The epidemiology of Waldenström macroglobulinemia

McMaster, Mary L.

doi:10.1053/j.seminhematol.2023.03.008

Cited by 9 publications

(7 citation statements)

References 87 publications

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“…In comparison, Orphanet data indicate that WM has an annual incidence rate of one in 260,000 people in the USA [11]. Meanwhile, between 2000 and 2019, the age-standardized incidence rate of WM in the United States was found to be 0.36 per 100,000 people [13] and a prevalence of roughly one in 102,220 in Europe [11]. The prevalence in Albania might suggest an artefact due to the small sample size and demographic differences [14].…”

Section: Discussionmentioning

confidence: 99%

Uncovering the Challenges of Rare Diseases: Insights From a Retrospective Cross-Sectional Study in Albania (2005-2022)

Perolla,

Çalliku,

Cili

et al. 2024

Cureus

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Background: Diagnosing and treating rare diseases pose significant challenges within global healthcare systems due to their low prevalence and varying criteria for defining them. In Albania, the absence of a dedicated registry for rare diseases exacerbates these challenges. Recognising this gap, a retrospective cross-sectional study was conducted from January 2005 to December 2022 to analyse the incidence and prevalence of rare haematologic diseases in the country, diagnosed in the Hematology Service at the University Hospital Centre "Mother Teresa," which is the sole diagnostic center for blood diseases in Albania. This study aims to provide insights into the frequency of these diseases within the adult Albanian population and seeks to underscore the critical need for improved data collection and research in this field of healthcare.

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Section: Discussionmentioning

confidence: 99%

Uncovering the Challenges of Rare Diseases: Insights From a Retrospective Cross-Sectional Study in Albania (2005-2022)

Perolla,

Çalliku,

Cili

et al. 2024

Cureus

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“…Surveillance, Epidemiology, and End Results (SEER) data from 2000 to 2019 shows that the age-adjusted incidence of WM in the US population is 0.36 per 100,000 (or 0.63 per 100,000 for WM and LPL combined, shown as WM/LPL). 8 Individuals under the age of 30 are rarely diagnosed with WM. The occurrence of the condition starts increasing at the age of 40, and the rates continue to increase with each successive decade.…”

Section: Definitionmentioning

confidence: 99%

Waldenström Macroglobulinemia - A State-of-the-Art Review: Part 1: Epidemiology, pathogenesis, clinicopathologic characteristics, differential diagnosis, risk stratification, and clinical problems.

Bibas,

Castillo,

Sarosiek

2024

Mediterr J Hematol Infect Dis

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Waldenström macroglobulinemia (WM) is an infrequent variant of lymphoma, classified as a B-cell malignancy identified by the presence of IgM paraprotein, infiltration of clonal, small lymphoplasmacytic B cells in the bone marrow, and the MYD88 L265P mutation, which is observed in over 90% of cases. The direct invasion of the malignant cells into tissues like lymph nodes and spleen, along with the immune response related to IgM, can also lead to various health complications, such as cytopenias, hyperviscosity, peripheral neuropathy, amyloidosis, and Bing-Neel syndrome. Chemoimmunotherapy has historically been considered the preferred treatment for WM, wherein the combination of rituximab and nucleoside analogues, alkylating drugs, or proteasome inhibitors has exhibited notable efficacy in inhibiting tumour growth. Recent studies have provided evidence that Bruton Tyrosine Kinase inhibitors (BTKI), either used independently or in conjunction with other drugs, have been shown to be effective and safe in the treatment of WM. The disease is considered to be non-curable, with a median life expectancy of 10 to 12 years.

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“…Waldenström macroglobulinemia (WM) is a rare B cell lymphoma, which accounts for <2% of all non-Hodgkin lymphomas. Hyperglobulinemia of monoclonal immunoglobulin M (IgM) protein is the hallmark of the disease (1). Recently, somatic mutation of the MYD88 gene has been reported in more than 90% of patients with WM (2).…”

Section: Introductionmentioning

confidence: 99%

Case report: Waldenstrom macroglobulinemia with systemic amyloidosis as the main manifestation

Yin,

Zhou,

et al. 2024

Front. Med.

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Systemic amyloidosis is a rare protein misfolding and deposition disorder leading to progressive organ failure. Waldenström macroglobulinemia (WM) with systemic amyloidosis as the main manifestation is even rarer. The patient in this study presented with recurrent diarrhea and had not been diagnosed in other hospitals on multiple occasions. Later, his diarrhea worsened and was accompanied by sunken edema of both lower limbs and dizziness. Renal biopsy showed deposits of PAS light-staining material in the glomeruli, interstitium, and small arteries, which stained positively with Congo red. Cardiac ultrasound showed interventricular septum thickening of 17 mm, right ventricular wall myocardial thickening of approximately 0.6 cm, and septal thickening of approximately 0.5 cm, considering myocardial amyloidosis. Electromyography showed abnormal peripheral nerve conduction. Lymphoplasmacytic cells were found in the bone marrow. Taken together, he was diagnosed with WM. He was treated with a BR (Bendamustine + Rituximab) regimen. After 6 courses, the patient’s discomfort was relieved, his weight gained 5 kg, the level of serum IgM and dFLC decreased, and cardiac and renal assessments were more relieved. The patient has been followed up for more than 1 month.

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The epidemiology of Waldenström macroglobulinemia

Cited by 9 publications

References 87 publications

Uncovering the Challenges of Rare Diseases: Insights From a Retrospective Cross-Sectional Study in Albania (2005-2022)

Uncovering the Challenges of Rare Diseases: Insights From a Retrospective Cross-Sectional Study in Albania (2005-2022)

Waldenström Macroglobulinemia - A State-of-the-Art Review: Part 1: Epidemiology, pathogenesis, clinicopathologic characteristics, differential diagnosis, risk stratification, and clinical problems.

Case report: Waldenstrom macroglobulinemia with systemic amyloidosis as the main manifestation

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