一名特纳综合征患者的原发性免疫性血小板减少性紫癜伴大量口腔表现。

IF 0.9 Q3 DENTISTRY, ORAL SURGERY & MEDICINE Special Care in Dentistry Pub Date : 2024-11-01 Epub Date: 2024-07-11 DOI:10.1111/scd.13039
Liz Oliveira Trajano Dos Santos, Angélica de Sousa Santos, Marielly de Souza Oliveira, Natalia Vitória de Araújo Lopes, José Klidenberg de Oliveira Júnior, Hianne Cristinne de Morais Medeiros, John Lennon Silva Cunha, Luan Éverton Galdino Barnabé
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引用次数: 0

摘要

背景:原发性免疫性血小板减少性紫癜(ITP原发性免疫性血小板减少性紫癜(ITP)是一种自身免疫性血液病,以孤立的血小板减少为特征,常伴有口腔表现。然而,特纳综合征(TS)患者原发性 ITP 的报道却异常罕见,文献中也鲜有记录。在此,我们描述了一例不同寻常的特纳综合征患者原发性 ITP 并伴有丰富口腔表现的病例:口腔外体格检查发现,患者上肢和下肢多处瘀斑,右眼巩膜出血(吞咽功能减退)。口腔内部检查发现,下唇、舌背和口腔粘膜上有多个充血的水泡和水疱,牙龈自发性出血,上颚有出血性瘀斑。实验室检查显示血小板减少(5000/mm3),而红细胞和白细胞计数正常。在排除了其他病因或相关疾病后,患者被诊断为重症 ITP,并开始在重症监护室接受全身皮质类固醇治疗,结果血小板成功上升。经过两年的随访,该患者至今未再复发:结论:口腔表现可能是 ITP 的首发症状之一。因此,牙医必须熟悉这种情况,在遇到不寻常的口腔出血时,应考虑到血液病(如 ITP)的可能性,以确保及早做出正确诊断。此外,ITP 的存在会进一步加重 TS 的相关并发症。因此,考虑到心血管疾病和自身免疫性疾病的高发病率以及这些患者预期寿命的缩短,对这些患者进行严格的随访至关重要。
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Primary immune thrombocytopenic purpura with exuberant oral manifestations in a patient with Turner syndrome.

Background: Primary Immune Thrombocytopenic Purpura (ITP) is an autoimmune hematological condition characterized by isolated thrombocytopenia and frequently presents with oral manifestations. However, reports of primary ITP in patients with Turner Syndrome (TS) are exceptionally rare, with few cases documented in the literature. Herein, we describe an unusual case of primary ITP with exuberant oral manifestations in a patient with TS.

Case report: A 29-year-old woman was referred to an oral diagnostic service with complaints of "blood blisters and gum bleeding" lasting 8 h. On extraoral physical examination, multiple petechiae were observed in the upper and lower limbs, in addition to hemorrhagic extravasation in the right ocular sclera (hyposphagma). On intraoral examination, multiple vesicles and blisters filled with blood were identified on the lower lip, back of the tongue, and buccal mucosa, along with spontaneous gingival bleeding and hemorrhagic petechiae on the palate. Laboratory tests revealed thrombocytopenia (5000/mm3), whereas the blood count showed normality in the red and white series. After excluding other etiological factors or associated diseases, the patient was diagnosed with severe ITP and began treatment with systemic corticosteroids in the intensive care unit, resulting in a successful increase in platelets. After a 2-year follow-up, the patient remains free of ITP recurrences.

Conclusion: Oral manifestations may be one of the first signs of ITP. Therefore, it is essential that dentists are familiar with the condition and, when faced with unusual oral bleeding, consider the possibility of a hematological disorder such as ITP, ensuring a correct and early diagnosis. Moreover, the presence of ITP can further exacerbate complications associated with TS. Therefore, rigorous follow-up of these patients is crucial, considering the high incidence of cardiovascular and autoimmune diseases and the reduced life expectancy of these patients.

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来源期刊
Special Care in Dentistry
Special Care in Dentistry DENTISTRY, ORAL SURGERY & MEDICINE-
CiteScore
2.40
自引率
14.30%
发文量
120
期刊介绍: Special Care in Dentistry is the official journal of the Special Care Dentistry Association, the American Association of Hospital Dentists, the Academy of Dentistry for Persons with Disabilities, and the American Society for Geriatric Dentistry. It is the only journal published in North America devoted to improving oral health in people with special needs.
期刊最新文献
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