Elsevier

Human Pathology

Volume 34, Issue 1, January 2003, Pages 95-98
Human Pathology

Case Studies
Recurrence of lymphangioleiomyomatosis after single lung transplantation: New insights into pathogenesis

https://doi.org/10.1053/hupa.2003.50Get rights and content

Abstract

Lymphangioleiomyomatosis (LAM) is a rare disease found primarily in white women of childbearing age. The present study describes a case of recurrent LAM after single lung transplantation. Double-staining nonisotopic in situ hybridization, immunohistochemistry, and short tandem repeat loci analysis demonstrated that the recurrent LAM lesions originated from the recipient. The data strongly support that metastatic spread of LAM cells or migration of progenitor cells plays an important role in the pathogenesis of LAM. HUM PATHOL 34:95-98. Copyright 2003, Elsevier Science (USA). All rights reserved.

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).

. A small LAM focus of the allografted lung

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.

References (22)

  • H Tanaka et al.

    Diagnosis of pulmonary lymphangioleiomyomatosis by HMB45 in surgically treated spontaneous pneumothorax

    Eur Respir J

    (1995)
  • Cited by (113)

    • Lymphangioleiomyomatosis

      2023, Presse Medicale
    • Lung transplantation for lymphangioleiomyomatosis

      2023, Journal of Heart and Lung Transplantation
    • Lymphangioleiomyomatosis: pathogenesis, clinical features, diagnosis, and management

      2021, The Lancet Respiratory Medicine
      Citation Excerpt :

      LAM has been reported to recur in donor allografts of patients who have undergone lung transplantation. The lesions are composed of LAM cells that have been genetically proven to arise from the recipient.43,44 Although these findings first supported a metastatic mechanism for the disease almost 20 years ago, the source of the invading cell has remained elusive.

    • Lung Transplantation: Recipient Selection

      2021, Encyclopedia of Respiratory Medicine, Second Edition
    • Lymphangioleiomyomatosis

      2021, Encyclopedia of Respiratory Medicine, Second Edition
    View all citing articles on Scopus

    Address correspondence and reprint requests to Iris Bittmann, MD, Institute of Pathology, Ludwig-Maximilians University, Thalkirchner Str. 36, 80337 Munich, Germany.

    View full text