AA amyloidosis associated with Castleman disease: A case report and review of the literature

Medicine (Baltimore). 2020 Feb;99(6):e18978. doi: 10.1097/MD.0000000000018978.


Rationale: AA amyloidosis (AA) is caused by a wide variety of inflammatory states, but is infrequently associated with Castleman disease (CD). CD describes a heterogeneous group of hematologic disorders that share characteristic lymph node histopathology. CD can present with a solitary enlarged lymph node (unicentric CD, UCD) or with multicentric lymphadenopathy (MCD), constitutional symptoms, cytopenias, and multiple organ dysfunction due to an interleukin-6 driven cytokine storm.

Patient concerns: We are reporting a case of a 26-year-old woman with no significant past medical history who presented with a 3-month history of fatigue and an unintentional 20-pound weight loss.

Diagnosis: A CT-scan of the abdomen and pelvis revealed hepatosplenomegaly and a mesenteric mass. Congo Red staining from a liver biopsy showed apple-green birefringence and serum markers were suggestive of an inflammatory process. Post-excision examination of the resected mass revealed a reactive lymph node with follicular hyperplasia with kappa and lambda stains showing polyclonal plasmacytosis consistent with CD.

Interventions: The patient underwent surgery to remove the affected lymph node.

Outcomes: IL-6, anemia, leukocytosis, and thrombocytosis resolved or normalized 2 weeks after resection; creatinine normalized 9 months postsurgery. Twenty two months post-surgery her IFN-γ normalized, her fatigue resolved, her proteinuria was reduced by >90% and she had returned to her baseline weight.

Lessons: Our case and literature review suggest that patients presenting with UCD or MCD along with organ failure should prompt consideration of concurrent AA amyloidosis.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Amyloidosis / blood
  • Amyloidosis / diagnosis
  • Amyloidosis / diagnostic imaging
  • Amyloidosis / etiology*
  • Castleman Disease / blood
  • Castleman Disease / complications*
  • Castleman Disease / diagnosis
  • Fatigue / etiology
  • Female
  • Humans
  • Peritoneal Diseases / blood
  • Peritoneal Diseases / diagnosis
  • Peritoneal Diseases / diagnostic imaging
  • Peritoneal Diseases / etiology*
  • Serum Amyloid A Protein / analysis
  • Tomography, X-Ray Computed
  • Weight Loss


  • Serum Amyloid A Protein