[Assessment of the treatment of pulmonary hamartolymphangiomyomatosis].

I have recently treated four cases of pulmonary hamartolymphangiomyomatosis (HAM), mainly with progesterone therapy and oophorectomy. In two of four cases (case 2 and case 4), more than five years had passed from the onset of symptoms until the initiation of therapy. In one patient (case 2), the lungs had already been extensively destroyed when the therapy was started. In spite of effective therapy, she died of respiratory failure triggered by pneumothorax. Three of four cases were treated effectively, while one (case 1) had poor results. Despite progesterone therapy, oophorectomy and tamoxifen administration, her condition worsened and she died four years after the onset of symptoms. It is necessary to devise new treatments for such patients.