Kasabach-Merritt现象新生儿严重血小板减少症的免疫学和临床特征
Immunological and clinical characteristics of severe thrombocytopenia in neonates with Kasabach-Merritt phenomenon.
摘要(中文译文)
本研究旨在描述卡萨巴赫-梅里特现象（KMP）新生儿的免疫特征，并识别与这些患者严重血小板减少相关的临床特征。本多中心回顾性研究纳入根据既定标准诊断为KMP的新生儿。比较KMP新生儿与对照组的外周血淋巴细胞亚群、补体水平及体液免疫。根据血小板计数是否<20×10⁹/L将KMP新生儿进一步分为重症组和非重症组。比较两组间的临床资料和治疗转归，并进行多因素分析以识别与严重血小板减少相关的独立因素。共纳入32例KMP新生儿（重症组13例，非重症组19例）。与对照组相比，KMP新生儿的CD3⁺CD4⁺和CD3⁺CD19⁺比例降低，IgG水平较低（均P<0.05）。KMP新生儿中最常见的KHE病变类型为表浅型+混合型（24/32，75.00%），最大直径中位数为68.50（52.50-87.25）mm，血小板计数为41.50（17.25-48.75）×10⁹/L。KMP新生儿中，25例达到完全缓解（78.1%），4例达到部分缓解（12.5%），3例未缓解（9.3%）。与非重症组相比，重症KMP组病变直径更大。重症组的CD3⁺CD4⁺比例亦较低，淋巴细胞百分比（L%）、PCT、CKMB和铁蛋白水平显著升高，APTT延长（P<0.05）。两组间预后无显著差异（P>0.05）。多因素回归分析确定APTT延长和铁蛋白升高与KMP严重血小板减少独立相关。KMP新生儿表现出免疫学异常，包括辅助性T细胞和B细胞减少。APTT延长和铁蛋白升高被确定为KMP严重血小板减少的独立危险因素。
Abstract
This study aimed to characterize the immune profile of neonates with Kasabach-Merritt phenomenon (KMP) and to identify clinical features linked to severe thrombocytopenia in these patients. This multicenter retrospective study included neonates diagnosed with KMP based on established criteria. The peripheral blood lymphocyte subsets, complement levels, and humoral immunity were compared between KMP neonates and controls. KMP neonates were further divided into severe and non-severe groups based on whether the platelet count was <20 × 10/L. Clinical data and treatment outcomes were compared between the two groups, and multivariate analysis was performed to identify independent factors associated with severe thrombocytopenia. A total of 32 KMP neonates were included (13 in the severe group and 19 in the non-severe group). Compared with controls, KMP neonates had reduced CD3CD4 and CD3CD19 proportions and lower IgG levels (all < 0.05). The most common type of KHE lesion in KMP neonates was superficial + mixed (24/32, 75.00%), with a median maximum diameter of 68.50 (52.50-87.25) mm and a platelet count of 41.50 (17.25-48.75) × 10/L. Of the KMP neonates, 25 achieved complete remission (78.1%), 4 achieved partial remission (12.5%), and 3 had no remission (9.3%). Compared to the non-severe group, the severe KMP group had larger diameters. The severe group also had lower CD3+CD4+ proportions, and significantly higher levels of lymphocytes percentage (L%), PCT, CKMB, and ferritin, along with prolonged APTT (P < 0.05). No significant difference in prognosis was observed between the two groups ( > 0.05). Multivariate regression analysis identified prolonged APTT and elevated ferritin as independently associated with severe thrombocytopenia in KMP. Neonates with KMP showed immunological abnormalities, including decreases in helper T cells and B cells. Prolonged APTT and elevated ferritin were identified as independent risk factors for severe thrombocytopenia in KMP.
如何引用
AMA
Cancan Li, Yang Li, Yan Huang, Yuanyuan Gao, Lili Li, Jinhui Hu, et al. Immunological and clinical characteristics of severe thrombocytopenia in neonates with Kasabach-Merritt phenomenon.. Frontiers in immunology. 2026; doi:10.3389/fimmu.2026.1847882.
APA
Cancan Li, Yang Li, Yan Huang, Yuanyuan Gao, Lili Li, Jinhui Hu, et al (2026). Immunological and clinical characteristics of severe thrombocytopenia in neonates with Kasabach-Merritt phenomenon.. Frontiers in immunology. https://doi.org/10.3389/fimmu.2026.1847882
GB/T 7714
Cancan Li, Yang Li, Yan Huang, Yuanyuan Gao, Lili Li, Jinhui Hu, et al. Immunological and clinical characteristics of severe thrombocytopenia in neonates with Kasabach-Merritt phenomenon.[J]. Frontiers in immunology, 2026 doi:10.3389/fimmu.2026.1847882.
在 PubMed增强版 阅读全文
翻译全文 · AI 文献问答 · PDF 原文下载 · 引用导出