Intensive Therapy in AL Amyloidosis and Light-Chain Deposition Disease

  1. Xavier Mariette, MD, PhD;
  2. Jean-Pierre Clauvel, MD; and
  3. Jean-Claude Brouet, MD, PhD
  1. Hopital Saint-Louis; 75010 Paris; France

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    TO THE EDITOR:

    Dr. van Buren and colleagues [1] reported clinical remission in a patient with AL amyloidosis after intensive chemotherapy followed by syngeneic bone marrow transplantation. Light-chain deposition disease, another immunoglobulinrelated complication in multiple myeloma, usually worsens despite conventional therapy [2]. We observed a striking recovery from multiorgan failure and clearing of light-chain deposits after high-dose chemoradiotherapy supported by peripheral blood stem-cell autograft in a patient with multiple myeloma and widespread light-chain deposits.

    A 42-year-old man presented in April 1991 with cardiac and renal failure (creatinine level, 4 mg/dL). The patient's liver was enlarged, and the alkaline phosphatase level was increased (410 IU/L; normal, less than 280 IU/L). Monoclonal κ light chains were detected in urine, and a bone marrow smear showed 32% atypical plasma cells. Lytic lesions were seen on skeletal radiographs. Characteristic deposits of κ light chains were found by immunofluorescence on cardiac, liver, and renal biopsy specimens. No amyloidosis was found.

    Semi-intensive chemotherapy with cyclophosphamide was begun and followed by collection of peripheral blood stem cells. After three courses of cyclophosphamide and prednisone, intensive chemotherapy and total body irradiation was administered in October 1991 [3]. Blood stem cells were used for autografting.

    One year after intensive therapy, the patient's multiple myeloma was in complete remission, as shown by a normal bone marrow smear, absence of urinary κ chains, and normal serum immunofixation. An echocardiogram and liver test results were normal, and the serum creatinine level was 1.5 mg/dL. Examination of biopsy specimens showed no light-chain deposits in the heart and scant deposits in the liver.

    This case shows that intensive therapy can clear widespread light-chain deposits and restore organ function and that it should be considered in patients with severe immunoglobulin deposition disease.

    Xavier Mariette, MD, PhD

    Jean-Pierre Clauvel, MD

    Jean-Claude Brouet, MD, PhD

    Hopital Saint-Louis; 75010 Paris; France

    The Editors welcome submissions for possible publication in the Letters section. Authors of letters should:

    •Include no more than 300 words of text, three authors, and five references

    •Type with double-spacing

    •Send three copies of the letter, an authors' form signed by all authors, and a cover letter describing any conflicts of interest related to the contents of the letter.

    Letters commenting on an Annals article will be considered if they are received within 6 weeks of the time the article was published. Only some of the letters received can be published. Published letters are edited and may be shortened; tables and figures are included only selectively. Authors will be notified that the letter has been received. If the letter is selected for publication, the author will be notified about 3 weeks before the publication date. Unpublished letters cannot be returned.

    Annals welcomes electronically submitted letters.

    References

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