPT, % | 181/237, 76 | 41/55, 75 | 27/34, 79 | 88/118, 75 | 41/47, 87 |
| ↑ Bilirubin, % | 123/239, 51 | 32/57, 56 | 23/34, 68 (↑†) | 56/117, 48 | 23/48, 48 |
| ↓ Albumin, % | 154/223, 69 | 33/52, 63 | 25/33, 76 | 64/105, 61 (↓†) | 36/46, 78 |
| Prolonged APTT, % | 127/227, 56 | 29/54, 54 | 21/32, 66 | 56/109, 51 | 29/48, 60 |
| ↓ Sodium, % | 119/234, 51 | 31/57, 54 | 13/33, 39 | 53/115, 46 | 33/47, 70 (↑‡) |
| ↑ Creatinine, % | 21/242, 9 | 3/56, 5 | 8/35, 23 (↑‡) | 4/119, 3 (↓‡) | 7, 14 |
| ↑ Cerebrospinal fluid cells, % | 82/205, 40 | 24/53, 45 | 10/24, 42 | 48/111, 43 | 6/40, 15 (↓*) |
| ↑ Cerebrospinal fluid proteins, % | 80/197, 41 | 22/48, 46 | 8/20, 40 | 46/106, 43 | 9/40, 23 (↓‡) |
Patient characteristics in history, clinical findings, and laboratory parameters before start of therapy in all patients, as well as in 4 different subgroups: (1) patients with an affected sibling; (2) patients who died within the first 8 weeks of therapy; (3) patients who underwent HSCT; and (4) patients who are alive and off all HLH therapy since ≥ 1 year without signs of disease activity or a HSCT. Patients could be included in several subgroups. Binary data have been used in the analyses (yes/no), and for laboratory data, standardized age-adjusted normal range values have been applied. Patients from a subgroup have been compared to the remaining patients. In the table body, numbers following commas are percentages.
HLH indicates hemophagocytic lymphohistiocytosis; MRI, magnetic resonance imaging; CT, computed tomography; LDH, lactate dehydrogenase; SGOT, serum glutamic oxaloacetic transaminase; SGPT, serum glutamic pyruvic transaminase; APTT, active partial thromboplastine time; ↑, an increased occurrence of the finding; and ↓, a decreased occurrence of the finding.
Symbols indicate statistical difference from other patients at the levels: *.001; †.05; or ‡.01.