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Neurology 2021 02 4;96(5):214-225. Epub 2020 Dec 4.

From the Department of Neurology (J.-M.V., M.D., L.R., K.G., L.M.), National Reference Center for "Rare Peripheral Neuropathies," Dupuytren University Hospital (CHU Limoges), University of Limoges; Department of Pathology (M.D.), Limoges University Hospital (CHU Limoges), University of Limoges; Department of Neurology and ALS Reference Center (P.C.), Bretonneau University Hospital (CHU Tours), University of Tours; and Department of Neurology (S.M.), Nerve-Muscle Unit, 4 Pellegrin University Hospital (CHU Bordeaux), University of Bordeaux, France.

Monoclonal gammopathy is encountered quite frequently in the general population. This type of hematologic abnormality may be mild, referred to as monoclonal gammopathy of undetermined significance or related to different types of hematologic malignancies. The association of a peripheral neuropathy with monoclonal gammopathy is also fairly common, and hemopathy may be discovered in an investigation of peripheral neuropathy. Read More

Ann Biol Clin (Paris) 2020 10;78(5):527-536

Service d'hématologie biologique, Hôpitaux universitaires Pitié-Salpêtrière-Charles-Foix, Paris, France.

We report the case of a man with a primary diagnosis of Waldenström macroglobulinemia. He secondarily presented a diffuse large B cell lymphoma (DLBCL) located in the nasal fossae, which relapsed later in the eye. The diagnosis of these two malignancies is based on a multidisciplinary biological approach using new sensitive and specific techniques. Read More

Neoplasma 2020 Jul 23;67(4):939-945. Epub 2020 Jun 23.

Department of Internal Medicine, Hematology and Oncology, University Hospital, Masaryk University, Brno, Czech Republic.

Monoclonal gammopathy of undetermined significance (MGUS) is a known precursor of more serious cancers, such as multiple myeloma (MM), Waldenström macroglobulinemia (MW) and other lymphoproliferative disorders. Using 18F-FDG PET/CT, we aimed to evaluate its benefit in early detection of various accompanying disorders and illnesses in MGUS patients. We prospectively analyzed the diagnostic relevance of 18F-FDG PET/CT in 390 newly diagnosed MGUS patients. Read More

J Clin Pathol 2020 Sep 24;73(9):571-577. Epub 2020 Jan 24.

Anatomic Pathology Service, Hematology Service and Translational Hematopathology Lab, Hospital Universitario Marques de Valdecilla/IDIVAL. Centro de Investigación Biomédica en Red de Cáncer (CIBERONC), Santander, Cantabria, Spain

Aims: The aim of this study was to describe the characteristics of the bone marrow infiltration found in a series of clinically defined lymphoplasmacytic lymphoma (LPL)/Waldenström macroglobulinaemia (WM) and IgM-monoclonal gammopathy of undetermined significance (MGUS) and to perform a targeted next-generation sequencing (NGS) for the identification of additional somatic mutations to p.L265P in LPL/WM.

Methods: We have reviewed a series of 35 bone marrow biopsies from 28 patients with a clinical diagnosis of LPL/WM (24 cases) or MGUS (4 cases). Read More

Hematol Oncol 2020 Feb 18;38(1):111-113. Epub 2019 Dec 18.

ASST Grande Ospedale Metropolitano Niguarda, Department of Hematology, Niguarda Cancer Center, Milan, Italy.

PLoS One 2019 4;14(9):e0221941. Epub 2019 Sep 4.

Department of Hematology, Chiba University Hospital, Chiba, Japan.

We investigated the feasibility of using next-generation sequencing (NGS) technique using molecular barcoding technology to detect MYD88 L265P mutation in unselected peripheral blood mononuclear cells (PBMCs) in 52 patients with Waldenström's macroglobulinemia [1] and 21 patients with IgM-monoclonal gammopathy of undetermined significance (MGUS). The NGS technique successfully detected the MYD88 L265P in unselected PBMCs at a sensitivity of 0.02%, which was ×5 higher than that of AS-PCR. Read More

Curr Opin Neurol 2019 10;32(5):658-665

National Hospital for Neurology and Neurosurgery, Centre for Neuromuscular Disease, Queen Square, London, England.

Purpose Of Review: There is an increasingly recognized association between haematological and neurological disease. This is especially true in the peripheral nervous system in which, to an extent, proof of a link is easier to achieve. The most sensitive low level paraprotein detection methods should always be employed in which a paraprotein is suspected. Read More

Br J Haematol 2019 11 12;187(4):413-415. Epub 2019 Jul 12.

Division of Haematology, Mayo Clinic, Rochester, MN, USA.

Br J Haematol 2019 11 5;187(4):441-446. Epub 2019 Jul 5.

Division of Haematology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.

IgM monoclonal gammopathies of undetermined significance (IgM MGUS) are associated with a risk of progression to Waldenström macroglobulinaemia (WM) or other lymphoproliferative disorders (LPD) of 1-2% per year. We analysed 176 consecutive patients with IgM MGUS to evaluate risk factors for progression. With a median follow-up of 83 months (1214 person-years), 15 patients (8·5%) progressed to WM (n = 14) or marginal zone lymphoma (n = 1). Read More

Rev Prat 2018 Sep;68(7):777-784

CHU de Rennes, service de médecine interne, hôpital Sud, Rennes, France. Faculté de médecine de Rennes, université Rennes-1, Rennes, France.

Conduct in case of monoclonal gammapathy ? Monoclonal gammopathies are frequent in general population (about 3 % beyond 50 years) and their prevalence increases with age. They may be associated with malignant haemopathy (multiple myeloma for IgG and IgA, Waldenström disease for IgM). In absence of malignant haemopathy, the diagnosis is that of monoclonal gammopathy of indeterminate significance (MGUS). Read More

Ann Rheum Dis 2019 05 12;78(5):583-585. Epub 2019 Jan 12.

Department of Clinical Sciences Lund, Rheumatology, Medical Faculty, Lund University, Lund 22185, Sweden

Klin Onkol Summer 2018;31(4):270-276

Background: Monoclonal gammopathy of undetermined significance (MGUS) is one of the most prevalent premalignant conditions associated with a risk of malignant transformation to multiple myeloma (MM) or other forms of lymphoproliferative disorders with risk of progression of approximately 1% per year. IgG and IgA MGUS are precursor conditions of multiple myeloma (MM), whereas light-chain MGUS is a precursor condition of light chain MM. IgM MGUS is a precursor condition of Waldenström macroglobulinemia (MW) or other lymphoproliferative diseases. Read More

J Glob Oncol 2018 09 9;4:1-8. Epub 2017 May 9.

Shebli Atrash, Qing Zhang, Xenofon Papanikolaou, Caleb Stein, Al-Ola Abdallah, and Bart Barlogie, University of Arkansas for Medical Sciences, Little Rock, Arkansas; and Qing Zhang, Levine Cancer Institute, Charlotte, NC.

Purpose: Multiple myeloma (MM) is a clonal bone marrow disease characterized by the neoplastic transformation of differentiated postgerminal B cells. It is a heterogeneous disease both at the genetic level and in terms of clinical outcome. Immunoglobulin M (IgM) MM is a rare subtype of myeloma. Read More

Hematol Oncol 2019 Apr 7;37(2):117-128. Epub 2018 Sep 7.

Division of Hematology, Fondazione IRCCS Policlinico S. Matteo, Pavia, Italy.

The finding of an IgM monoclonal gammopathy often represents a diagnostic challenge. In fact, there are many pathological disorders associated with this condition, each of which has distinctive characteristics and requires specific clinical, instrumental, and laboratory assessments to set the appropriate treatment. This review has two aims. Read More

Hematol Oncol Clin North Am 2018 10 26;32(5):787-809. Epub 2018 Jul 26.

Clinical Genetics Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, US Department of Health and Human Services, 9609 Medical Center Drive, Room 6E516, MSC 9772, Bethesda, MD 20892-9772, USA; Commissioned Corps of the US Public Health Service, US Department of Health and Human Services, 330 C Street SW, Washington, DC 20416, USA. Electronic address:

Familial clustering of Waldenström macroglobulinemia (WM) has been observed for nearly 6 decades. Family studies have provided seminal observations in delineating the phenotypic spectrum of WM susceptibility and confirming the importance of immunoglobulin M (IgM) monoclonal gammopathy of undetermined significance (IgM MGUS) as a precursor condition for WM, providing the rationale for large population-based epidemiologic studies of IgM MGUS and WM, and providing both the basis and the material for ongoing genetic studies aimed at identifying WM predisposition genes. Together, these investigations may help elucidate the host factors underlying WM development. Read More

Blood Cancer J 2018 05 1;8(4):40. Epub 2018 May 1.

Division of Hematology, Mayo Clinic, 200 First Street, SW, Rochester, MN, 55905, USA.

Waldenström macroglobulinemia is often an indolent disorder, and many patients are candidates for observation with careful monitoring. For symptomatic patients, one must distinguish between those patients whose symptoms are related to immunologic manifestations associated with the IgM monoclonal protein and those that have symptoms related to progressive marrow and nodal infiltration with lymphoplasmacytic lymphoma. In Waldenström macroglobulinemia, the driver for therapy in the majority of patients is progressive anemia, secondary to bone marrow replacement by lymphoplasmacytic lymphoma. Read More

Cancer Res 2018 05 27;78(10):2457-2462. Epub 2018 Apr 27.

Department of Cancer Biology, Lerner Research Institute, Cleveland Clinic, Cleveland, Ohio.

Next-generation sequencing has revealed cancer genomic landscapes, in which over 100 driver genes that, when altered by intragenic mutations, can promote oncogenesis. is a driver gene found in hematologic B-cell malignancies. A missense mutation (L265P) changing leucine at position 265 to proline in MYD88 is found in ∼90% of Waldenström macroglobulinemia (WM) cases and in significant portions of activated B-cell diffuse large B-cell lymphomas and IgM monoclonal gammopathy of undetermined significance. Read More

Clin Lymphoma Myeloma Leuk 2018 06 31;18(6):431-437. Epub 2018 Mar 31.

Department of Clinical Therapeutics, University of Athens School of Medicine, Athens, Greece. Electronic address:

Background: Serum receptor activator of nuclear factor κB ligand (sRANKL) and chemokine (C-C) motif ligand 3 (CCL-3) have been reported to be elevated in Waldenström macroglobulinemia (WM) patients. However, there are no published data regarding the prognostic value of these molecules in WM regarding progression-free and overall survival.

Methods: To evaluate the effect of these markers of bone remodeling on survival parameters, we prospectively evaluated serum cytokines and biological markers in 55 patients with symptomatic WM before they received any kind of treatment. Read More

Dtsch Arztebl Int 2018 03;115(11):190

Ann Hematol 2018 Mar 20;97(3):475-484. Epub 2018 Jan 20.

Hematology Department, University Hospital of Salamanca and Research Biomedical Institute of Salamanca (IBSAL), Paseo de San Vicente, 58-182, 37007, Salamanca, Spain.

Immunoglobulin M (IgM) monoclonal gammopathies show considerable variability, involving three different stages of presentation: IgM monoclonal gammopathy of undetermined significance (IgM-MGUS), asymptomatic Waldenström's macroglobulinemia (AWM), and symptomatic WM (SWM). Despite recent findings about the genomic and transcriptomic characteristics of such disorders, we know little about the causes of this clinical heterogeneity or the mechanisms involved in the progression from indolent to symptomatic forms. To clarify these matters, we have performed a gene expression and mutational study in a well-characterized cohort of 69 patients, distinguishing between the three disease presentations in an attempt to establish the relationship with the clinical and biological features of the patients. Read More

J Clin Exp Hematop 2017 ;57(2):47-53

Department of Transfusion Medicine, Asahi General Hospital.

Many patients with immunoglobulin M (IgM) monoclonal gammopathy remain asymptomatic and, consequently, untreated; however, few studies have evaluated the clinical course and prognosis of these patients. Using the screening procedures at our hospital, 74 patients with IgM monoclonal gammopathy were selected. We excluded 11 patients in whom the treatment for lymphoid neoplasms had been initiated at the time of IgM monoclonal protein detection. Read More

Haematologica 2017 12 5;102(12):2077-2085. Epub 2017 Oct 5.

Department of Hematology Oncology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.

We analyzed and CXCR4 mutation status of 260 patients with Waldenström macroglobulinemia or IgM monoclonal gammopathy of undetermined significance using allele-specific real time quantitative polymerase chain reaction and Sanger sequencing, respectively. A subgroup of 119 patients was further studied with next-generation sequencing of 11 target genes (, , , , , , , , , , and ). (L265P) was found at diagnosis in 91% of patients with Waldenström macroglobulinemia and in 60% of patients with IgM monoclonal gammopathy of undetermined significance using allele-specific polymerase chain reaction analysis. Read More

Klin Onkol Summer 2017;30(Supplementum2):43-50

Background: The Registry of Monoclonal Gammopathies (RMG) was established by the Czech Myeloma Group in 2007. RMG is a registry designed for the collection of clinical data concerning diagnosis, treatment, treatment results and survival of patients with monoclonal gammopathies. Data on patients with monoclonal gammopathy of undetermined significance (MGUS), Waldenström macroglobulinaemia (WM), multiple myeloma (MM) or primary AL ("amyloid light-chain") amyloidosis are collected in the registry. Read More

Mayo Clin Proc 2017 05;92(5):838-850

Division of Hematology, Mayo Clinic, Rochester, MN. Electronic address:

Monoclonal gammopathies comprise a spectrum of clonal plasma cell disorders that include monoclonal gammopathy of undetermined significance, multiple myeloma, and Waldenström macroglobulinemia. In this review, we outline the epidemiology, etiology, classification, diagnosis, and treatment of monoclonal gammopathy-associated peripheral neuropathy. Monoclonal gammopathy of undetermined significance is relatively common in the general population, with a prevalence of 3% to 4% among individuals older than age 50 years. Read More

Ann Hematol 2017 Jun 9;96(6):971-976. Epub 2017 Mar 9.

Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, 100730, China.

A broad spectrum of diseases are associated with IgM monoclonal gammopathy, including Waldenstrom macroglobulinemia (WM), various types of B cell non-Hodgkin's lymphoma (NHL), multiple myeloma (MM), primary amyloidosis (AL), and monoclonal gammopathy of undetermined significance (MGUS); these are called IgM monoclonal gammopathy related diseases (IgM-RD). We investigated MYD88 L265P and WHIM-like CXCR4 mutations in various IgM-RD. Patients with serum immunofixation electrophoresis confirmed IgM monoclonal gammopathy who had enough material for DNA extraction and presented between January 2008 and October 2016 at Peking Union Medical College Hospital were enrolled in this cohort. Read More

Best Pract Res Clin Haematol 2016 06 4;29(2):187-193. Epub 2016 Sep 4.

Myeloma Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, NY, United States.

Since the initial identification of patients with monoclonal elevation of gamma globulin and associated clinical symptoms in 1944 by Jan Waldenström, several new insights have been gained using a range of approaches. For example, IgM monoclonal gammopathy of undetermined significance and smoldering Waldenström's macroglobulinemia are defined clinical precursor states to symptomatic Waldenström's macroglobulinemia. Epidemiologic studies have established the prevalence of these conditions and the estimated risk of progression to symptomatic disease. Read More

Br J Haematol 2017 12 8;179(5):849-851. Epub 2016 Sep 8.

Amyloidosis and Myeloma Unit, Department of Haematology, Hospital Clínic de Barcelona, Institut d'Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain.

Zhonghua Xue Ye Xue Za Zhi 2016 Aug;37(8):692-5

Key Laboratory of Thrombosis and Hemostasis of Ministry of Health, Jiangsu Institute of Hematology, The First Affiliated Hospital of Soochow University, Collaborative Innovation Center of Hematology, Soochow University, Suzhou 215006, China.

Objective: To deepen the understanding of acquired von Willebrand syndrome (AVWS).

Methods: The clinical data of 3 patients were analyzed and related literature were reviewed.

Results: ① Case 1, a 70- year- old male, diagnosed as Waldenstrom macroglobulinemia and AVWS, was presented with spontaneous epitaxis and bruising. Read More