[Sporadic lymphangioleiomyomatosis and pulmonary hypertension. Clinical and pathologic study in patients undergoing lung transplantation]

Abstract

Background and objective: Lymphangioleiomyomatosis (LAM) is a rare disease characterized by an aberrant immature smooth muscle cells proliferation. This excessive growth of LAM cells around airways, parenchyma, lymphatic ganglia and lung vessels, produces cystic damages. LAM affects mainly women and can lead to pulmonary hypertension (PH). The aim of this research is the clinical study, as well as the analysis of the morphological features, expressing PH, of the native lungs from lung transplant (LT) patients.

Patients and methods: Several clinical variables were studied in 7 patients who had a LAM and who were subjected to LT. The variables analyzed using morphologic and immunohistochemistry techniques were: histological alterations in native lungs, their severity rate, the presence of haemosiderosis in the air spaces and correlation index between thickness of the middle layer and the pulmonary arterial vascular diameter, morphological expression of HP.

Results: The average age was 34 years old and the predominant symptom was dyspnea. The respiratory functional pattern was mixt, with decrease of DLCO% and KCO%, serious hypoxemia and normocapnia. In the cardiological study, subclinical signs of HP were found. The immunohistochemistry was positive for smooth muscle and melanic expression markers, estrogen receptor in 6 cases and progesterone receptor in 4. Vascular correlation index showed HP criteria at all.

Conclusions: This research shows the presence of clinical and morphological HP criteria in advanced LAM patients. Therefore, cardiological assessment before LT with right catheterization will be justified. Future research should be addressed to clarify whether the HP should be criteria for LT in LAM patients.