Systemic Amyloidosis Caused by Monoclonal Immunoglobulins: Soft Tissue and Vascular Involvement

James E. Hoffman, Naomi G. Dempsey, Vaishali Sanchorawala

Research output: Contribution to journalReview articlepeer-review

1 Scopus citations


Clinical features of soft tissue amyloid light-chain (AL) amyloidosis include macroglossia, arthropathy, muscle pseudohypertrophy, skin plaques, and carpal tunnel syndrome. Vascular manifestations of AL amyloid include periorbital ecchymosis, jaw or limb claudication, and even myocardial infarction caused by occlusion of small vessel coronary arteries. Some of these features, such as macroglossia, periorbital ecchymosis, and the so-called shoulder-pad sign, are pathognomonic for AL amyloidosis. These findings may be the initial presenting features of the disease, and the recognition of these red flag symptoms is very important for the diagnosis and early intervention on the underlying plasma cell disease.

Original languageEnglish (US)
Pages (from-to)1099-1113
Number of pages15
JournalHematology/Oncology Clinics of North America
Issue number6
StatePublished - Dec 2020


  • Amyloid arthropathy
  • Amyloid myopathy
  • Carpal tunnel syndrome
  • Macroglossia
  • Periorbital ecchymosis
  • Raccoon eyes
  • Shoulder-pad sign

ASJC Scopus subject areas

  • Hematology
  • Oncology


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