Waldenstrom Macroglobulinemia Staging

Updated: Dec 21, 2020

Author: Joseph M Tuscano, MD; Chief Editor: Emmanuel C Besa, MD

Diagnostic Studies

Recommended diagnostic studies to evaluate a suspected diagnosis of Waldenstrom macroglobulinemia/lymphoplasmacytic lymphoma are as follows[1] :

History and physical examination
Review of systems (B symptoms, hepatosplenomegaly, lymphadenopathy, neuropathy, Raynaud syndrome, rash, dyspnea, hyperviscosity symptoms including mucosal bleeding, blurred vision, headaches and dizziness; include fundoscopic examination to evaluate for retinal-vein engorgement, flame-shaped hemorrhages, papilledema if IgM is high and hyperviscosity is suspected)
Complete blood count
Complete metabolic panel
Serum immunoglobulin levels (IgA, IgG, IgM)
Serum and urine electrophoresis with immunofixation
Serum beta2-microglobulin level
Viral serology (hepatitis B and C viruses and HIV)
Bone marrow aspiration and biopsy with immunohistochemistry (IHC; required for diagnosis), flow cytometry (optional; consider if IHC not available), and testing for MYD88L265P gene mutation
Computed tomography scan of the chest, abdomen, and pelvis (to assess for lymphadenopathy and hepatosplenomegaly; if clinically indicated and in all patients being considered for therapy)

Optional studies, if clinically indicated, include the following:

Cryoglobulins
Cold agglutinin titer
Serum viscosity
Screening for acquired von Willebrand disease
24-h urine protein quantification
Serum free light chain assay
N-terminal pro b-type natriuretic peptide, cardiac troponins
Electromyogram, anti–myelin-associated globulin antibody (anti-MAG), anti-ganglioside M1 (anti-GM1); to be ordered in consultation with a neurologist

Staging/Prognostic Scoring System for Waldenstrom Macroglobulinemia/Lymphoplasmacytic Lymphoma

Kastritis et al developed and validated an updated international prognostic score system for Waldenström macroglobulinemia.[2] The system uses the following criteria:

Age: ≤65 years: 0 points, 66-75 years: 1 point; ≥75 years: 2 points
Beta2-microglobulin >4 mg/L: 1 point
Lactate dehydrogenase >250 IU/L: 1 point
Serum albumin < 3.5 g/dL: 1 point

Based on the total score, the system classifies patients into 5 risk groups (see table 1 below).

Table 1. Prognostic scoring system for Waldenstrom macroglobulinemia (Open Table in a new window)

Score	Stage	3-year WM-related mortality	5-year overall survival	10-year overall survival
0	Very low	0%	95%	84%
1	Low	10%	86%	59%
2	Intermediate	14%	78%	37%
3	High	38%	47%	19%
4-5	Very high	48%	36%	9%

Questions & Answers

Overview

How is Waldenstrom macroglobulinemia/lymphoplasmacytic lymphoma diagnosed?

How is the prognostic score for Waldenstrom macroglobulinemia/lymphoplasmacytic lymphoma determined?

Author

Joseph M Tuscano, MD Associate Chief, Department of Hematology/Oncology/Internal Medicine, Veterans Administration Northern California System of Clinics; Professor, Department of Internal Medicine, Division of Hematology/Oncology, University of California, Davis, School of Medicine

Joseph M Tuscano, MD is a member of the following medical societies: American Association of Immunologists, American College of Physicians, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Hematology

Disclosure: Nothing to disclose.

Coauthor(s)

Christina Poh, MD Clinical Assistant Professor of Medicine, Division of Medical Oncology (Lymphoma), University of Washington School of Medicine

Christina Poh, MD is a member of the following medical societies: American Association for Cancer Research, American Society of Clinical Oncology, American Society of Hematology, Hemostasis and Thrombosis Research Society

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Incyte Morphosys<br/>Received research grant from: Incyte Morphosys.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Christopher D Braden, DO Hematologist/Oncologist, Chancellor Center for Oncology at Deaconess Hospital; Medical Director, Deaconess Hospital Outpatient Infusion Centers; Chairman, Deaconess Hospital Cancer Committee

Christopher D Braden, DO is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology

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.

Waldenstrom Macroglobulinemia Staging

Diagnostic Studies

Staging/Prognostic Scoring System for Waldenstrom Macroglobulinemia/Lymphoplasmacytic Lymphoma

Questions & Answers

Contributor Information and Disclosures

References