Waldenstrom Macroglobulinemia Staging
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Recommended diagnostic studies to evaluate a suspected diagnosis of Waldenstrom macroglobulinemia/lymphoplasmacytic lymphoma are as follows [1] :
History and physical examination with complete neurologic and ophthalmologic examination
Serum protein electrophoresis (SPEP)
Quantitative serum immunoglobulin G, immunoglobulin A, and immunoglobulin M (IgM) (total levels of serum immunoglobulins should be assessed with nephelometry)
Serum immunofixation
Twenty-four–hour urine collection for protein electrophoresis
Serum free light-chain assay
Bone marrow biopsy with cytogenetics, fluorescence in situ hybridization (FISH) (for lymphoma and myeloma), and flow cytometry (should include CD19, CD20, CD38, and CD138)
Serum beta2-microglobulin
CT scanning of the chest, abdomen, and pelvis (to assess for lymphadenopathy and splenomegaly)
Complete blood cell (CBC) count and serum chemistries
Serum viscosity
Autoantibodies, Coombs test, cold agglutinins, cryoglobulins, anti–myelin-associated glycoprotein (MAG) antibodies (based on history and physical finding)
Each of the following positive prognostic factors is assigned a value of one, with the final sum used to assess prognostic score and predicted survival: [2]
IgM level >7 g/dL
Age >65 years
Hemoglobin level ≤11.5 g/dL
Platelet count ≤100,000/µL
Beta2-microglobulin level >3 µg/mL
The sum is then used to estimate prognosis and survival (see Table 1).
Table 1. Assessment of Prognostic Score and Predicted Survival (Open Table in a new window)
Score
Stratum
Median Survival (mo)
0-1a
Low
142
2b
Intermediate
96
>2
High
43
a Except age.
b Age >65 years is also intermediate risk.
Buske C, Leblond V, Dimopoulos M, Kimby E, Jäger U, Dreyling M, et al. Waldenstrom’s macroglobulinaemia: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2013 Oct. 24 Suppl 6:vi155-9. [Medline]. [Full Text].
Morel P, Duhamel A, Gobbi P, Dimopoulos MA, Dhodapkar MV, McCoy J, et al. International prognostic scoring system for Waldenstrom macroglobulinemia. Blood. 2009 Apr 30. 113(18):4163-70. [Medline].
Score
Stratum
Median Survival (mo)
0-1a
Low
142
2b
Intermediate
96
>2
High
43
a Except age.
b Age >65 years is also intermediate risk.
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 at 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 of Hematology, American Society for Blood and Marrow Transplantation
Disclosure: Received honoraria from Genentech for speaking and teaching.
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.
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
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