From: Childhood Langerhans cell histiocytosis with severe lung involvement: a nationwide cohort study
Characteristics | Deceased patients, n = 6 | Surviving patients, n = 11 | P value |
---|---|---|---|
Age at PLCH diagnosis, median [min, max] | 1.0 y [0.2–5.0] | 1.4 y [0.1–13.7] | 0.8 |
RO- LCH disease | 3 | 7 | 0.6 |
RO+ LCH disease | 3 | 4 | |
Maximal DAS Score, median [min, max] | 13.5 [7,27] | 5 [4,10] | 0.04 |
First-line LCH therapy | |||
VLB Steroid | 5 | 11 | 0.4 |
2CDA | 1 | 0 | |
Second-line LCH therapy | |||
2CDA | 2 | 5 | 0.4 |
VP16 ARA-C | 2 | 1 | |
Targeted therapy | 0 | 2 | |
CT Score at PLCH diagnosis | (n = 4 evaluated cases) | (n = 9 evaluated cases) | |
Cyst, median [min, max] | 16 [8,24] | 10 [1,18] | 0.14 |
Nodule, median [min, max] | 0 [0,0] | 3 [0,9] | 0.08 |
BRAFV600E mutated LCH | 0/2 investigated cases | 4/9 investigated cases | 0.5 |
Time to death after PLCH diagnosis, median [min, max] | 6.9 months [4.6,13.7] | ||
Cause of death | |||
Repeated pneumothorax | 3 | _ | |
Diffuse micronodular lung infiltration | 2 | ||
Post lung transplant | 1 |