Department of Women's and Children's Health, Uppsala University and University Children's Hospital, Uppsala, Sweden.
Department of Molecular Medicine and Surgery, Karolinska Institute, and Clinical Genetics, Karolinska University Hospital, Stockholm, Sweden.
Br J Haematol. 2022 Sep;198(6):1023-1031. doi: 10.1111/bjh.18360. Epub 2022 Jul 18.
The human cathelicidin hCAP-18 (pro-LL-37) is the pro-protein of the antimicrobial peptide LL-37. hCAP-18 can be produced by many different cell types; bone marrow neutrophil precursors are the main source of hCAP-18 in the circulation. Neutrophil count is used as a marker for myelopoiesis but does not always reflect neutrophil production in the bone marrow, and thus additional markers are needed. In this study, we established the reference interval of serum hCAP-18 level in healthy children and compared serum hCAP-18 levels between different diagnostic groups of children with haemato-oncological diseases, at diagnosis. We found that children with diseases that impair myelopoiesis, such as acute leukaemia, aplastic anaemia, or myelodysplastic syndrome, presented with low hCAP-18 levels, whereas patients with non-haematological malignancies displayed serum hCAP-18 levels in the same range as healthy children. Children with chronic myeloid leukaemia presented with high circulating levels of hCAP-18, probably reflecting the high number of all differentiation stages of myeloid cells. We suggest that analysis of serum hCAP-18 provides additional information regarding myelopoiesis in children with haemato-oncological diseases, which may have future implications in assessment of myelopoiesis in clinical management.
人源杀菌肽 hCAP-18(前体-LL-37)是抗菌肽 LL-37 的前体蛋白。hCAP-18 可以由许多不同的细胞类型产生;骨髓中性粒细胞前体是循环中 hCAP-18 的主要来源。中性粒细胞计数可作为骨髓生成的标志物,但并不总是反映骨髓中的中性粒细胞生成,因此需要其他标志物。在这项研究中,我们建立了健康儿童血清 hCAP-18 水平的参考区间,并比较了不同血液病患儿在诊断时不同诊断组之间的血清 hCAP-18 水平。我们发现,造血功能受损的疾病患儿,如急性白血病、再生障碍性贫血或骨髓增生异常综合征,hCAP-18 水平较低,而非血液系统恶性肿瘤患儿的血清 hCAP-18 水平与健康儿童相同。慢性髓性白血病患儿循环中 hCAP-18 水平较高,可能反映了髓系细胞所有分化阶段的数量较多。我们认为,分析血清 hCAP-18 可提供有关血液病患儿骨髓生成的额外信息,这可能对临床管理中评估骨髓生成具有未来意义。