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Case Reports: Unusual association of diseases/symptoms
Recovery from AA amyloidosis-cardiomyopathy complexed with unicentric Castleman disease
  1. Kanako Imamura1,
  2. Sunao Kojima2,
  3. Takahisa Imamura3 and
  4. Kenichi Tsujita4
  1. 1Department of Laboratory Medicine, Kumamoto University Hospital, Kumamoto, Japan
  2. 2Department of Internal Medicine, Sakura-jyuji Yatsushiro Rehabilitation Hospital, Yatsushiro, Japan
  3. 3Department of Life Science, Shokei University, Kumamoto, Japan
  4. 4Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan
  1. Correspondence to Sunao Kojima; kojimas{at}kumamoto-u.ac.jp

Abstract

We report a case of cardiac amyloid A (AA) amyloidosis due to unicentric Castleman disease (UCD) in a patient whose cardiac function was restored 15 years after surgical resection of the mesenteric lymph node lesion. A man in his 40s had recurrent palpitations and fainting spells. ECG revealed torsades de pointes. Increased C-reactive protein, interleukin-6 and serum AA levels, and marked concentric thickening of the left ventricular (LV) wall with diastolic restrictive filling pattern were observed. Duodenal biopsy revealed AA amyloid deposits. He had a mesenteric tumour, comprising many plasma cells. He was diagnosed with plasma cell-type UCD associated with secondary AA amyloidosis. C-reactive protein, interleukin-6 and serum AA levels were normalised 2 months postresection. Episodes of lethal ventricular arrhythmias decreased. LV wall thickness was gradually reduced. Approximately 15 years postresection, the LV wall thickness nearly normalised and ventricular arrhythmias disappeared. Better outcomes are expected following surgical tumour resection.

  • Arrhythmias
  • Haematology (incl blood transfusion)
  • Cardiovascular medicine
  • Surgery
  • Heart failure

https://doi.org/10.1136/bcr-2022-250338

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    Footnotes

    • Contributors First Author: KI, Department of Laboratory Medicine, Kumamoto University Hospital, Kumamoto, Japan. Co First Author: SK, Department of Internal Medicine, Sakurajyuji Yatsushiro Rehabilitation Hospital, Yatsushiro, Japan. Second Author: TI, Department of Life Science, Shokei University, Kumamoto, Japan. Third Author: KT, Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan. We hereby certify that submitted work, titled 'Recovery from AA amyloidosis-cardiomyopathy complexed with unicentric Castleman disease’, to BMJ Case Reports journal is written by above four authors. Any work that is not our work has been referenced. All references and sources of information have been clearly cited within the text. We can confirm that KI and SK gathered the clinical data including the photograph following gaining written consent for the patient. KI and SK contributed equally in writing the paper following planning, researching and interpreting the results. Hence, KI and SK are considered as first authors. TI contributed in writing of the paper, editing and optimising the image. TI is considered as the second author. KT contributed in submitting the paper to BMJ Case Reports journal. KT is considered as the third author.

    • Funding This study was funded by Grants-in-Aid for Scientific Research from the Japan Society for the Promotion of Science (21K07356).

    • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

    • Competing interests None declared.

    • Provenance and peer review Not commissioned; externally peer reviewed.