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Long-term successful outcome of sequential cardiac and allogeneic bone marrow transplantations in severe AL amyloidosis

Bone Marrow Transplantation volume 35, pages 419–420 (2005)Cite this article

AL amyloidosis is a severe, untreatable disease related to multiorgan infiltration by β-fibrils derived from monoclonal immunoglobulin light chain (LC) (1). Cardiac involvement carries a very poor prognosis.1 We report an 18-month follow-up of a 45-year woman who successfully underwent both a cardiac and an unrelated bone marrow transplantation (BMT).

Symptoms of restrictive cardiomyopathy led to a biopsy proven diagnosis of AL amyloidosis in May 1999. At diagnosis, there was no evidence of other organ dysfunction. There was no detectable monoclonal Ig in the serum, but moderate proteinuria (0.3g/24 h) consisting mainly (70%) of a monoclonal kappa LC (uKLC). Only 2% plasma cells were identified in the bone marrow (BM), confirming the diagnosis of primary amyloidosis. After initial improvement following high-dose melphalan and autologous stem cell transplantation in July 1999, she developed congestive heart failure and cardiac arrest. She underwent orthotopic heart transplantation in July 2001 in association with quadruple immunosuppressive therapy (ATG 10 mg/kg, Tacrolimus, MMF, methylprednisolone). The postoperative course was uneventful.

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Authors and Affiliations

  1. Service d'hematologie adultes, Hopital Necker, Paris, France

    O Thaunat, G Damaj, B Varet & A Buzyn

  2. Laboratoire d'immunologie, Hopital Necker, Paris, France

    M A Alyanakian

  3. Service de chirurgie cardiaque, Hopital de la Pitié-Salpétrière, Paris, France

    S Varnous, V Bors & I Gandjbakhch

  4. Service de transplantation, Hopital Necker, Paris, France

    J P Rerolle

  5. Service d'hematologie, Hopital Ambroise Pare, Boulogne, France

    K Jondeau

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  1. O Thaunat
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  2. M A Alyanakian
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  3. S Varnous
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  4. V Bors
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  5. G Damaj
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Thaunat, O., Alyanakian, M., Varnous, S. et al. Long-term successful outcome of sequential cardiac and allogeneic bone marrow transplantations in severe AL amyloidosis. Bone Marrow Transplant 35, 419–420 (2005). https://doi.org/10.1038/sj.bmt.1704772

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