Hakan KUTLAY, Serdar ŞEN, Hüseyin ÇAKMAK, Erkan DİKMEN, Şevket KAVUKÇU, Cemil EKİNCİ

Ender pulmoner bir intertisyel patoloji: Pulmoner lenfanjiyoleiomiyomatozis olgusu

Pulmoner lenfanjiyoleiomiyomatozis menapoz öncesi doğurganlık çağındaki kadınları etkileyen ender bir hastalıktır. Patolojik olarak akciğer dokusunda yaygın peribronşiyal, perivasküler ve perilenfatik düz kas proliferasyonu ve sonuçta hava yolu obstrüksiyonu, alveollerde parçalanma ve parankimde kist gelişimi ile karakterizedir. Hastalarda nefes darlığı, hemoptizi, tekrarlayan pnömotoraks atakları ve şilöz plevral efüzyon görülür. Bu hastalık genellikle ölüme sebep olan solunum yetmezliğine ilerler. Biz burada 22 yaşında rekürrens pnömotoraksı olan kadın hastayı inceledik. Postoperatif patolojik tanısı "pulmoner lenfanjiyoleiomiyomatozis" gelen hasta, hastalığının 6. yılında solunum yetmezliğinden kaybedildi.

Pulmonary lymphangioleiomyomatosis: Case report

Pulmonary Lymphangioleiomyomatosis is a rare disease which affects premenopausal women of childbearing age. It is characterised pathologically by extensive peribronchial, perivascular and perilymphatic smooth muscle proliferation which results in airway obstruction, alveolar disruption and the development of cysts in the lung tissue. Patients present with dyspnoea, recurrent pneumothoraces, heamoptysis and chylous pleural effusions. The disease usually progresses to cause death from respiratory failure. A 22-year women with recurrent pneumothoraces has been reported, whose postoperative diagnosis was pulmonary lymphangioleiomyomatosis. The patient died because of respiratory failure, six year after the diagnosis.

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