Table 1 Characteristics of the HLH cohort and 1-year survival (95% CI)
Characteristic | Number at risk at start of study (%) | Number of deaths | Crude survival probability at 1 year | CI (95%)a | |
|---|---|---|---|---|---|
Overall | – | 1628 (100.00%) | 811 | 0.50 | (0.48–0.53) |
Gender | Female | 708 (43.49%) | 317 | 0.55 | (0.52–0.59) |
Male | 920 (56.51%) | 494 | 0.46 | (0.43–0.50) | |
Age group | 0–4 | 315 (19.35%) | 124 | 0.61 | (0.55–0.66) |
5–14 | 196 (12.04%) | 47 | 0.76 | (0.70–0.82) | |
15–34 | 290 (17.81%) | 80 | 0.72 | (0.67–0.78) | |
35–54 | 266 (16.34%) | 136 | 0.49 | (0.43–0.55) | |
55–74 | 421 (25.86%) | 315 | 0.25 | (0.21–0.30) | |
75+ | 140 (8.60%) | 109 | 0.22 | (0.16–0.30) | |
Epoch | 2003–2008 | 306 (18.80%) | 134 | 0.56 | (0.51–0.62) |
2009–2013 | 488 (29.98%) | 235 | 0.52 | (0.48–0.56) | |
2014–2018 | 834 (51.23%) | 442 | 0.47 | (0.44–0.51) | |
Chronic conditions | Any inflammatory rheumatological disease/IBD | 378 (23.22%) | 140 | 0.63 | (0.58–0.68) |
Inflammatory bowel disease | 70 (4.30%) | 34 | 0.51 | (0.41–0.65) | |
Adult-onset Still's disease | 30 (1.84%) | 7 | 0.77 | (0.63–0.93) | |
Systemic juvenile idiopathic arthritis | 78 (4.79%) | 8 | 0.90 | (0.83–0.97) | |
Rheumatoid arthritis | 40 (2.46%) | 26 | 0.35 | (0.23–0.53) | |
Other inflammatory arthritis | 9 (0.55%) | 5 | 0.44 | (0.21–0.92) | |
Vasculitis | 64 (3.93%) | 38 | 0.41 | (0.30–0.55) | |
Other connective tissue diseases | 40 (2.46%) | 12 | 0.70 | (0.57–0.86) | |
Systemic lupus erythematosus | 47 (2.89%) | 10 | 0.79 | (0.68–0.91) | |
Haematological malignancies | Any haematological malignancy | 461 (28.32%) | 331 | 0.28 | (0.24–0.33) |
B-cell lymphoma | 114 (7.00%) | 86 | 0.25 | (0.18–0.34) | |
Hodgkin lymphoma | 40 (2.46%) | 26 | 0.35 | (0.23–0.53) | |
Lymphoma NOS | 32 (1.97%) | 25 | 0.22 | (0.11–0.42) | |
T-cell lymphoma | 84 (5.16%) | 66 | 0.21 | (0.14–0.32) | |
Leukaemia | 125 (7.68%) | 94 | 0.25 | (0.18–0.34) | |
Other haematological histiocytic/myelodysplastic/malignancy/unspecified | 66 (4.05%) | 34 | 0.48 | (0.38–0.62) | |
Non-haematological malignancies | Any non-haematological malignancy excluding non-melanoma skin cancer | 107 (6.57%) | 74 | 0.31 | (0.23–0.41) |
Malignant neoplasms of breast | 17 (1.04%) | 10 | 0.41 | (0.23–0.73) | |
Malignant neoplasms of genital organs | 21 (1.29%) | 14 | 0.33 | (0.18–0.61) | |
Malignant neoplasms of urinary tract | 22 (1.35%) | 16 | 0.27 | (0.14–0.54) | |
Other non-haematological malignancies | 47 (2.89%) | 34 | 0.28 | (0.17–0.44) | |
Hierarchical chronic conditionsb | Haematological malignancy | 461 (28.32%) | 331 | 0.28 | (0.24–0.33) |
Rheumatological disease or IBD | 322 (19.78%) | 99 | 0.69 | (0.64–0.74) | |
Non-haematological malignancy excluding non-melanoma skin cancer | 72 (4.42%) | 45 | 0.37 | (0.28–0.51) | |
None recorded | 773 (47.48%) | 336 | 0.57 | (0.53–0.60) |