Last reviewed: November 2017
Last updated: March  2017

Summary

Definition

History and exam

Key diagnostic factors

  • age >75 years
  • male gender
  • white ancestry

Other diagnostic factors

  • hx of IgM monoclonal gammopathy of undetermined significance (MGUS)
  • FHx of B-cell lymphoma or myeloma
  • FHx of WM with or without monoclonal gammopathy of undetermined significance (MGUS) and multiple myeloma
  • fatigue
  • anorexia
  • peripheral neuropathy
  • weight loss
  • fever
  • hepatomegaly
  • splenomegaly
  • lymphadenopathy
  • Raynaud phenomenon
  • purpura
  • hemorrhage

Risk factors

  • IgM component monoclonal gammopathy of undetermined significance (MGUS)
  • FHx of B-cell lymphoproliferative disease or multiple myeloma
  • FHx of Waldenström macroglobulinemia
  • hepatitis C virus

Diagnostic investigations

1st investigations to order

  • CBC with differential
  • renal function panel
  • LFTs
  • CRP
  • high-resolution serum protein electrophoresis (SPEP) with immunofixation
  • high-resolution urine protein electrophoresis (UPEP) and immunofixation
  • serum free light chains
  • cold agglutinins and cryoglobulins
  • serum beta-2 microglobulin
  • relative serum viscosity
  • bone marrow biopsy
  • lymph node biopsy
  • CT chest, abdomen, and pelvis
Full details

Investigations to consider

  • 18F-FDG PET/CT chest, abdomen, and pelvis
  • quantitative immunoglobulins
  • skeletal survey
  • fat pad biopsy
Full details

Treatment algorithm

Contributors

Authors VIEW ALL

Assistant Professor

NYU School of Medicine

New York

NY

Disclosures

BK declares that he has no competing interests.

Associate Professor of Medicine

NYU Langone Medical Center

New York

NY

Disclosures

KH declares that he has no competing interests.

Peer reviewers VIEW ALL

Consultant

Department of Hematology

Mayo Clinic

Rochester

MN

Disclosures

SK declares that he has no competing interests.

Professor of Medicine

Chief, Section of Hematology

Department of Internal Medicine

Yale University School of Medicine

New Haven

CT

Disclosures

MD declares that he has no competing interests.

Instructor in Hematology

Department of Hematology

Hopital Huriez CHRU

Lille

France

Disclosures

XL has received lecture fees and research funding from Janssen-Cilag, Celgene, Chugai, Amgen, Novartis, Mundipharma, and Roche. XL is an author of a number of references cited in this monograph.

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