特发性血小板减少性紫癜 (ITP) – 它是什么？

什么是 ITP？

Idiopathic Thrombocytopenic Purpura (ITP) is an autoimmune disorder in which the immune system attacks platelets, leading to a low platelet count. Having a reduced amount of platelets stops the body from clotting normally, thereby increasing the risk of bleeding. The term “immune thrombocytopenia” is used more commonly to refer to this condition.

ITP is caused by a low platelet count in the blood. This increases the risk of bleeding and bruising. 

这 骨髓 负责血小板的生成。当血小板聚集在一起时，就会形成血凝块，封住血管和组织壁上的小裂口或切口，从而止血。 

当特发性血小板减少性紫癜导致血液中血小板不足时，血液凝结速度会减慢。因此，患者可能会出现内出血以及皮下出血。

免疫性血小板减少性紫癜 (ITP) 患者皮肤或口腔黏膜上会出现多处紫色瘀斑。除了瘀斑外， 瘀点, which are small red or purple dots on the skin, can appear with these bruises. Petechiae are frequently confused with a rash.

ITP 是一种可影响任何年龄段儿童和成人的疾病。根据现有数据，特发性血小板减少性紫癜 (ITP) 的发病率在特定年龄段的男性和女性之间似乎存在差异。成年女性可能更容易患上 ITP。

ITP类型

ITP is now categorized by time since diagnosis: acute (<3 months), persistent (3–12 months), and chronic (>12 months).

急性特发性血小板减少症

Acute ITP often comes on suddenly and lasts for a period of less than 3 months. It is the most common form of ITP, primarily affecting children and frequently occurs in children after a viral illness. Oftentimes, treatment is not needed as it will resolve on its own.

慢性 ITP

Chronic ITP refers to a disease persisting for more than 12 months. It can last for several years or even an entire lifetime.. Generally speaking, it affects adults, but it can also affect adolescents and children.

Even people who have severe forms of ITP can live for decades. Most people who have chronic ITP can stop treatment at some point and maintain a safe platelet count.

特发性血小板减少性紫癜的病因是什么？

“特发性”是指病因不明的疾病。该术语以前用于描述 ITP，因为该疾病首次被发现时病因不明。

It is now well established that the immune system plays a significant role in the development of ITP (hence the preferred name “immune thrombocytopenia”).

在成人中，感染 艾滋病病毒, 肝炎或幽门螺杆菌（一种导致胃溃疡的细菌）都可能引发这种疾病。大多数儿童特发性血小板减少性紫癜是病毒性疾病（例如腮腺炎或流感）的后遗症。

ITP的症状

以下是最常见的 ITP症状:

• 便血
• 尿液中带血
• 容易出现的瘀伤
• 自发性鼻出血
• 割伤后长时间出血
• 月经量异常多
• 手术期间大量出血
• 小腿上出现针尖大小的瘀点
• 牙龈出血（如牙科手术）

一些 ITP 患者没有任何症状。

特发性血小板减少性紫癜的诊断程序是什么？

您的医生将进行全面的身体检查，并询问您的病史和您正在服用的任何药物。

您的医生还会开具 全血细胞计数（CBC）, among other tests. Routine platelet autoantibody testing is not recommended in typical ITP because of limited accuracy; it is often used as an adjunct to other diagnostic tests to rule in a diagnosis of ITP.

还需要进行血涂片检查。这项检查需要将少量血液涂在载玻片上。然后在显微镜下观察载玻片，以检查血小板和其他血细胞。 

A provider may also perform a bone marrow biopsy; however, this is normally reserved for select patients where previous findings could suggest another diagnosis.

ITP 的可用治疗方案

您的医生将根据您血液中的血小板数量以及您经历的出血频率和量来确定最佳治疗方案。 

In some cases, medical intervention is not required. Children who develop the newly diagnosed form of ITP, for example, typically recover without treatment within a few weeks or months.

Adults who have milder cases of ITP may also be able to avoid treatment in some instances. However, your doctor will likely monitor your platelets to ensure you don’t need additional treatments in the future. 

如果您的血小板计数过低，您将面临出血的风险，并且通常需要治疗。

药物

如果您或您的孩子需要就医，医生几乎肯定会先给您开一些药物来缓解症状。免疫性血小板减少性紫癜有几种不同的药物治疗，如下所列。

静脉注射免疫球蛋白（免疫球蛋白)

行政管理 静脉注射免疫球蛋白 may be necessary if your bleeding has reached a critical level or you are scheduled for surgery and need a rapid rise in platelets. In these cases, your platelet count needs to be increased as soon as possible.

免疫球蛋白抗D

这种药物适用于以下人群 Rh阳性血 脾脏功能正常。

皮质类固醇

Your doctor may prescribe a corticosteroid such as prednisone or dexamethasone for a short course to raise platelets. Long-term steroid use is discouraged as it can cause several side effects, including infections, glaucoma, high blood sugar, and osteoporosis.

抗生素

幽门螺杆菌是导致绝大多数消化性溃疡的细菌，少数人患特发性血小板减少性紫癜也与该细菌有关。 

H. pylori is treated with antibiotics to eradicate the infection. The eradication of H. pylori has been shown to subsequently increase platelet counts in some individuals. 

血小板生成素受体激动剂

药物如 罗米司亭（Nplate） 和艾曲波帕（Promacta) 通过刺激骨髓中血小板的产生起作用，有助于防止免疫性血小板减少性紫癜患者出现瘀伤和出血。

These types of drugs can increase your risk of blood clots. Additional options include avatrombopag (Doptelet).

Fostamatinib

Fostamatinib is an oral SYK (spleen tyrosine kinase) inhibitor approved for adults with chronic ITP who have had an insufficient response to previous therapies. Fostamatinib works to stop the breakdown of platelets already circulating in the body.

Splenectomy

如果您患有严重的 ITP，并且服药后症状或血小板计数没有改善，您的医生可能会建议进行脾切除手术（脾切除术）。 

脾脏 抗体 （蛋白质）有助于抵抗感染。在特发性血小板减少性紫癜中，这些抗体会错误地破坏血小板。因此，切除脾脏可以减少血小板的破坏。 

Because effective medical options exist, splenectomy is typically deferred (often ≥12 months after diagnosis) and used for refractory cases. Vaccination is recommended pre-operatively to reduce infection risk.

特发性血小板减少性紫癜的紧急治疗是什么？

Some people who have ITP with severe bleeding may require platelet transfusions and hospitalization. In life-threatening bleeding or urgent surgery, treatment typically combines IVIG + corticosteroids + platelet transfusions, and antifibrinolytics may be added.

改变生活方式

Your doctor may recommend that you make the following changes to your lifestyle following an ITP diagnosis:

• 参与低冲击活动而不是竞技运动或其他高强度活动，因为这有助于降低受伤、擦伤和出血的风险。
• 适量饮酒，因为它有可能损害血液凝固。
• 使用非处方药如阿司匹林和布洛芬时要小心，因为它们可能会损害血小板功能。

妊娠期免疫性血小板减少性紫癜的治疗

患有 ITP 的孕妇通常不会影响胎儿。然而，有些婴儿可能出生时血小板计数低，或出生后不久就会出现血小板计数低的情况。

婴儿的血小板计数几乎总是无需任何治疗就能恢复正常。然而，对于血小板计数极低的婴儿，治疗可以加快康复速度。

妊娠期特发性血小板减少性紫癜的治疗取决于孕妇的血小板计数。如果需要治疗，医生会仔细评估其对胎儿的潜在影响。

轻度 ITP 女性通常无需治疗即可顺利度过妊娠期。血小板计数极低或出血量大的孕妇更容易在分娩期间或产后出现大量出血。 

为了防止大量出血，这些女性通常会接受某种形式的治疗。

特发性血小板减少性紫癜的治疗前景如何？

大多数患有免疫性血小板减少性紫癜的人并没有严重或危及生命的疾病。

Children with acute ITP  typically recover within 3–6 months or less and oftentimes do not require treatment.

Chronic ITP can last for several years or even decades. Those suffering from severe cases can still expect to live for decades. Many people with ITP can safely manage their condition and avoid long-term complications. With modern therapies (e.g., thrombopoietin receptor agonists, fostamatinib), many adults achieve remission or stable platelet counts.

结论

如果您或您的孩子出现不明原因的瘀伤或出血，必须立即就医。许多疾病都可能导致出血和瘀伤，包括流感。 

诊断结果可能为特发性血小板减少性紫癜，且预后良好。部分病例需要治疗以预防或控制可能出现的并发症。

如果症状复发，即使您目前正在服用药物或病情似乎在一段时间内有所改善，也必须尽快就医。

一旦医生诊断出您的病情，您就可以接受治疗 AmeriPharma™ 专业药房. 我们为患有特发性血小板减少性紫癜的患者提供家庭 IVIG 输注服务。 

我们的 专业药房 甚至可以派遣专门的输液护士到您家里进行治疗并帮助您解决任何问题。 联系我们 现在就可以在家接受专科护理，享受全方位服务协调、全天候支持以及 共同支付援助.

常见问题解答

免疫性血小板减少性紫癜能治愈吗？

虽然 ITP 无法治愈，但许多患者发现治疗后血小板计数有所改善。许多 ITP 患者面临的挑战是找到一种既有效又无副作用的治疗方法。

一些患者报告说，改变他们的 饮食或生活方式 帮助他们感觉更好。这种疾病可以长期处于缓解状态，甚至可能伴随患者的余生。但特发性血小板减少性紫癜也可能复发。 

目前尚无办法预测该疾病的病程。

特发性血小板减少性紫癜的病因是什么？

目前尚不清楚导致某些人患上 ITP 的原因，但已知 ITP 会导致人体免疫系统破坏健康的血小板，从而导致容易或过度瘀伤或出血。

一些病例是在病毒或细菌感染、免疫接种、接触毒素后出现的，或与其他疾病（如狼疮或艾滋病毒 (HIV)）有关。 

ITP is generally not considered to be inherited or passed from one generation to another.