What are the clinical guidelines for staging and risk assessment of Waldenstrom macroglobulinemia (WM)?

Answer

Both guidelines concur that the initial workup for WM includes the following^{[55, 54]}:

Complete blood cell (CBC) count
Serum chemistry
Beta-2 microglobulin (B2M)
Serum protein electrophoresis
IgM quantification
CT scans with contrast when possible

Although CXCR4 mutations are found in approximately 30% of patients with WM, testing for this genetic mutation is recommended only for patients considering treatment with ibrutinib.^{[55, 54]}

A neurologist should be consulted for neuropathy, since it may not be associated with WM. Amyloidosis should be ruled out in patients presenting with nephrotic syndrome or unexplained cardiac issues. Amyloidosis is uncommon in patients with WM. If it is suspected, a fat aspirate stained with Congo red and cardiac and renal biomarkers should be evaluated.^{[55, 54]}

A retinal examination is recommended in patients with hyperviscosity symptoms.^{[55, 54]}

NCCN finds the following additional tests useful in selected patients^[54]:

Serum viscosity
Hepatitis C testing and cryocrit for patients with suspected cryoglobulinemia; if cryocrit is positive, initial serum IgM test should be repeated and all subsequent IgM levels should be obtained in warm conditions
Hepatitis B testing, if treatment with rituximab is planned
Coagulation testing should be considered in patients with symptoms of excess bruising or bleeding; von Willebrand disease (VWD) testing is indicated only if clinical bleeding and bruising are present

The ESMO guidelines include the following additional recommendations:

Anemia should prompt consideration of Coombs testing, cold agglutinins, cryoglobulins, and iron status.
Risk assessment is currently based on the International Prognostic Scoring System for WM (IPSSWM).

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Media Gallery

Purpura from Waldenström macroglobulinemia is evident in the forearm of a 65-year-old man who presented with a purpuric rash on all of his extremities. Although the patient had a history of hepatitis C, the possibility of hepatitis C cryoglobulinemia was excluded because the rash extended well beyond the hands and feet, and blood testing identified a type I cryoglobulinemia. Image courtesy of Jason Kolfenbach, MD, and Kevin Deane, MD, Division of Rheumatology, University of Colorado Denver School of Medicine.

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Author

Karen Seiter, MD Professor, Department of Internal Medicine, Division of Oncology/Hematology, New York Medical College

Karen Seiter, MD is a member of the following medical societies: American Association for Cancer Research, American College of Physicians, American Society of Hematology

Disclosure: Received honoraria from Novartis for speaking and teaching; Received consulting fee from Novartis for speaking and teaching; Received honoraria from Celgene for speaking and teaching.

Coauthor(s)

Doris Ponce, MD Fellow, Department of Hematology/Oncology, New York Medical College

Doris Ponce, MD is a member of the following medical societies: American College of Physicians, American Medical Association, American Society of Hematology, American Society of Clinical Oncology

Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD Professor Emeritus, Department of Medicine, Division of Hematologic Malignancies and Hematopoietic Stem Cell Transplantation, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American Society of Clinical Oncology, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, New York Academy of Sciences

Disclosure: Nothing to disclose.

Acknowledgements

Wendy Hu, MD Consulting Staff, Department of Hematology/Oncology and Bone Marrow Transplantation, Huntington Memorial Medical Center

Wendy Hu, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Physicians, American Society for Blood and Marrow Transplantation, American Society of Hematology, and Physicians for Social Responsibility