Table 2

Patient characteristics in history, clinical findings, and laboratory parameters before start of therapy in all patients

Patient subgroupAll 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, 5535, 5820, 5773, 5919, 39 (↓)
    Familial disease, %60/247, 2460, 1005/34, 1544/123, 36 (↑*)0 (↓*)
    Consanguinity, %50/240, 2118/56, 32 (↑)12/34, 35 (↑)26/119, 223/48, 6 (↓)
    Infectious history, %109/244, 4519/58, 33 (↓)15, 4347/120, 3925/48, 52
Clinical parameters
    Hepatomegaly, %237, 952, 9734, 97119, 9643, 88 (↓)
    Lymphadenopathy, %80/244, 3312, 20 (↓)10/34, 2936/122, 3022, 45 (↑)
    Edema, %97/243, 4024/59, 4121/34, 62 (↑)36/121, 30 (↓)21, 43
    Jaundice, %84/244, 3421/58, 3620/34, 59 (↑)38/121, 3114, 29
    Rash, %77/246, 3120/59, 349/34, 2640/122, 3319, 39
    Neurological symptoms, %80/245, 3323, 3810/33, 3043, 358, 16 (↓)
    MRI or CT of brain pathological, %43/135, 325/26, 196/10, 60 (↑)22/75, 296/28, 21
Laboratory findings
    ↑ Ferritin, %198/212, 9341/48, 85 (↓)26/29, 9096/102, 9444/47, 94
    ↑ LDH, %182/209, 8741/48, 8529/31, 9481/100, 81 (↓)42/44, 95
    ↑ SGOT, %164/216, 7637/53, 7025/30, 7180/107, 7538/45, 84
    ↑ SGPT, %181/237, 7641/55, 7527/34, 7988/118, 7541/47, 87
    ↑ Bilirubin, %123/239, 5132/57, 5623/34, 68 (↑)56/117, 4823/48, 48
    ↓ Albumin, %154/223, 6933/52, 6325/33, 7664/105, 61 (↓)36/46, 78
    Prolonged APTT, %127/227, 5629/54, 5421/32, 6656/109, 5129/48, 60
    ↓ Sodium, %119/234, 5131/57, 5413/33, 3953/115, 4633/47, 70 (↑)
    ↑ Creatinine, %21/242, 93/56, 58/35, 23 (↑)4/119, 3 (↓)7, 14
    ↑ Cerebrospinal fluid cells, %82/205, 4024/53, 4510/24, 4248/111, 436/40, 15 (↓*)
    ↑ Cerebrospinal fluid proteins, %80/197, 4122/48, 468/20, 4046/106, 439/40, 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.