Last reviewed: 6 Nov 2021
Last updated: 13 Apr 2021

Summary

Definition

History and exam

Key diagnostic factors

  • jugular venous distention
  • lower extremity edema
  • periorbital purpura
  • eyelid petechiae
  • macroglossia

Other diagnostic factors

  • fatigue
  • weight loss
  • dyspnea on exertion
  • peripheral neuropathy
  • autonomic neuropathy
  • claudication
  • nausea or vomiting
  • abdominal cramps
  • alternating bowel habit
  • lightheaded/orthostatic hypotension
  • submandibular salivary gland enlargement
  • hepatomegaly
  • shoulder pad sign
  • diffuse muscular weakness
  • Carpal tunnel syndrome

Risk factors

  • monoclonal gammopathy of undetermined significance (MGUS)
  • inflammatory polyarthropathy
  • chronic infections
  • inflammatory bowel disease
  • familial periodic fever syndromes
  • Castleman disease

Diagnostic investigations

1st investigations to order

  • serum immunofixation
  • urine immunofixation
  • immunoglobulin free light chain assay
  • CBC
  • comprehensive metabolic profile
  • 24-hour urine collection

Investigations to consider

  • tissue biopsy
  • mass spectrometry
  • immuno-electron microscopy
  • immuno-histochemical studies
  • genetic testing
  • serum troponin level
  • N-terminal pro-B-type natriuretic peptide
  • beta-2-microglobulin
  • ECG
  • echocardiogram (with tissue Doppler and global longitudinal strain)
  • cardiac MRI
  • scintigraphy (bone)

Treatment algorithm

Contributors

Authors

Morie A. Gertz, MD, MACP
Morie A. Gertz

Seidler Jr. Professor of Medicine

Consultant in Hematology

Chair Emeritus of the Department of Medicine

Mayo Distinguished Clinician

Mayo Clinic College of Medicine

Rochester

MN

Disclosures

MAG has received personal fees from Ionis/Akcea, Alnylam, Prothena, Janssen, Annexon, Appellis, Amgen, Medscape, Physicians Education Resource, and Research to Practice; and grants and personal fees from Spectrum. MAG has received personal fees from AbbVie and Celgene for a Data Safety Monitoring board, and personal fees from Sanofi for workforce training. MAG has received speaker fees from Teva, Johnson & Johnson, Medscape, and DAVA Oncology; and advisory board fees from Pharmacyclics and Procalara. MAG has participated in the development of educational materials for the i3Health Educational Program development. MAG has received royalties from Springer Publishing. MAG has received grant funding from the Amyloidosis Foundation, International Waldenstrom's Macroglobulinemia Foundation, and National Cancer Institute (SPORE MM SPORE 5P50 CA186781-04). MAG has stock options in Aurora Bio. MAG is an author of references cited in this topic.

Peer reviewers

Donna Reece, MD

Associate Professor of Medicine

Director

Program for Multiple Myeloma and Related Diseases

Princess Margaret Hospital

Toronto

Ontario

Canada

Disclosures

DR has been reimbursed by Millennium Pharmaceuticals, Inc and Johnson & Johnson, the manufacturers of bortezomib, for attending several conferences, for speaking at educational meetings, and for consulting work. She has also been reimbursed by Celgene, the manufacturer of lenalidomide and thalidomide, for attending several symposia and serving as a speaker.

Jeffrey Zonder, MD

Assistant Professor of Medicine and Oncology

Division of Hematology/Oncology

Wayne State University School of Medicine

Barbara Ann Karmanos Cancer Institute

Detroit

MI

Disclosures

JZ declares that he has no competing interests.

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