Department of Dermatology, Rabin Medical Center, Beilinson Hospital, 4941492, Petach Tikva, Israel.
Dermatology Unit, Schneider Children's Medical Center of Israel, Petach Tikva, Israel.
Arch Dermatol Res. 2017 Dec;309(10):851-856. doi: 10.1007/s00403-017-1783-7. Epub 2017 Sep 25.
Pediatric mycosis fungoides (MF) is a rare disease characterized by over-representation of atypical clinical variants, with a different prognosis from adult MF. Several human leukocyte antigen (HLA) alleles have been associated with MF in certain adult populations, including Israeli Jews. However, HLA data on pediatric MF as a group are lacking. To evaluate the possible association of the HLA system with pediatric MF, 59 Israeli Jewish patients diagnosed with MF at age ≤ 18 years underwent high- and intermediate-resolution genotyping for HLA class I (HLA-A*, HLA-B*) and class II (HLA-DRB1*, DQB1*) loci. The results were compared with data on 4169 umbilical cord blood units retrieved from a public cord blood bank in Jerusalem and samples from 252 healthy, unrelated Israeli Jewish volunteers. No statistically significant associations were found between pediatric MF and any of the alleles examined except HLA-B73. However, given the extremely low frequency of B73 in both the control group (0.1%) and the study group (2%), the biological significance of this finding is questionable. Further subgroup analyses by ethnicity (Ashkenazi and non-Ashkenazi) and clinicopathologic variant (follicular and non-follicular) yielded no significant between-group differences. These results suggest that the associations with the HLA system, reported previously in adult MF, do not hold true for pediatric MF. Thus, pediatric MF differs from its adult counterpart not only in clinical manifestations and course, but apparently also in the underlying immuno-pathogenetic mechanism.
儿童蕈样真菌病 (MF) 是一种罕见疾病，其特征是不典型临床变异型的过度表现，与成人 MF 的预后不同。在某些成年人群中，已发现几种人类白细胞抗原 (HLA) 等位基因与 MF 相关，包括以色列犹太人。然而，关于儿童 MF 作为一个群体的 HLA 数据是缺乏的。为了评估 HLA 系统与儿童 MF 的可能关联，59 名在 18 岁以下被诊断患有 MF 的以色列犹太患者接受了 HLA Ⅰ类 (HLA-A*、HLA-B*) 和Ⅱ类 (HLA-DRB1*、DQB1*) 基因座的高分辨率和中分辨率基因分型。将结果与从耶路撒冷公共脐带血库中检索的 4169 个脐带血单位的数据以及 252 名健康、无关的以色列犹太志愿者的样本进行了比较。除了 HLA-B73 之外，儿童 MF 与所检查的任何等位基因之间均未发现统计学上显著的关联。然而，鉴于 B73 在对照组 (0.1%) 和研究组 (2%) 中的极低频率，该发现的生物学意义是值得怀疑的。通过民族 (阿什肯纳兹人和非阿什肯纳兹人) 和临床病理变异型 (滤泡型和非滤泡型) 进行进一步的亚组分析，未发现组间有显著差异。这些结果表明，先前在成人 MF 中报道的与 HLA 系统的关联不适用于儿童 MF。因此，儿童 MF 不仅在临床表现和病程上与成人 MF 不同，而且显然在潜在的免疫发病机制上也不同。
