Correspondence

Pulmonary Lymphangiomyomatosis in a Preadolescent Girl

N Engl J Med 1998; 338:473-474February 12, 1998

Article

To the Editor:

A 12-year-old girl presented in 1996 with cough and dyspnea on exertion. A chest roentgenogram taken when she was three years old showed reticulonodular infiltrations that were interpreted as evidence of recurrent pneumonia. At the age of 10 years the chest roentgenogram showed bilateral patchy interstitial infiltrates with a reticular and vesicular pattern. The results of open-lung biopsy were initially interpreted as showing interstitial fibrosis and emphysema. Corticosteroid treatment resulted in symptomatic improvement, but the roentgenographic picture remained unchanged. Eight months later bilateral reticulonodular infiltrates and chylous effusion developed. A reappraisal of the open-lung–biopsy specimen and detailed immunohistochemical study established the diagnosis of lymphangiomyomatosis. The proliferating smooth-muscle cells positive for alpha–smooth-muscle actin in the lung showed a strong progesterone-receptor positivity (Figure 1Figure 1Lymphangiomyomatosis Characterized by the Subpleural Proliferation of Smooth-Muscle Cells with Progesterone-Receptor Positivity (Right) (ABC Immunoperoxidase Staining for Progesterone Receptor, ×400).) and estrogen-receptor negativity. No signs of menarche or secondary sex characteristics were noted at the time of the diagnosis. Serum levels of sex hormones were barely detectable: estradiol, <10 pg per milliliter; follicle-stimulating hormone, 0.6 mIU per milliliter; luteinizing hormone, 0.1 mIU per milliliter; and progesterone, 0.15 ng per milliliter. The manifestation of lymphangiomyomatosis was thought to have been triggered by a surge of sex hormones. Treatment was begun with an analogue of luteinizing hormone–releasing hormone, triptorelin (3.75 mg per month intramuscularly). After 10 months of therapy, the child's lung function had not improved (less than 30 percent of the predicted value) and the roentgenographic findings were unchanged.

Several authors have suggested that assays and immunohistochemical tests for estrogen and progesterone receptors in lung-biopsy specimens might be used to determine the most appropriate hormonal treatment for lymphangiomyomatosis.1 Our experience, however, does not seem to support the idea that high-affinity estrogen and progesterone receptors in smooth-muscle cells are the underlying pathogenetic mechanism of lymphangiomyomatosis.2 Drastic hormonal manipulations by means of medroxyprogesterone therapy or oophorectomy are not acceptable in a preadolescent girl, who may be the youngest patient reported to have severe pulmonary manifestations of lymphangiomyomatosis. We are of the opinion that our patient's present hormonal “silence” cannot be maintained for long, because it is associated with psychological problems. Lung transplantation may be an acceptable form of therapy in this girl.

Béla Nagy, M.D., Ph.D.
Zoltán Nábrády, M.D.
Zoltán Nemes, M.D., D.Sc.
University Medical School, H-4012 Debrecen, Hungary

2 References

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   Taylor JR, Ryu J, Colby TV, Raffin TA. Lymphangioleiomyomatosis: clinical course in 32 patients. N Engl J Med 1990;323:1254-1260
   Full Text | Web of Science | Medline

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   Dishner W, Cordasco EM, Blackburn J, Demeter S, Levin H, Carey WD. Pulmonary lymphangiomyomatosis. Chest 1984;85:796-799
   CrossRef | Web of Science | Medline

Citing Articles (2)

Citing Articles

1. 1

   Angelo M. Taveira–DaSilva, Gustavo Pacheco–Rodriguez, Joel Moss. (2010) The Natural History of Lymphangioleiomyomatosis: Markers of Severity, Rate of Progression and Prognosis. Lymphatic Research and Biology 8:1, 9-19
   CrossRef

2. 2

   D. Spencer. (2001) Rare single system diseases. Paediatric Respiratory Reviews 2:1, 63-69
   CrossRef