Clinical Remission after Syngeneic Bone Marrow Transplantation in a Patient with AL Amyloidosis

  1. Marjolein van Buren, MD;
  2. Ronald J. Hene, MD;
  3. Leo F. Verdonck, MD;
  4. Fred J. Verzijlbergen, MD; and
  5. Henk M. Lokhorst, MD
  1. From the University Hospital Utrecht, Utrecht, the Netherlands. Requests for Reprints: Henk M. Lokhorst, MD, Department of Hematology, University Hospital Utrecht (G03.647), Heidelberglaan 100, 3584 CX Utrecht, the Netherlands.

    Systemic amyloidosis is characterized by accumulation of an eosinophilic amorphous material. Two proteins compose the deposits, one of which is the α-glycoprotein amyloid P. The nature of the other protein depends on the underlying cause of the illness (light chains in cases of AL amyloidosis associated with monoclonal gammopathy or amyloid A in cases of AA amyloidosis associated with chronic inflammatory disease).

    In secondary AA amyloidosis, resorption may occur when the stimulus for amyloid formation is eliminated [1, 2]. It is generally agreed that no effective therapy exists for AL amyloidosis (formerly known as primary systemic amyloidosis), but a few patients have shown responses to alkylating agent-based chemotherapy [3]. The median survival after diagnosis is only 14 months. In patients with multiple myeloma, new therapeutic strategies involving higher doses of cytotoxic drugs sometimes combined with total body irradiation and autologous or allogeneic bone marrow transplantation have significantly increased the rate of complete remission [4, 5]. On the basis of these results we performed a syngeneic bone marrow transplantation in a 32-year-old patient with AL amyloidosis who had not benefited from treatment with melphalan and prednisone.

    Figure 1. Abdominal anterior view 24 hours after injection of Iodine-123-labeled serum albumin precursor. The uptake in the spleen is high; uptake in the liver is slightly lower. There is scarcely any background activity. Abdominal anterior view 24 hours after injection of Iodine-123-labeled serum amyloid precursor. A pronounced background (heart and blood vessels), shows the lower uptake in all tissues. Activity in spleen, liver, and kidneys (which are now visible because of the lower level of activity in the spleen and the liver) is about equal. Abbreviations: H = heart; K = kidney; L = liver; S = spleen. Investigation before and 2 years after bone marrow transplantation.Top.Bottom.

    Case Report

    In April 1991, a 32-year-old woman was hospitalized with edema and increased fatigue. She had orthostatic hypotension; her supine blood pressure was 105/60 …

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