Intensive Therapy in AL Amyloidosis and Light-Chain Deposition Disease

  1. Henk M. Lokhorst, MD; and
  2. Leo F. Verdonck, MD
  1. University Hospital Utrecht; Utrecht, the Netherlands

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    IN RESPONSE:

    The case described by Mariette and associates further supports the idea that therapy for disorders associated with plasma cell dyscrasia and abnormal immunoglobulin deposits—such as AL amyloidosis, light-chain deposition disease, and probably also heavy-chain disease—should be aimed at completely eliminating the proliferation of clonal plasma cells.

    We previously described one patient [1] and treated two others who had generalized AL amyloidosis using high-dose chemoradiotherapy supported by peripheral blood stem-cell transplantation. Of these two, one remains unevaluable because of insufficient treatment duration. In the other patient, who was refractory to intermediate-dose melphalan (70 mg/m2 body surface area), evaluation 1 year after stem-cell transplantation showed that monoclonal plasma cell and light-chain excretion was no longer detectable. Furthermore, symptoms associated with amyloid deposits, such as the nephrotic syndrome and orthostatic hypotension, had greatly improved. Amyloid deposits, present in repeated bone marrow biopsies before stem-cell transplantation, had cleared completely.

    We agree with Mariette and colleagues that high-dose chemoradiotherapy supported by stem-cell transplantation should be strongly considered in patients with immunoglobulin deposition disease, unless the patient is eligible for an allogeneic transplant. The effect of grafts against myeloma, which has recently been shown (unpublished data), might result in more complete and endurable remissions in fatal plasma-cell disorders.

    Henk M. Lokhorst, MD

    Leo F. Verdonck, MD

    University Hospital Utrecht; Utrecht, the Netherlands

    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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