Patient | 1 | 2 | 3 | 4 | 5 | 6 | 7 | 8 | 9 |
---|---|---|---|---|---|---|---|---|---|
Sex | Male | Male | Female | Male | Male | Male | Male | Female | Female |
Age at diagnosis of LCH | 1 y 10 mo | 2 y 9 mo | 1 y 10 mo | 3 y 4 mo | 2 y | 2 y 8 mo | 16 y 10 mo | 2 y 10 mo | 6 mo |
Organs involved at diagnosis of LCH | Bone, skin, liver, lung, spleen, hematopoietic system | Bone, skin | Bone, skin, liver | Bone, skin, DI | Bone, skin | Bone, skin, liver, spleen | Bone, skin, DI | Bone, skin, hematopoietic system, DI | Skin, liver |
Course of LCH (outside the CNS) | Chronic active | 3 RA | 2 RA | no RA | 4 RA | 1 RA | 2 RA | 2 RA | Chronic active |
Age at diagnosis of ND-LCH | 6 y 6 mo | 7 y 3 mo | 5 y 7 mo | 4 y 5 mo | 2 y 6 mo | 8 y 2 mo | 17 y 11 mo | 4 y 3 mo | 4 y 3 mo |
Interval diagnosis LCH to the diagnosis of ND | 4 y 7 mo | 4 y 7 mo | 3 y 10 mo | 1 y 1 mo | 5 mo | 5 y 7 mo | 1y | 1 y 5 mo | 3 y 9 mo |
MR imaging follow-up period | 7 y 5 mo | 10 y 3 mo | 5 y 2 mo | 7 y 6 mo | 11 y | 7 y 3 mo | 5 y | 11 y 8 mo | 6 y 8 mo |
No. of evaluated MR imaging studies | 4 | 4 | 3 | 3 | 5 | 4 | 3 | 5 | 3 |
Age at last MR imaging | 13 y 10 mo | 17 y 6 mo | 10 y 10 mo | 11 y 8 mo | 13 y 1 mo | 15 y 5 mo | 22 y 11 mo | 15 y 10 mo | 10 y 10 mo |
Additional intracranial abnormalities | |||||||||
HPR abnormalities** | −/+ | +/+ | +/+ | +/+ | −/+ | +/+ | +/+ | +/+ | +/+ |
Space-occupying meningeal lesions** | −/− | −/+ | −/− | −/− | −/+ | −/− | −/− | +/+ | −/− |
Craniofacial bone lesions** | +/+ | +/+ | +/+ | +/− | +/+ | +/− | +/+ | +/+ | −/− |
Space-occupying intraparenchymal lesions** | −/− | −/− | −/− | −/− | −/− | −/− | +/+ | −/− | −/− |
DI* | −/+ | +/+ | −/+ | +/+ | −/+ | +/+ | +/+ | +/+ | +/+ |
Growth hormone deficiency* | −/+ | +/+ | −/+ | −/− | −/− | −/− | +/+ | −/+ | −/− |
Neurologic symptoms* | −/− | −/− | −/− | Ataxia/severe cerebellar symptoms | −/− | −/− | −/− | −/ataxia, spastic diplegia | −/− |
Note:—ND indicates neurodegeneration; LCH, Langerhans cell histiocytosis; y, year; mo, month; DI, diabetes insipidus; RA, reactivation; HPR, hypothalamic pituitary region; −, absent; +, present.
* At diagnosis/at the last follow-up.
** before or at the diagnosis of ND-LCH/after the diagnosis of ND-LCH.