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Autologous PBSCT in patients with cardiac amyloidosis

Bone Marrow Transplantation volume 26, pages 588–589 (2000)Cite this article

We read with interest the recent review by Gertz et al,1 expressing concern about patient selection and toxicity of high-dose therapies (HDT) for patients with primary amyloidosis. Whilst we agree on many points, we would argue that measures taken to modify treatment-related morbidity and mortality, as illustrated in our following case report, may extend the applicability of HDT to poor risk patients.

Our patient, a 57-year-old male, presented with symptomatic cardiac failure which required treatment with diuretics and angiotensin converting enzyme inhibitor for 3 months prior to autologous HDT. A cardiac biopsy showed amyloid and echocardiography showed poor diastolic function as indicated by a left ventricular end diastolic pressure of 28 mmHg. His ejection fraction was 37%. A serum IgG lambda paraprotein (total IgG 18.3 g/l) was present as were trace amounts in the urine (urine protein 0.01 g/l) but a bone marrow aspirate and trephine biopsy did not show increased plasma cells or evidence of amyloidosis. Renal function was normal. Asymptomatic hepatomegaly was present with a raised bilirubin (50 μmol/l, normal <21) and a raised alkaline phosphatase (262 IU/l, normal <120) and hepatic biopsy confirmed amyloidosis. No gastrointestinal or neurological symptoms were present and biopsies of these and other organs were not performed. These features were diagnostic of primary amyloid with predominant cardiac manifestations but involvement of at least two major organ systems, predictive of both a poor prognosis without specific treatment and of high morbidity and mortality following HDT. Our patient was also a low priority candidate for cardiac allograft and we proceeded to autologous HDT following advice from Dr Comenzo that we should consider elective patient admission to our intensive therapy unit.

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References

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Authors and Affiliations

  1. Department of Haematology, Christchurch Hospital, Christchurch, New Zealand

    JM Stevens, WN Patton, DC Heaton & RL Spearing

  2. Department of Intensive Care, Christchurch Hospital, Christchurch, New Zealand

    G Shaw & D Bowie

  3. Department of Cardiology, Christchurch Hospital, Christchurch, New Zealand

    D Smyth

  4. Haematology Services, Memorial Sloan-Kettering Cancer Center, New York, USA

    R Comenzo

Authors
  1. JM Stevens
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  2. WN Patton
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  3. DC Heaton
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  4. RL Spearing
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  5. G Shaw
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  6. D Bowie
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  7. D Smyth
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  8. R Comenzo
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Stevens, J., Patton, W., Heaton, D. et al. Autologous PBSCT in patients with cardiac amyloidosis. Bone Marrow Transplant 26, 588–589 (2000). https://doi.org/10.1038/sj.bmt.1702551

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