A practical guide to laboratory investigations at diagnosis and follow up in Waldenström macroglobulinaemia: recommendations from the Medical and Scientific Advisory Group, Myeloma Australia, the Pathology Sub-committee of the Lymphoma and Related Diseases Registry and the Australasian Association of Clinical Biochemists Monoclonal Gammopathy Working Group

Maqbool, M. Gohar; Tam, Constantine S.; Morison, Ian M.; Simpson, David; Mollee, Peter; Schneider, Hans G.; Chan, Henry; Juneja, Surender; Harvey, Yasmin; Nath, Lakshmi; Hissaria, Pravin; Prince, H. Miles; Wordsworth, Helen; Opat, Stephen; Talaulikar, Dipti

doi:10.1016/j.pathol.2019.11.002

Cited by 19 publications

(28 citation statements)

References 60 publications

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“…Furthermore, the concentration of IgM varies widely in WM, which makes it impossible to distinguish WM from other lymphoproliferative disorders based on a certain concentration ( 15 ). However, with advancements in diagnostic techniques, including laboratory examination, bone marrow aspiration, biopsy evaluation, imaging, and real-time allele-specific polymerase chain reaction (AS-PCR) MYD88 L265P-mutated assay ( 16 ), more accurate diagnoses of patients with WM can be achieved ( 17 , 18 ). Specifically, flow immunophenotyping can better distinguish between lymphoid plasma cells and plasma cells ( 19 , 20 ).…”

Section: Discussionmentioning

confidence: 99%

Trends in Incidence and Mortality of Waldenström Macroglobulinemia: A Population-Based Study

et al. 2020

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Background: The incidence of Waldenström macroglobulinemia (WM) has increased in certain groups over several decades in the United States. It is unclear whether the increasing incidence is associated with mortality trends. Methods: The incidence and incidence-based mortality (IBM) rates were obtained from the Surveillance, Epidemiology, and End Results (SEER) database (1980-2016) with SEER * Stat software. The secular trends stratified by demographic characteristics were analyzed by joinpoint regression. Results: The incidence of WM showed an initial rapid increase from 1980 to 1993 {annual percentage change (APC), 14.1% [95% confidence interval (CI), 10 to 18.4%]}, whereas it began to stabilize from 1993 to 2016 [APC, 0.5% (95% CI, −0.3 to 1.3%)]. The WM IBM trend followed a similar pattern, with a decrease occurring around 1994. The trends in the incidence and mortality significantly differed according to geographic location, race, age, sex, primary site of involvement and subtype, which could help in further investigations into the specific etiology. Moreover, a dramatic increase in the 5-year survival rate from the 1980s to 2010s was observed (47.84 vs. 69.41%). Conclusions: Although both the incidence and IBM of WM continued to increase during the study period, a reduction in the rate of increase occurred around 1993. We believe that further advances in healthcare delivery and research can ensure a low mortality rate. Future studies can use the findings of this paper to monitor the results of WM therapy.

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Section: Discussionmentioning

confidence: 99%

Trends in Incidence and Mortality of Waldenström Macroglobulinemia: A Population-Based Study

et al. 2020

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“…Clinically, patients are in the seventh decade and often present with symptoms related to hyperviscosity and cytopenias due to bone marrow replacement [ 72 , 73 ].…”

Section: Lymphoplasmacytic Lymphomamentioning

confidence: 99%

“…LPL/WM shares immunophenotypic findings that overlap those of other CD5 negative, CD10 negative small B-cell lymphomas ( Figure 6 ). Characteristically, LPL/WM expresses CD20 and CD79a and is generally negative for CD5 and CD10 [ 72 , 73 ]. CD38 is typically strongly expressed in LPL/WM and the plasma cells in this disorder often co-express CD45 and CD19 [ 74 ].…”

Section: Lymphoplasmacytic Lymphomamentioning

confidence: 99%

“…SDRPL does not show the plasmacytic differentiation that is sometimes seen in other small B-cell neoplasms [ 72 ]. In the bone marrow, neoplastic B-cells typically involve marrow sinusoids, and in the spleen, diffuse involvement of the red pulp cord and sinus infiltration is typical, leading to massive splenomegaly [ 7 , 72 , 73 ]. Table 3 compares the morphologic features of SRDPL to the other CD5-CD10-LPDs.…”

Section: Splenic Diffuse Red Pulp Small B-cell Lymphomamentioning

confidence: 99%

“…SDRPL has a good long-term survival rate of greater than 90%, and more than 85% of patients in one series responded to splenectomy [ 72 , 82 ]. However, this entity does not respond to typical therapies for HCL, HCL-v, and SMZL [ 73 ]. Therefore, identification of this entity is required to appropriately direct therapy.…”

Section: Splenic Diffuse Red Pulp Small B-cell Lymphomamentioning

confidence: 99%

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CD5-Negative, CD10-Negative Low-Grade B-Cell Lymphoproliferative Disorders of the Spleen

Schmieg¹,

Muir²,

Aguilera

et al. 2021

Current Oncology

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CD5-negative, CD10-negative low-grade B-cell lymphoproliferative disorders (CD5-CD10-LPD) of the spleen comprise a fascinating group of indolent, neoplastic, mature B-cell proliferations that are essential to accurately identify but can be difficult to diagnose. They comprise the majority of B-cell LPDs primary to the spleen, commonly presenting with splenomegaly and co-involvement of peripheral blood and bone marrow, but with little to no involvement of lymph nodes. Splenic marginal zone lymphoma is one of the prototypical, best studied, and most frequently encountered CD5-CD10-LPD of the spleen and typically involves white pulp. In contrast, hairy cell leukemia, another well-studied CD5-CD10-LPD of the spleen, involves red pulp, as do the two less common entities comprising so-called splenic B-cell lymphoma/leukemia unclassifiable: splenic diffuse red pulp small B-cell lymphoma and hairy cell leukemia variant. Although not always encountered in the spleen, lymphoplasmacytic lymphoma, a B-cell lymphoproliferative disorder consisting of a dual population of both clonal B-cells and plasma cells and the frequent presence of the MYD88 L265P mutation, is another CD5-CD10-LPD that can be seen in the spleen. Distinction of these different entities is possible through careful evaluation of morphologic, immunophenotypic, cytogenetic, and molecular features, as well as peripheral blood and bone marrow specimens. A firm understanding of this group of low-grade B-cell lymphoproliferative disorders is necessary for accurate diagnosis leading to optimal patient management.

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Waldenström macroglobulinemia: 2023 update on diagnosis, risk stratification, and management

Gertz

2023

American J Hematol

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Disease Overview: Waldenström macroglobulinemia (WM) is a lymphoplasmacytic lymphoma with immunoglobulin M (IgM) monoclonal protein. Clinical features include anemia, thrombocytopenia, hepatosplenomegaly, lymphadenopathy, and rarely hyperviscosity.Diagnosis: Presence of IgM monoclonal protein associated with ≥10% clonal lymphoplasmacytic cells in bone marrow confirms the diagnosis. The L265P mutation in MYD88 is detectable in more than 90% of patients and is found in most IgM MGUS patients. MYD88 is not required for the diagnosis.Risk Stratification: Age, hemoglobin level, platelet count, β 2 microglobulin, LDH, and monoclonal IgM concentrations are characteristics that are predictive of outcomes.Risk-Adapted Therapy: Not all patients who fulfill WM criteria require therapy; these patients can be observed until symptoms develop. Rituximab-monotherapy is inferior to regimens that combine it with bendamustine, an alkylating agent, a proteosome inhibitor, or a BTK inhibitor. The preferred Mayo Clinic induction is either rituximab and bendamustine (without rituximab maintenance) or zanubrutinib. Management of RefractoryDisease: Bortezomib, cyclophosphamide, fludarabine, thalidomide, everolimus, Bruton Tyrosine Kinase inhibitors, carfilzomib, lenalidomide, bendamustine, and venetoclax have all been shown to have activity in relapsed WM. Given WM's natural history, the reduction of therapy toxicity is an important part of treatment selection. 1 | PATIENT A 55-year-old male was found to have an IgM monoclonal gammopathy in January 2005. He was observed until November 2010 when his IgM level climbed to 9135 mg/dl. He was treated with rituximab, bortezomib, and dexamethasone for 4 months. Treatment was interrupted due to neuropathy, but the IgM level fell to 1150. He was observed until April 2015. At the time of progression his IgM was 11 500 mg/dl and he was found to have acquired von Willebrand disease. 1 He was treated with single-agent rituximab which failed to produce a minor response. He received bendamustine, lenalidomide, and rituximab with a response of <1 year. In 2018 he was placed on an experimental trial of oprozomib with a response of 5 years duration. At relapse in August of 2022, he was placed on a trial of ixazomib and ibrutinib. Ixazomib was poorly tolerated due to diarrhea, and he continued ibrutinib alone and has achieved a very good partial response.

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Cited by 19 publications

References 60 publications

Trends in Incidence and Mortality of Waldenström Macroglobulinemia: A Population-Based Study

Trends in Incidence and Mortality of Waldenström Macroglobulinemia: A Population-Based Study

CD5-Negative, CD10-Negative Low-Grade B-Cell Lymphoproliferative Disorders of the Spleen

Waldenström macroglobulinemia: 2023 update on diagnosis, risk stratification, and management

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