第123课肺淋巴管肌瘤病（Pulmonary lymphangiomyomatosis）（一）

zskyteacher 2019-08-16

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上期试题：化脓性脑膜炎的并发症不包括d

A．智力障碍

B．脑积水

C．脑性低钠血症

D．原发性癫痫

E．脑室管膜炎

这期说说这个罕见病例，到目前为止，近年来文献统计。发现的全部病例中全为女性，接下来我们看看这个只发生在女性的罕见病是怎样的表现的。

This issue about this rare case, so far, the literature statistics in recent years. All found in all cases for women, let's look at how this occurs only in women's illness.

肺淋巴管肌瘤病（Pulmonary lymphangiomyomatosis）是一种原因不明的弥漫性肺部疾病，主要病理改变为支气管、血管和淋巴管内出现未成熟的平滑肌增生。该病可散发或与遗传病结节性硬化症有关。

Pulmonary lymphatics fibroid disease (Pulmonary lymphangiomyomatosis) is a kind of diffuse lung disease of unknown origin, the main pathological changes of bronchi and blood vessels and lymphatic vessels in immature smooth muscle hyperplasia. The disease can send out or related to genetic disorders of tuberous sclerosis.

目前认为该病与雌激素有一定的相关性；弹性蛋白酶/α1-抗胰蛋白酶系统的不平衡；该病细胞可能来源于淋巴管平滑肌脂肪瘤的转移或前体细胞移位；近年来发现该病患者的异常平滑肌细胞TSC2/TSC1基因异常，在平滑肌过度增殖中发挥作用。

Now that the disease has certain correlation with estrogen; Elastase/alpha 1 - antitrypsin system imbalance; Cells may come from the disease of lymphatic vessel smooth muscle lipoma transfer or shift of the precursor cells; In recent years found that patients with the disease of abnormal smooth muscle cells TSC2 / TSC1 gene abnormalities, play a role in the smooth muscle of excessive proliferation.

肺淋巴管肌瘤病（Pulmonary lymphangiomyomatosis）诊断需要组织活检和/或病史与胸部HRCT。HMB-45免疫组化染色阳性可增加诊断的敏感性和特异性。

Pulmonary lymphatics fibroid disease (Pulmonary lymphangiomyomatosis) diagnosis requires biopsy and/or history with the chest HRCT. HMB - 45 immunohistochemical staining positive can increase diagnostic sensitivity and specificity.

影像表现：

肺部多发囊腔是最常见的影像学表现，出现率100%，双肺广泛分布囊状影，直径在2-20mm之间，多数小于10mm，囊壁多小于3mm，囊壁间组织相对正常，弥漫性分布，无分布规律；血管位于囊腔周围，不位于中央；随着病程的发展，囊状影有增大增多的趋势，部分融合为肺大泡。

Multiple cystic cavity is the most common lung imaging performance, occurrence rate of 100%, double lung cystic shadow, widely distributed between 2-20 mm in diameter, most less than 10 mm, capsule wall is much less than 3 mm, relatively normal capsule wall between organizations, diffuse distribution, without distribution; The lumen of the blood vessels in surrounding, is located in the central; With the development of the course of the disease, cystic shadow has a tendency to increase more, part of the fusion of pulmonary bullae.

双肺呈斑片状及网格状改变，由于小叶间隔增厚，淋巴管水肿，双肺呈网格状，此征象多见于双肺下叶基底段。

Double lung is patchy and grid change, because the interlobular septal thickening, lymphatic edema, double lung in grid, the signs in both lungs and lower lobe basal segment.

患者双侧或单侧可反复见乳糜性胸腔积液，通过胸水化验其蛋白含量明显高于正常，可确诊。

Patients with bilateral or unilateral can see chylous pleural effusion, repeatedly by pleural effusion tests its protein content is significantly higher than normal, can be confirmed.

如肺部病越严重，腹部也可合并其他病灶，包括肝脏、肾脏的血管平滑肌脂肪瘤，腹腔及腹膜后淋巴结肿大、腹膜后淋巴结肌瘤。

Such as the more severe lung disease, abdominal can also be merged other lesions, including liver, renal angiomyolipomas, abdominal and retroperitoneal lymph node enlargement, retroperitoneal lymph node fibroids.

病例一：女，46岁↓