Olaya-López EE, García-Ramírez JR, Carrasco-Daza D, Guzmán de AE, Mondragón-Armijo EV

Language: Spanish
References: 15
Page: 118-122
PDF size: 106.91 Kb.

ABSTRACT

Lymphangioleiomyomatosis (LAM) is a rare, slowly progressive systemic disease, affecting mainly women during their reproductive years; lung involvement is the rule. Two clinical forms are recognized: S-LAM (sporadic) and TSC (associated to Tuberous Sclerosis Complex). The disease has a slow progressive course that eventually leads to respiratory insufficiency. Despite a wide variety of therapeutic options, there is no single treatment with a beneficial effect on morbidity and mortality. We present the case of a 34 year old woman with dyspnea, lung cysts on CT scan and histologic diagnosis of LAM.

REFERENCES

1. Johnson SR. Lymphangioleiomyomatosis. Eur Respir J 2006;27:1056-1065.

2. Van Stossel E. Uber muskulare cirrhose der lunge. Beitr Klin Tuberk Spezif Tuberkuloseforsch 1937; 90:432-442.

3. Laniado-Laborín R, Valenzuela-Espinaza A, Cabrales-Vargas MN. Linfangioleiomiomatosis. Presentación de un caso y revisión de la literatura. Rev Inst Nal Enf Resp Mex 2002;15:162-165.

4. McCormack FX. Lymphangioleiomyomatosis: a clinical update. Chest 2008;133:507-516.

5. Ryu JH, Swensen SJ. Cystic and cavitary lung diseases: focal and diffuse. Mayo Clin Proc 2003;78:744-752.

6. 6 Chan JK, Tsang WY, Pau MY, Tang MC, Pang SW, Fletcher CD. Lymphangioleiomyomatosis and angiomyolipoma: closely related entities characterized by hamartomatous proliferation of HMB-45-positive smooth muscle. Histopathology 1993; 22:445-455.

7. Glassberg MK. Lymphangioleiomyomatosis. Clin Chest Med 2004;25:573-582.

8. Matsui K, Takeda K, Yu ZX, Travis WD, Moss J, Ferrans VJ. Role for activation of matrix metalloproteinases in the pathogenesis of pulmonary lymphangioleiomyomatosis. Arch Pathol Lab Med 2000;124:267-275.

9. Young LR, Inoue Y, McCormack FX. Diagnostic potential of serum VEGF-D for lymphangioleiomyomatosis. N Engl J Med 2008;358:199-200.

10. Yu J, Astrinidis A, Howard S, Henske EP. Estradiol and tamoxifen stimulate LAM-associated angiomyolipoma cell growth and activate both genomic and nongenomic signaling pathways. Am J Physiol Lung Cell Mol Physiol 2004;286:L694-700.

11. Eliasson AH, Phillips YY, Tenholder MF. Treatment of lymphangioleiomyomatosis. A meta-analysis. Chest 1989;96:1352-1355.

12. Glassberg MK, Elliot SJ, Fritz J, et ál. Activation of the estrogen receptor contributes to the progression of pulmonary lymphangioleiomyomatosis via matrix metalloproteinase-induced cell invasiveness. J Clin Endocrinol Metab 2008;93:1625-1633.

13. Kalomenidis I. Octreotide and chylothorax. Curr Opin Pulm Med 2006;12: 264-267.

14. Bissler JJ, McCormack FX, Young LR, et ál. Sirolimus for angiomyolipoma in tuberous sclerosis complex or lymphangioleiomyomatosis. N Engl J Med 2008;358: 140-151.

15. 15.Moses MA, Harper J, Folkman J. Doxycycline treatment for lymphangioleiomyomatosis with urinary monitoring for MMPs. N Engl J Med 2006;354:2621-2622.