POEMS 综合征

概述

POEMS 综合征是一种罕见的血液疾病，它会损伤神经并累及身体的其他部位。POEMS 的体征和症状如下：

• 多发性神经病。您的双腿会感到麻木、刺痛和无力，久而久之，您的双手会出现同样的症状，并且您会感到呼吸困难。这是 POEMS 综合征诊断的一个基本特征。
• 器官肥大。脾脏、肝脏或淋巴结肿大。
• 内分泌障碍/水肿。激素水平异常，可能导致甲状腺机能低下（甲状腺功能减退症）、糖尿病、性问题、疲劳、四肢肿胀、新陈代谢和其他基本功能问题。
• 单克隆蛋白。异常的骨髓细胞（浆细胞），可产生一种存在于血液中的蛋白质（单克隆蛋白）。这是 POEMS 综合征诊断的一个基本特征。单克隆蛋白通常与异常的骨硬化或增厚有关。
• 皮肤变化。皮肤色素沉着，有红点，皮肤可能增厚，面部或腿部毛发增多。

POEMS 综合征的病因尚不清楚。但是 POEMS 综合征患者的浆细胞数量会升高。这些细胞会产生一种异常数量的蛋白质，可对身体的其他部位造成损伤。

POEMS 综合征可能被误诊，因为它的体征和症状与其他疾病相似。如果不经治疗，POEMS 综合征会迅速进展并可能危及生命，因此早期诊断非常重要。

治疗 POEMS 综合征可能会改善您的症状，但不能治愈这种病症。治疗方法包括放疗、化疗和干细胞移植。在过去的十几年里，POEMS 综合征患者的前景有了很大的改善。

症状

The symptoms of POEMS syndrome make up its name. Symptoms most often appear over weeks to months. They include the following:

• Polyneuropathy. This may include numbness, tingling and weakness in the legs. In time, it might affect the hands. It also involves trouble breathing. Polyneuropathy must be present for a POEMS diagnosis.
• Organomegaly. This is an enlarged spleen, liver or lymph nodes.
• Endocrinopathy and edema. Atypical hormone levels can cause an underactive thyroid, also called hypothyroidism. The hormone levels also can cause diabetes, sexual issues, tiredness, and swelling in arms and legs.
• Monoclonal protein. This is a protein that atypical plasma cells in bone marrow make. The monoclonal protein goes into the bloodstream. This must be present for the diagnosis of POEMS syndrome. Monoclonal protein often is linked with bones getting harder or thicker.
• Skin changes. This may involve having more color than usual on the skin. There may be red spots, which may be harder to see on Black or brown skin. The skin might be thicker. There might be more hair on the face or the legs.

Not all the symptoms of POEMS syndrome are in the name. And not everyone with POEMS syndrome has all the symptoms in the name. But everyone with POEMS syndrome has polyneuropathy and monoclonal protein.

Other symptoms some people with POEMS syndrome have spell out PEST. PEST stands for:

• Papilledema. This is swelling in the back of the eyes.
• Extravascular volume overload. This might include ankle swelling, fluid between the lung and the ribs, or fluid in the belly.
• Sclerotic bone lesions. X-rays can show these bone spots. They're most often not painful.
• Thrombocytosis and erythrocytosis. These are higher levels of red cells and platelets in the bloodstream.

When to see a doctor

Make an appointment with your healthcare professional if you have any of the symptoms of POEMS syndrome.

病因

Experts don't know the cause of POEMS syndrome. But people with POEMS syndrome have a higher number of plasma cells. These cells make too much of a type of protein, called monoclonal protein. This protein can damage other parts of the body.

风险因素

Factors that raise the risk of POEMS syndrome include:

• Older age. POEMS syndrome most often happens after age 50.
• Male sex. People assigned male at birth are a bit more likely to get POEMS syndrome than are people assigned female at birth.

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