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Volume 2022, Issue 1
  • ISSN: 0253-8253
  • EISSN: 2227-0426

Abstract

Cardiac amyloidosis is a rare disorder caused by the myocardial deposition of abnormal fibrils. A 52-year-old man was referred to our center with clinical features of heart failure, after cardiac magnetic resonance imaging showed restrictive cardiomyopathy. Abdominal fat pad biopsy showed features of amyloidosis, and after hematological workup, he was diagnosed with Waldenstrom macroglobulinemia (WM). He was initiated on a rituximab-based chemotherapy regimen, and his cardiac function was assessed serially. Because of non-response, he was switched to a bortezomib-based regimen. Unfortunately, three days into this regimen, the patient died. WM is a rare plasma cell dyscrasia with a nonspecific presentation. It uncommonly presents with sequelae of amyloidosis–the IgM subtype of amyloid-light chain (AL) amyloidosis. Diagnostic delays are common, contributing to an already poor prognosis. Amyloidosis in WM requires urgent treatment – clonal chemotherapy, and supportive cardiac care in heart involvement. Bortezomib-based regimens are commonly recommended, with diuretics as the mainstay for cardiac treatment. However, in most advanced cases, the prognosis is poor; thus, a high degree of suspicion is necessary for early diagnosis. This case illustrates the possible presentation of cardiac amyloidosis as a rare malignancy.

© 2022 Lobo, Bhat, Anandram, Devi, Rao, Vinister, Lobo, Ross, licensee HBKU Press.
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2022-03-22
2024-12-27
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References

  1. Witteles RM, Liedtke M. AL Amyloidosis for the cardiologist and oncologist. JACC: Cardiooncology. 2019;1:117–30.
     [Google Scholar]
  2. Muchtar E, Blauwet LA, Gertz MA. Restrictive cardiomyopathy: genetics, pathogenesis, clinical manifestations, diagnosis, and therapy. Circ Res. 2017;121:819–37.
     [Google Scholar]
  3. Lousada I, Comenzo RL, Landau H, Guthrie S, Merlini G. Light chain amyloidosis: patient experience survey from the amyloidosis research consortium. Adv Ther. 2015;32:920–8.
     [Google Scholar]
  4. Owen RG, Pratt G, Auer RL, et al. Guidelines on the diagnosis and management of Waldenström macroglobulinaemia. Br J Haematol. 2014;165:316–33.
     [Google Scholar]
  5. Gertz MA, Merlini G, Treon SP. Amyloidosis and Waldenström's macroglobulinemia. Hematology Am Soc Hematol Educ Program. 2004;2004:257–82.
     [Google Scholar]
  6. Zanwar S, Abeykoon JP, Ansell SM, et al. Primary systemic amyloidosis in patients with Waldenström macroglobulinemia. Leukemia. 2019;33:790–4.
     [Google Scholar]
  7. Munjewar C, Agrawal R, Sharma S. Cardiac amyloidosis: a report of two cases. Indian Heart J. 2014;66:473–6.
     [Google Scholar]
  8. Vrana JA, Theis JD, Dasari S, Mereuta OM, Dispenzieri A, Zeldenrust SR, et al. Clinical diagnosis and typing of systemic amyloidosis in subcutaneous fat aspirates by mass spectrometry-based proteomics. Haematologica. 2014;99:1239–47.
     [Google Scholar]
  9. Dimopoulos MA, Kastritis E. How I treat Waldenström macroglobulinemia. Blood. 2019;134:2022–35.
     [Google Scholar]
  10. Dimopoulos MA, Roussou M, Kastritis E, et al. Primary treatment of Waldenstrom's macroglobulinemia with dexamethasone, rituximab and cyclophosphamide (DRC): final analysis of a phase II study. Blood (ASH Annual Meeting Abstracts). 2012;120: 438.
     [Google Scholar]
  11. Sidana S, Larson DP, Greipp PT, He R, McPhail ED, Dispenzieri A, et al. IgM AL amyloidosis: delineating disease biology and outcomes with clinical, genomic and bone marrow morphological features. Leukemia. 2020;34:1373–82.
     [Google Scholar]
/content/journals/10.5339/qmj.2022.7
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Rare Complication of a Rare Malignancy: Case Report of Cardiac Amyloidosis Secondary to Waldenstrom Macroglobulinemia
Qatar Medical Journal 2022, 7 (2022); https://doi.org/10.5339/qmj.2022.7
/content/journals/10.5339/qmj.2022.7
/content/journals/10.5339/qmj.2022.7
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  • Article Type: Case Report
Keyword(s): amyloidosis, chemotherapy, heart failure, monoclonal gammopathy and restrictive cardiomyopathy

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