Case StudiesRecurrence of lymphangioleiomyomatosis after single lung transplantation: New insights into pathogenesis☆
Section snippets
Case summary
A 31-year-old white woman was in good health until 1990, when she developed hemoptysis and cough. The hemoptysis improved spontaneously, but in November 1991 the patient began to notice dyspnea. She was admitted to the hospital in February 1992 with exertional dyspnea and enlarged hilar lymph nodes. Despite an open lung biopsy, the correct diagnosis was not made.
In July 1993, the patient underwent right single lung transplantation of a male donor. The explanted right lung showed LAM with
Materials and methods
The recurrent LAM lesions of the allografted lung were reevaluated by an improved technique of double-staining IHC and NISH and subsequent multiplex polymerase chain reaction (PCR) analysis.
Double-staining IHC/NISH
Double staining by IHC with antibodies against α-actin and HMB45 and NISH applying a Y chromosome–specific probe casted doubt on whether the recurrent LAM lesions were of donor origin. Most of the spindle-shaped HMB45-positive cell groups did not exhibit any signal for the Y chromosome. Only some surrounding spindle-shaped cells without HMB45 expression, which were sometimes intermixed with the LAM cells, exhibited signals for the Y chromosome (Fig 2).
Discussion
LAM is a rare lung disease almost exclusively affecting women of childbearing age. Little progress has been made in understanding the pathogenesis of LAM since the disease was first described in 1937.9
The present study demonstrated a case of sporadic LAM with recurrence after single lung transplantation. By means of double-staining NISH and IHC and genotyping of STR polymorphism, our study seems to indicate that the recurrent LAM lesions originated from the recipient organism and cannot be
Acknowledgements
The authors thank Beate Luthardt for excellent technical skills and Dr. W. Wöckel and Dr. A. Morresi-Hauf, Zentralkrankenhaus Gauting, for providing the autopsy results.
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Address correspondence and reprint requests to Iris Bittmann, MD, Institute of Pathology, Ludwig-Maximilians University, Thalkirchner Str. 36, 80337 Munich, Germany.