360 results match your criteria: "Waldenstrom Hypergammaglobulinemia"

From MGUS to multiple myeloma: Unraveling the unknown of precursor states.

Blood Rev

November 2024

Myeloma Institute, Sylvester Comprehensive Cancer Center, Division of Myeloma, University of Miami, Miami, FL, United States of America. Electronic address:

Article Synopsis
  • In the 1960s, Dr. Waldenström identified "benign monoclonal gammopathy," a condition in asymptomatic patients with detectable monoclonal protein, which later led to the recognition of its potential progression to multiple myeloma by Dr. Kyle in 1978, coining the term MGUS.
  • Following this, Drs. Kyle and Greipp in 1980 proposed the term "smoldering multiple myeloma" (SMM) for asymptomatic cases that didn't fit existing definitions, establishing criteria based on plasma cell percentage and serum protein levels.
  • Current research focuses on advanced technologies (like whole genome sequencing) to enhance understanding of monoclonal gammopathy and improve individualized
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Currently, the role of DNA methylation in the immunoglobulin M (IgM) monoclonal gammopathy disease spectrum remains poorly understood. In the present study, a multiomics prospective analysis was conducted integrating DNA methylation, RNA sequencing (RNA-seq), and whole-exome sequencing data in 34 subjects (23 with Waldenström macroglobulinemia [WM], 6 with IgM monoclonal gammopathy of undetermined significance [MGUS], and 5 normal controls). Analysis was focused on defining differences between IgM gammopathies (WM/IgM-MGUS) compared with controls, and specifically between WM and IgM-MGUS.

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Monoclonal Gammopathy of Undetermined Significance (MGUS) is a clonal plasma cell disorder that is considered preneoplastic, asymptomatic, and only requiring observation. However, MGUS may result in cutaneous complications, which are poorly understood, causing treatment delays and patient suffering. We present 30 patients with cutaneous findings associated with MGUS, characterizing clinical presentations, isoforms, treatments, and outcomes.

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Cryoglobulins are immunoglobulins that precipitate at temperatures below 37 °C and dissolve upon reheating. They can induce small-vessel vasculitis with renal involvement. Cryoglobulinemic glomerulonephritis is a rare manifestation that occurs in patients with monoclonal gammopathy, specifically Waldenström's macroglobulinemia.

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Introduction: Anti-MAG neuropathies are associated with an IgM monoclonal gammopathy of undetermined significance (MGUS) or with a malignant haemopathy. Our objective was to determine whether the presence of a haemopathy or somatic mutations of MYD88 and CXCR4 genes influences disease presentation and response to rituximab (RTX).

Methods: We included 79 patients (mean age 74 years, disease duration 9.

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Article Synopsis
  • A study screened 534 patients with monoclonal IgM disorders over nine years, revealing that 134 had type I cryoglobulinaemia, predominantly associated with Waldenström macroglobulinaemia (76%).
  • Coexisting clinically relevant disorders were found in 31% of patients, with a significant prevalence in those with IgM monoclonal gammopathy of undetermined significance (MGUS) compared to Waldenström macroglobulinaemia or non-Hodgkin lymphoma.
  • Half of the patients experienced active symptoms related to cryoglobulins, and after a median follow-up of three years, 77% had not required treatment for cryoglobulinaemia, with age being the main factor influencing overall survival.
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Screening for and diagnosis of monoclonal gammopathy.

J Clin Pathol

November 2023

Department of Pathology, Northern Health, Epping, Victoria, Australia

Monoclonal gammopathy is a spectrum of disorders characterised by clonal proliferation of plasma cells or lymphocytes, which produce abnormal immunoglobulin or its components (monoclonal proteins). Monoclonal gammopathies are often categorised as low-tumour-burden diseases (eg, amyloid light chain (AL) amyloidosis), premalignant disorders (such as monoclonal gammopathy of undetermined significance and smouldering multiple myeloma), and malignancies (eg, multiple myeloma and Waldenström's macroglobulinaemia). Such diversity of concentration and structure makes monoclonal protein a challenging clonal marker.

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Investigation and Management of Immunoglobulin M- and Waldenström-Associated Peripheral Neuropathies.

Hematol Oncol Clin North Am

August 2023

Department of Haematology, Centre for Waldenströms Macroglobulinaemia and Related Conditions, University College London Hospitals NHS Foundation Trust, 250 Euston Road, London NW1 2PG, UK. Electronic address:

Article Synopsis
  • IgM-associated peripheral neuropathies (PN) are a diverse group of disorders linked to conditions like IgM monoclonal gammopathy of undetermined significance (MGUS) or Waldenström macroglobulinemia.
  • Identifying the relationship between paraproteins and neuropathies is crucial for determining effective treatment strategies.
  • The most common IgM-PN is Antimyelin-Associated-Glycoprotein neuropathy, but many cases have different underlying causes; treatment options include rituximab or chemotherapy, especially when functional impairment worsens.
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Clinical Implications of Genomic Profile in Waldenström Macroglobulinemia.

Hematol Oncol Clin North Am

August 2023

Department of Hematology, Amyloidosis and Myeloma Unit, Hospital Clínic de Barcelona, Villarroel 170, 08036, Barcelona, Spain; Institut D'Investigacions Biomèdiques August Pi I Sunyer, University of Barcelona, Spain. Electronic address:

Article Synopsis
  • Advances in sequencing and PCR techniques are enhancing our understanding of the genomic profile of Waldenström macroglobulinemia (WM).
  • MYD88 and CXCR4 mutations are commonly found in all WM stages, from early stages to more advanced forms.
  • Understanding genotypes is crucial for determining appropriate treatment approaches and for research in clinical trials.
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Article Synopsis
  • A study of 191 patients with IgM monoclonal gammopathy of undetermined significance (MGUS) found that 43% developed IgM-related disorders (IgM-RD), such as peripheral neuropathy, cryoglobulinemia, and cold agglutinin disease (CAD).
  • Clonal plasma and B cells were identified in a notable proportion of patients, with distinctive characteristics observed in CAD cases, including a lower rate of MYD88 mutations.
  • Overall, while IgM-RD is prevalent among IgM MGUS patients, those with or without these disorders exhibited similar clinical and pathologic features, with no significant difference in survival rates.
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Epidemiology of monoclonal gammopathy in Morocco - A hospital-based study.

Cancer Rep (Hoboken)

May 2023

Clinical Chemistry Department, Faculty of Medicine and Pharmacy, Ibn Zohr University, Agadir Teaching Hospital, Agadir, Morocco.

Article Synopsis
  • This study focused on monoclonal gammopathies, a group of disorders involving abnormal growth of plasma cells that produce a specific type of protein, aiming to analyze their characteristics in a Moroccan hospital over 19 years.! -
  • A total of 443 patients participated in the research, predominantly men (72.23%), with an average age of about 62 years. Common reasons for hospital admission included bone pain and renal failure.! -
  • The findings revealed that conditions like multiple myeloma and monoclonal gammopathy of undetermined significance were the most common types, with the study highlighting a tendency for male patients and suggesting that diagnoses are often delayed.!
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Monoclonal gammopathy of undetermined significance (MGUS) is a benign hematological condition with the potential to progress to malignant conditions including multiple myeloma and Waldenstrom macroglobulinemia. Medications that modify progression risk have yet to be identified. To investigate, we leveraged machine-learning and electronic health record (EHR) data to screen for drug repurposing candidates.

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Purpose: IgM monoclonal gammopathy of undetermined significance (MGUS) and Waldenström macroglobulinemia (WM) represent a disease spectrum with highly varied therapeutic management, ranging from observation to chemoimmunotherapy. The current classification relies solely on clinical features and does not explain the heterogeneity that exists within each of these conditions. Further investigation is warranted to shed light on the biology that may account for the clinical differences.

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Monoclonal Gammopathy of Undetermined Significance.

Ann Intern Med

December 2022

Division of Hematology, Mayo Clinic, Rochester, Minnesota (W.I.G., S.V.R.).

Monoclonal gammopathy of undetermined significance (MGUS) is of considerable clinical importance to primary care physicians given its high prevalence in the general population. MGUS has a variable but lifelong risk for progression to hematologic cancer, such as multiple myeloma, Waldenström macroglobulinemia, or light-chain amyloidosis. In addition, MGUS has been associated with several nonmalignant yet symptomatic disorders that require therapy directed toward eliminating the monoclonal gammopathy.

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[Waldenstrom’s macroglobulinemia highlighted by turbidity interference: a case report].

Ann Biol Clin (Paris)

September 2022

Service de biochimie et génétique moléculaire, CHU Clermont-Ferrand, France

A 65-year-old female was admitted to the Emergency Department for a fall. Upon admission, a blood sample was drawn for routine laboratory tests. Blood glucose measurement using the hexokinase method was made impossible due to a high lipemia index measured by the instrument despite a clear plasma specimen.

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Waldenström macroglobulinaemia (WM) is characterized by recurrent somatic mutations in MYD88 and CXCR4 genes. However, limitations arise when analysing these mutations in IgM monoclonal gammopathy of undetermined significance (MGUS) or smouldering WM (SWM) given the lower tumour load. Here, we used droplet digital polymerase chain reaction (ddPCR) to analyse MYD88 L265P and CXCR4 S338* mutations (C1013G and C1013A) in unsorted bone marrow (BM) or cell-free DNA (cfDNA) samples from 101 IgM MGUS and 69 SWM patients.

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Background: Due to the various clinical and pathological manifestations of kidney involvement in lymphoproliferative disorder (LPD), the whole spectrum of kidney disease in LPD is still unclear, and data on kidney prognosis is scarce.

Methods: We retrospectively reviewed the renal pathology profiles from January 2010 to December 2021, and 28 patients with B-cell LPD combined with intact renal biopsy data were included.

Results: There were 20 men and eight women aging 41 to 79 years at the time of renal biopsy (median age 62 years).

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Monoclonal gammopathy of undetermined significance (MGUS) is a premalignant clonal disorder that progresses to multiple myeloma (MM), or other plasma-cell or lymphoid disorders at a rate of 1%/year. We evaluate the contribution of body mass index (BMI) to MGUS progression beyond established clinical factors in a population-based study. We identified 594 MGUS through a population-based screening study in Olmsted County, Minnesota, between 1995 and 2003.

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When a Monoclonal Gammopathy Is Not Multiple Myeloma.

Am Soc Clin Oncol Educ Book

April 2022

Department of Hematology, Cebeci Hospital, Ankara University Dikimevi, Ankara, Turkey.

Our knowledge of monoclonal gammopathies is continuously evolving. Once accepted as a possible precursor condition to multiple myeloma, monoclonal gammopathies as an entity are now associated with many renal, neurologic, and dermatologic disorders of clinical significance. This change has created a challenge for patients and clinicians, as a monoclonal gammopathy may be a harbinger not of multiple myeloma but of other lymphoproliferative disorders such as light-chain amyloidosis and Waldenström macroglobulinemia.

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Background: Hypergammaglobulinemic purpura of Waldenström is an uncommon disease, which presents mostly in women on the lower extremities. It is sometimes associated with underlying immune dysregulation. Sjögren syndrome is the most common association; however, rare occurrences of the self-resolving syndrome with lymphoma or myeloma have been reported.

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A patient was diagnosed with Waldenström's macroglobulinemia (WM) after the initial findings of anemia and ophthalmological findings of retinal hemorrhage. Upon further workup, the patient was found to have an IgM predominant monoclonal gammopathy on serum protein electrophoresis (SPEP) and urine protein electrophoresis (UPEP). This highlights the need for open communication between different specialties, streamlining rapid and accurate diagnosis.

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