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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

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

Leuk Lymphoma 2017 04 22;58(4):773-780. Epub 2016 Aug 22.

b Division of Hematology , Mayo Clinic , Rochester , MN , USA.

In recent years, the survival of patients with plasma cell dyscrasias has improved due to improvements in anticancer and supportive therapy. However, the risk of secondary malignancies has increased, thought to be due to a combination of environmental and disease-related factors, as well as treatment. In the present review, we evaluate the risk of secondary malignancies in patients with monoclonal gammopathy of undetermined significance (MGUS), multiple myeloma (MM) and Waldenström macroglobulinemia (WM). Read More

Leuk Res 2016 07 4;46:85-8. Epub 2016 May 4.

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

Objectives: We retrospectively evaluated the clinical features, serum levels of IgM, and prevalence of IgM related diseases in patients with serum immunofixation electrophoresis (sIFE) confirmed IgM monoclonal gammopathy at our center.

Methods: We included patients with sIFE confirmed IgM monoclonal gammopathy between January 2008 and December 2014 in this retrospective study. We evaluated clinical data, sIFE, serum IgM levels, and diagnosis. Read More

Pan Afr Med J 2015 1;22:92. Epub 2015 Oct 1.

Service de Médecine Interne, Hôpital Razi, La Manouba 2010, Faculté de médecine de Tunis, Tunisie.

Int J Lab Hematol 2016 Apr 21;38(2):133-40. Epub 2016 Jan 21.

NeoGenomics Laboratories, Irvine, CA, USA.

Introduction: Detection of mutations in the myeloid differentiation primary response gene 88 (MYD88) has clinical implications on diagnosis and therapy, especially in patients with Waldenström's macroglobulinemia (WM) and IgM monoclonal gammopathy of unknown significance (IgM-MGUS). We describe a method that provides greatly increased sensitivity for detecting minority mutations in MYD88.

Methods: We used a locked nucleic acid oligonucleotide to block amplification of wild-type DNA during polymerase chain reaction (PCR). Read More

Br J Haematol 2016 Mar 13;172(5):735-44. Epub 2015 Dec 13.

Bing Center for Waldenström's Macroglobulinemia, Dana Farber Cancer Institute, Boston, MA, USA.

CXCR4(WHIM) somatic mutations are distinctive to Waldenström Macroglobulinaemia (WM), and impact disease presentation and treatment outcome. The clonal architecture of CXCR4(WHIM) mutations remains to be delineated. We developed highly sensitive allele-specific polymerase chain reaction (AS-PCR) assays for detecting the most common CXCR4(WHIM) mutations (CXCR4(S338X C>A and C>G) ) in WM. Read More

PLoS One 2015 9;10(9):e0136505. Epub 2015 Sep 9.

Hospices Civils de Lyon, Pierre Bénite, France; INSERM 1052, CNRS 5286, CRCL, Lyon, France; University of Lyon, Lyon, France; ProfilExpert, Lyon, France.

The MYD88 L265P is a recurrent somatic mutation in neoplastic cells from patients with Waldenström Macroglobulinemia (WM). We identified the MYD88 L265P mutation in three individuals from unrelated families, but its presence did not explain the disease segregation within these WM pedigrees. We observed the mutation in these three individuals at high allele fractions in DNA extracted from EBV-immortalized Lymphoblastoid cell lines established from peripheral blood (LCL), but at much lower allele fractions in DNA extracted directly from peripheral blood, suggesting that this mutation is present in a clonal cell subpopulation rather than of germ-line origin. Read More

Blood 2015 Apr;125(15):2318-9

MAYO CLINIC.

In this issue of Blood, Paiva et al provide important information on the cell of origin in Waldenström macroglobulinemia (WM), a longstanding puzzle due to conflicting and incomplete data. Read More

Ophthalmic Surg Lasers Imaging Retina 2015 Feb;46(2):262-5

Waldenström's macroglobulinemia (WM) is associated with retinal findings of hyperviscosity, such as venous dilation, and findings of immunogammopathy maculopathy, such as serous macular detachment. This report describes a case of bilateral serous macular detachment with intraretinal schisis-like fluid in a patient with WM. Enhanced depth imaging optical coherence tomography revealed a thickened choroid with hyperreflective accumulations in the retinal pigment epithelium layer. Read More

Int J Cancer 2015 Sep 26;137(5):1076-84. Epub 2015 Feb 26.

José Carreras Center for Immuno and Gene Therapy, Department of Internal Medicine I, Saarland University Medical School, Homburg/Saar, Germany.

Hyperphosphorylated paratarg-7 (pP-7) carrier state is the strongest and most frequent molecular risk factor for MGUS, multiple myeloma (MM) and Waldenström's macroglobulinemia (WM), inherited autosomal-dominantly and, depending on the ethnic background, found in up to one third of patients with MGUS/MM. Since P-7 is the antigenic target of paraproteins that do not distinguish between wtP-7 and pP-7, we investigated CD4(+) T-cell responses in pP-7(+) patients and controls. Peptides spanning amino acids 1-35 or 4-31 containing phosphorylated or nonphosphorylated serine17 were used for stimulation. Read More

Blood 2015 Apr 5;125(15):2370-80. Epub 2015 Feb 5.

Clinica Universidad de Navarra, Centro de Investigacion Medica Aplicada, Instituto de Investigación Sanitaria de Navarra, Pamplona, Spain;

Although information about the molecular pathogenesis of Waldenström macroglobulinemia (WM) has significantly advanced, the precise cell of origin and the mechanisms behind WM transformation from immunoglobulin-M (IgM) monoclonal gammopathy of undetermined significance (MGUS) remain undetermined. Here, we undertook an integrative phenotypic, molecular, and genomic approach to study clonal B cells from newly diagnosed patients with IgM MGUS (n = 22), smoldering (n = 16), and symptomatic WM (n = 11). Through principal component analysis of multidimensional flow cytometry data, we demonstrated highly overlapping phenotypic profiles for clonal B cells from IgM MGUS, smoldering, and symptomatic WM patients. Read More

Rev Med Interne 2015 Jul 5;36(7):444-9. Epub 2014 Nov 5.

Service des maladies du sang, hôpital Huriez, CHRU de Lille, rue M.-Polonovski, 59037 Lille, France. Electronic address:

Introduction: Monoclonal gammopathy of undetermined significance (MGUS) is a frequent entity in the general population. The incidence rate of fortuitous discovery of a monoclonal component in asymptomatic patients is increasing nowadays. The majority of MGUS is being addressed to a hematologist for diagnosis or follow-up by their generalist practitioners. Read More

J Neurol Sci 2015 Feb 26;349(1-2):60-4. Epub 2014 Dec 26.

Department of Neurology, Odense University Hospital, Odense, Denmark.

Background And Purpose: For several decades an association between MGUS, IgM-MGUS in particular, and peripheral neuropathy has been suspected. Several histopathology studies have shown binding of IgM to myelin and a secondary widening of myelin lamellae in cutaneous nerves and in the sural nerve of patients with IgM-MGUS, or Waldenström's Macroglobulinaemia (WM), and peripheral neuropathy. In this retrospective study we investigated the value of skin biopsy examination in the diagnosis of MGUS- and WM-associated peripheral neuropathy. Read More

Clin Lymphoma Myeloma Leuk 2015 Mar 28;15(3):177-186.e4. Epub 2014 Sep 28.

Department of Medicine, Medical College of Wisconsin, Milwaukee, WI.

Background: Clinical guidelines have recommended annual follow-up examinations of most patients with monoclonal gammopathy of undetermined significance (MGUS); however, evidence supporting this practice is lacking. We performed a population-based study to examine the patterns of disease presentation and outcomes of patients with multiple myeloma, Waldenström macroglobulinemia, and lymphoplasmacytic lymphoma (monoclonal gammopathy-associated malignancies) comparing those with or without a previous MGUS follow-up examination.

Materials And Methods: Patients with monoclonal gammopathy-associated malignancy from 1994 through 2007 were identified using the Surveillance, Epidemiology, and End Results-Medicare linked database and divided into 2 cohorts: those with follow-up (MGUS follow-up examination preceding the diagnosis) and those with no follow-up (no such follow-up examination). Read More

Int J Hematol 2015 Feb 28;101(2):133-9. Epub 2014 Nov 28.

Department of Hematology, Rheumatology & Infectious Diseases, Kumamoto University of Medicine, 1-1-1 Honjo, Kumamoto, 860-8556, Japan.

Amyloid light-chain amyloidosis (ALA) is a rare disease with poor prognosis and is often associated with monoclonal gammopathy of undetermined significance, multiple myeloma, or Waldenström macroglobulinemia. Only high-dose melphalan with auto-peripheral blood stem cell transplantation (PBSCT) has shown high long-term hematological response rates, but combinations with novel agents, including bortezomib or lenalidomide, have recently shown high hematological response rates for AL amyloidosis patients. In the present study, we treated eight Japanese patients with AL amyloidosis using bortezomib, cyclophosphamide, and dexamethasone (CyBorD). Read More

Am J Hematol 2015 Mar 16;90(3):E51-2. Epub 2015 Jan 16.

Babak Myeloma Group, Department of Pathological Physiology, Faculty of Medicine, Masaryk University, Brno, Czech Republic; Department of Clinical Hematology, University Hospital Brno, Brno, Czech Republic.

Vnitr Lek 2014 Oct;60(10):861-79

Presence of monoclonal immunoglobulin in serum or urine is a relatively common event affecting about 3.2 % of people over 50. Isolated increase of only one type of free light chain, either κ or λ, is detected in 0. Read More

Appl Immunohistochem Mol Morphol 2014 Nov-Dec;22(10):768-73

*Department of Hematology, University Hospital of Salamanca †Center of Investigation in Cancer (CIC), Instituto Biosanitario de Salamanca (IBSAL), Salamanca, Spain.

MYD88 L265P mutation has been reported in ∼90% of Waldenström's Macroglobulinemia (WM) patients and immunoglobulin M (IgM) monoclonal gammopathies of uncertain significance (MGUS), as well as in some cases of lymphoma and chronic lymphocytic leukemia. The present study aimed to develop a real-time allele-specific oligonucleotide PCR (ASO-RQ-PCR) to detect the MYD88 L265P mutation. We first evaluated the reproducibility and sensitivity of the technique with a diluting experiment of a previously known positive sample. Read More

Ned Tijdschr Geneeskd 2014 ;158:A6717

Leids Universitair Medisch Centrum, Leiden.

A monoclonal gammopathy is a condition in which a monoclonal immunoglobulin (M-protein, formerly known as paraprotein) produced by a clonal proliferation of plasma cells is present in the blood. The spectrum of monoclonal gammopathies includes monoclonal gammopathy of uncertain significance (MGUS), multiple myeloma, Waldenström disease, plasmacytoma and primary amyloidosis. Various skin diseases are associated with monoclonal gammopathies. Read More

Int J Cancer 2014 Nov 5;135(9):2046-53. Epub 2014 Apr 5.

José-Carreras-Center for Immuno- and Gene Therapy, Internal Medicine I, Saarland University Medical School, Homburg (Saar), Germany.

Hyperphosphorylated paratarg-7 (pP-7) carrier state is the strongest molecularly defined risk factor for monoclonal gammopathy of undetermined significance (MGUS), multiple myeloma (MM) and Waldenstrom's macroglobulinemia (WM). pP-7 is inherited as autosomal-dominant trait and depending on the ethnic background is found in over one-third of MGUS/MM patients. P-7, which is the antigenic paraprotein target in these patients, is hyperphosphorylated at serine17. Read More

Leukemia 2014 Aug 10;28(8):1698-704. Epub 2014 Feb 10.

1] Bing Center for Waldenström's Macroglobulinemia, Dana-Farber Cancer Institute, Boston, MA, USA [2] Department of Medicine, Harvard Medical School, Boston, MA, USA.

MYD88 L265P is highly prevalent in Waldenstrom's Macroglobulinemia (WM) and IgM monoclonal gammopathy of unknown significance (MGUS). We investigated whether MYD88 L265P could be identified by peripheral blood (PB) allele-specific PCR. MYD88 L265P was detected in untreated WM (114/118; 96. Read More

Leuk Lymphoma 2013 Nov 29;54(11):2345-6. Epub 2013 Jul 29.

Department of Medicine, Mayo Clinic , Rochester, MN , USA.

Rev Med Interne 2014 Mar 7;35(3):154-9. Epub 2013 Jun 7.

Service de médecine interne, hôpital européen Georges-Pompidou, AP-HP, 20, rue Leblanc, 75015 Paris, France; Faculté de médecine, université Paris Descartes, Paris Sorbonne Cité, 75005 Paris, France. Electronic address:

Purpose: Whereas von Willebrand disease is the most common constitutional bleeding disorder, acquired von Willebrand syndrome is rare.

Methods: Retrospective, monocentric descriptive study of consecutive cases of acquired von Willebrand syndrome diagnosed between 2000 and 2012. Diagnostic criteria included: absence of a past history of mucocutaneous bleeding, with low plasma levels of factor VIII (FVIII) and von Willebrand factor (VWF), ristocetine cofactor activity (RCo) and antigen (Ag). Read More

Leukemia 2014 Jan 22;28(1):166-73. Epub 2013 Apr 22.

1] Hospital Universitario de Salamanca, Department of Hematology, Salamanca, Spain [2] IBSAL IBMCC (USAL-CSIC), Salamanca, Spain.

Although multiparameter flow cytometry (MFC) has demonstrated clinical relevance in monoclonal gammopathy of undetermined significance (MGUS)/myeloma, immunophenotypic studies on the full spectrum of Waldenström's Macroglobulinemia (WM) remain scanty. Herein, a comprehensive MFC analysis on bone marrow samples from 244 newly diagnosed patients with an immunoglobulin M (IgM) monoclonal protein was performed, including 67 IgM-MGUS, 77 smoldering and 100 symptomatic WM. Our results show a progressive increase on the number and light-chain-isotype-positive B-cells from IgM-MGUS to smoldering and symptomatic WM (P<. Read More

Rev Invest Clin 2012 Nov-Dec;64(6 Pt 2):604-8

Centro de Hematología y Medicina Interna de Puebla.

Material And Methods: In a 30-year period in a single institution, 23 cases of systemic immunoglobulin light chain amyloidosis (AL) were identified, within a group of 1,388 individuals with some form of a hematological malignancy.

Results: AL is 14 times less frequent in Mexico than in Caucasians and it represents 15% of all monoclonal gammopathies. Median age was 57 years (range 39-98); there were 11 males and 12 females. Read More

Arch Pathol Lab Med 2013 Apr;137(4):580-5

Department of Pathology and Laboratory Medicine, University of Wisconsin, Madison, WI 53792-2472, USA.

Lymphoplasmacytic lymphoma (LPL) is a low-grade, B-cell neoplasm composed of small lymphocytes, plasmacytoid lymphocytes, and plasma cells that typically involve the bone marrow, and it is associated with an immunoglobulin M (IgM) gammopathy. The definition of Waldenström macroglobulinemia (WM) and its relationship to LPL has been confusing in the past. In addition, the diagnosis of LPL itself can be challenging because LPL lacks disease-specific morphologic, immunophenotypic, and genetic features to differentiate it from other mature B-cell neoplasms. Read More

Clin Lymphoma Myeloma Leuk 2013 Apr 13;13(2):187-90. Epub 2013 Mar 13.

Department of Medicine, Division of Hematology, Karolinska University Hospital, Solna, Karolinska Institutet, Stockholm, Sweden.

Monoclonal gammopathy of undetermined significance (MGUS) of IgM type is the strongest risk factor for the development of Waldenström macroglobulinemia (WM). The clinical management of WM has changed considerably over recent years, which is reflected in the use of new therapeutic agents (eg, purine analogues, monoclonal antibodies, and thalidomide- and bortezomib-based therapies), risk-and-response-adjusted stratification of treatments, and improvement in supportive care measures. However, because of the rarity of WM, there are few phase III randomized clinical studies to guide therapy and evaluate overall survival. Read More

Clin Lymphoma Myeloma Leuk 2013 Apr 13;13(2):184-6. Epub 2013 Mar 13.

Division of Hematology, College of Medicine, Mayo Clinic, Rochester, MN 55905, USA.

Monoclonal gammopathy of undetermined significance of the immunoglobulin M class was diagnosed in 213 patients at the Mayo Clinic, 29 (14%) of whom developed lymphoma, Waldenström macroglobulinemia, or a related disorder over 1567 person-years of follow-up. The cumulative probability of progression was 10% at 5 years, 18% at 10 years, and 24% at 15 years, or approximately 1.5% per year. Read More