Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2016 Jun;29(2):179-186.
doi: 10.1016/j.beha.2016.08.014. Epub 2016 Aug 23.

Prognostic factors and indications for treatment of Waldenström's Macroglobulinemia

Robert A Kyle  1 , Stephen M Ansell  2 , Prashant Kapoor  2
Affiliations
Review

Prognostic factors and indications for treatment of Waldenström's Macroglobulinemia

Robert A Kyle et al. Best Pract Res Clin Haematol. 2016 Jun.

Abstract

Waldenström's Macroglobulinemia (WM) is characterized by the presence of an IgM monoclonal protein regardless of its size, 10% or more bone marrow infiltration by small lymphocytes with a plasmacytoid or plasma cell differentiation. These cells usually have the following markers: IgM+, CD5-, CD10-, CD19+, CD20+ and CD23-. Chronic lymphocytic leukemia as well as other lymphoproliferative disorders such as mantle cell, marginal zone and mucosa-associated lymphoid tissue (MALT) lymphoma must be excluded. Weakness or fatigue from anemia, fever, night sweats, or weight loss represent the most common symptoms. Hepatosplenomegaly may be a major feature. Anemia, thrombocytopenia, hyperviscosity or peripheral neuropathy may be prominent features. Systemic amyloidosis, renal insufficiency and cryoglobulinemia may also be seen. WM must be differentiated from smoldering Waldenström's Macroglobulinemia (SWM) which is an intermediate disease state characterized by an IgM protein ≥3 g/dL and/or a bone marrow containing ≥10% bone marrow lymphoplasmacytic infiltration but no end-organ damage such as symptomatic anemia, constitutional symptoms, hyperviscosity, symptomatic hepatosplenomegaly or lymphadenopathy.

Keywords: IgM monoclonal gammopathy; Indolent lymphoma; Lymphoplasmacytic lymphoma; Lymphoproliferative disorder.

PubMed Disclaimer

Figures

Fig. 1
Fig. 1
Probability of progression in 213 patients. Patients were residents of southeastern Minnesota in whom monoclonal gammopathy of undetermined significance (MGUS) of IgM class was diagnosed from 1960 through 1994. Curve shows probability of progression of MGUS to lymphoma, Waldenström macroglobulinemia, primary amyloidosis, or chronic lymphocytic leukemia. Bars show 95% confidence intervals. Numbers at bottom of the horizontal axis are numbers of patients at risk at each interval. This research was originally published in Blood. Kyle RA et al., Long-Term follow-up of IgM monoclonal gammopathy of undetermined significance. Blood. 2003;Vol 102:3759-3764. © the American Society of Hematology.
Fig. 2
Fig. 2
Competitive model in 213 patients. Patients were residents of southeastern Minnesota in whom monoclonal gammopathy of undetermined significance (MGUS) of IgM class was diagnosed from 1960 through 1994. Upper curve shows probability of dying of nonlymphoid diseases. Lower curve shows probability of progression to lymphoma or a related disorder. This research was originally published in Blood. Kyle RA et al., Long-Term follow-up of IgM monoclonal gammopathy of undetermined significance. Blood. 2003;Vol 102:3759-3764. © the American Society of Hematology.
Fig. 3
Fig. 3
Relative risk of disease progression in 213 patients. Patients were residents of southeastern Minnesota I whom monoclonal gammopathy of undetermined significance (MGUS) of IgM class as diagnosed from 1960 through 1994. Risk is by monoclonal protein value at diagnosis. Error bars show 95% CI. This research was originally published in Blood. Kyle RA et al., Long-Term follow-up of IgM monoclonal gammopathy of undetermined significance. Blood. 2003;Vol 102:3759-3764. © the American Society of Hematology.
Fig. 4
Fig. 4
Cumulative probability of progression to symptomatic WM requiring therapy, amyloidosis or lymphoma in the cohort of 48 patients with SWM. This research was originally published in Blood. Kyle RA et al., Progression in smoldering Waldenström macroglobulinemia: long-term results. Blood. 2012;Vol 119:4462-4466. © the American Society of Hematology.
Fig. 5
Fig. 5
Probability of progression to symptomatic WM requiring therapy on the basis of lymphoplasmacytic bone marrow infiltration. This research was originally published in Blood. Kyle RA et al., Progression in smoldering Waldenström macroglobulinemia: long-term results. Blood. 2012;Vol 119:4462-4466. © the American Society of Hematology.
See this image and copyright information in PMC

References

    1. Waldenstrom J. Incipient myelomatosis or "essential" hyperglobulinemia with fibrinogenopenia: A new syndrome? Acta Med Scand. 1944;216:433–4. [Editorial]
    1. Treon SP, Xu L, Yang G, Zhou Y, Liu X, Cao Y, et al. MYD88 L265P somatic mutation in Waldenstrom's macroglobulinemia. N Engl J Med. 2012 Aug 30;367(9):826–33. [Research Support, Non-U.S. Gov't] - PubMed
    1. Kyle RA, Rajkumar SV. Criteria for diagnosis, staging, risk stratification and response assessment of multiple myeloma. Leukemia. 2009 Jan;23(1):3–9. - PMC - PubMed
    1. Owen RG, Treon SP, Al-Katib A, Fonseca R, Greipp PR, McMaster ML, et al. Clinicopathological definition of Waldenstrom's macroglobulinemia: consensus panel recommendations from the Second International Workshop on Waldenstrom's Macroglobulinemia. Semin Oncol. 2003 Apr;30(2):110–5. - PubMed
    1. Kyle RA, Treon SP, Alexanian R, Barlogie B, Bjorkholm M, Dhodapkar M, et al. Prognostic markers and criteria to initiate therapy in Waldenstrom's macroglobulinemia: consensus panel recommendations from the Second International Workshop on Waldenstrom's Macroglobulinemia. Seminars in Oncology. 2003;30(2):116–20. [Review] [17 refs] - PubMed

Publication types

LinkOut - more resources