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Recovery from AA amyloidosis-cardiomyopathy complexed with unicentric Castleman disease

Kanako Imamura1, Sunao Kojima2, Takahisa Imamura3

  • 1Department of Laboratory Medicine, Kumamoto University Hospital, Kumamoto, Japan.

BMJ Case Reports
|August 18, 2022
PubMed

Insights

Cardiac amyloidosis (AA) linked to Castleman disease (UCD) can be reversed. Surgical removal of the UCD tumor led to near-complete cardiac function restoration 15 years later, highlighting effective treatment for this rare condition.

Area of Science:

  • Cardiology
  • Oncology
  • Pathology

Background:

  • Cardiac amyloidosis (AA) is a serious condition often linked to chronic inflammation.
  • Unicentric Castleman disease (UCD) is a rare lymphoproliferative disorder that can trigger secondary AA amyloidosis.
  • Left ventricular (LV) dysfunction and arrhythmias are common manifestations of cardiac amyloidosis.

Observation:

  • A middle-aged male patient presented with palpitations and syncope due to ventricular arrhythmias.
  • Diagnostic workup revealed left ventricular hypertrophy and restrictive filling pattern, consistent with cardiac amyloidosis.
  • The patient was diagnosed with plasma cell-type UCD and secondary AA amyloidosis.

Findings:

  • Surgical resection of the mesenteric UCD tumor led to normalization of inflammatory markers (C-reactive protein, interleukin-6, serum AA) within 2 months.
  • A significant reduction in ventricular arrhythmias and progressive improvement in LV wall thickness were observed post-surgery.
  • Fifteen years after resection, cardiac function nearly normalized, and ventricular arrhythmias resolved.

Implications:

  • Surgical resection of the primary UCD lesion is a potentially curative treatment for associated cardiac AA amyloidosis.
  • This case demonstrates the long-term efficacy of surgical intervention in reversing cardiac dysfunction caused by secondary amyloidosis.
  • Early diagnosis and treatment of UCD may prevent irreversible cardiac damage and improve patient outcomes.

Keywords:
ArrhythmiasCardiovascular medicineHaematology (incl blood transfusion)Heart failureSurgery

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