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慢性特发性血小板减少性紫癜患儿的临床及实验室特征

Clinical and Laboratory Characteristics of Children with Chronic Idiopathic Thrombocytopenic Purpura.

作者信息

Cekerevac Milica, Pantovic Jelena, Medovic Marija, Igrutinovic Nebojsa, Knezevic Sanja, Markovic Bojana, Mihajlovic Isidora, Todorovic Zeljko, Maksic Tijana, Vitosevic Natalija, Zivojinovic Suzana, Cekovic Djordjevic Jelena, Prodanovic Tijana, Medovic Rasa

机构信息

Pediatric Clinic, University Clinical Centre Kragujevac, Zmaj Jovina 30, 34000 Kragujevac, Serbia.

Department of Pediatrics, Faculty of Medical Science, University of Kragujevac, Svetozara Markovica 68, 34000 Kragujevac, Serbia.

出版信息

Diagnostics (Basel). 2025 May 12;15(10):1217. doi: 10.3390/diagnostics15101217.

DOI:10.3390/diagnostics15101217
PMID:40428210
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12109821/
Abstract

: Chronic idiopathic thrombocytopenic purpura (chITP) is an autoimmune disease which develops in 10-30% of patients with newly diagnosed idiopathic thrombocytopenic purpura (ndITP). It is defined as thrombocytopenia which lasts longer than 12 months, with extremely diverse clinical expressions. The aim is to present the most significant clinical and laboratory characteristics of children with chITP. : This is retrospective, observational research, which included children between 2-18 years with chITP who were treated in the Republic of Serbia for 25 years. We analyzed clinical data from personal and family medical histories and different laboratory analyses. : The total number of respondents was 152, with female predominance (F:M = 1.27:1) and mild predominance of adolescents. Of the patients, 15% were asymptomatic, but 15% had periodically life-threatening bleeding. Transfusion was not required for 70% of patients. Thirty-five percent of patients had chITP alone, and 45% had high titer levels of autoantibodies. The most frequent comorbidity was Hashimoto thyroiditis (15%). The same percentage (45%) of family members were reported with and without autoimmune diseases. Twenty-five percent of patients were resistant to initial therapy. was detected in 20%, 70% had higher levels of lactate dehydrogenase (LDH), three patients had sufficient serum vitamin D levels, splenomegaly was found in 25%, and accessory spleen in 14% of patients. Around 50% of patients had a platelet count between 20-50 × 10⁹/L, and 40% below 20 × 10⁹/L. Mean platelet volume (MPV) was 10.6 ± 1.4 fL. No dysplastic changes were noted in bone marrow aspirate. Initial first-line therapy was sufficient for 45% of patients, second-line therapy was administered in 25%, splenectomy was performed in 20%, and 10% received all available treatments. : The severe clinical form of pediatric chITP is accompanied by a low platelet count, the presence of autoimmune comorbidities, a positive family medical history, resistance to initial therapy, hypovitaminosis D, and rare megakaryocytes in the bone marrow.

摘要

慢性特发性血小板减少性紫癜(chITP)是一种自身免疫性疾病,在10% - 30%新诊断的特发性血小板减少性紫癜(ndITP)患者中发生。它被定义为血小板减少持续超过12个月,临床表现极为多样。目的是呈现chITP患儿最重要的临床和实验室特征。

这是一项回顾性观察研究,纳入了在塞尔维亚共和国接受治疗25年的2至18岁chITP患儿。我们分析了个人和家族病史的临床数据以及不同的实验室分析结果。

受访者总数为152人,女性占优势(女∶男 = 1.27∶1),青少年略占优势。患者中,15%无症状,但15%有周期性危及生命的出血。70%的患者无需输血。35%的患者仅患有chITP,45%的患者自身抗体滴度高。最常见的合并症是桥本甲状腺炎(15%)。报告有自身免疫性疾病和无自身免疫性疾病的家庭成员比例相同(45%)。25%的患者对初始治疗耐药。20%检测到[此处原文缺失相关内容],70%的患者乳酸脱氢酶(LDH)水平较高,3例患者血清维生素D水平充足,25%的患者发现脾肿大,14%的患者发现副脾。约50%的患者血小板计数在20 - 50×10⁹/L之间,40%的患者低于20×10⁹/L。平均血小板体积(MPV)为10.6±1.4 fL。骨髓穿刺未发现发育异常改变。45%的患者初始一线治疗就足够,25%的患者接受二线治疗,20%的患者进行了脾切除术,10%的患者接受了所有可用治疗。

小儿chITP的严重临床形式伴有血小板计数低、自身免疫合并症、家族病史阳性、对初始治疗耐药、维生素D缺乏和骨髓中巨核细胞罕见。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d3ac/12109821/cb6142920e21/diagnostics-15-01217-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d3ac/12109821/e394bd3def8c/diagnostics-15-01217-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d3ac/12109821/a2a039187137/diagnostics-15-01217-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d3ac/12109821/f4c545462f75/diagnostics-15-01217-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d3ac/12109821/cb6142920e21/diagnostics-15-01217-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d3ac/12109821/e394bd3def8c/diagnostics-15-01217-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d3ac/12109821/a2a039187137/diagnostics-15-01217-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d3ac/12109821/f4c545462f75/diagnostics-15-01217-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d3ac/12109821/cb6142920e21/diagnostics-15-01217-g004.jpg

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本文引用的文献

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Diagnostic value of platelet parameters and megakaryocyte formula of bone marrow in idiopathic thrombocytopenic purpura.特发性血小板减少性紫癜的血小板参数和骨髓巨核细胞公式的诊断价值。
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