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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

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

Clin Lymphoma Myeloma Leuk 2013 Apr 9;13(2):208-10. Epub 2013 Mar 9.

Department of Hematology, Niguarda Ca' Granda Hospital, Milan, Italy.

Waldenström macroglobulinemia (WM) (symptomatic and indolent) and immunoglobulin M (IgM) monoclonal gammopathy of undetermined significance (IgMMGUS) can be identified based on the bone marrow (BM) infiltration and the existence of symptoms. The purpose of this study was to investigate the biological and genetic characteristics of both disorders comparing the molecular signature of WM versus IgMMGUS using microarray analysis. We investigated BM CD19(+) cells isolated from 21 WM patients and 10 IgMMGUS cases, and CD138(+) BM cells isolated from all of the WM patients and 4 of the IgMMGUS cases. Read More

Neth J Med 2013 Mar;71(2):54-62

St. Antonius Hospital Nieuwegein, The Netherlands.

On behalf of the lymphoma and multiple myeloma working parties of the Dutch/Belgian Haemato-Oncology Foundation for Adults in The Netherlands (HOVON), we present a guideline for diagnosis and management of Waldenström's macroglobulinemia (WM). Considering the indolent behaviour and heterogeneous clinical presentation of WM, it is crucial to determine the right indications for treatment, as well as to individualise therapeutic options. There are significant differences from the approach to multiple myeloma or the treatment of other indolent non-Hodkgin lymphomas, and these results cannot always be extrapolated. Read More

Leuk Lymphoma 2013 Nov 17;54(11):2485-9. Epub 2013 Apr 17.

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

We characterized immunoglobulin heavy chain (IGH) gene rearrangements and searched for clusters of stereotyped B-cell receptors in 123 patients with Waldenström macroglobulinemia (WM; n = 59) or immunoglobulin M monoclonal gammopathy of undetermined significance (IgM-MGUS) (n = 64). A productive monoclonal IGHV-D-J rearrangement was obtained in 99/123 patients (80%). Immunoglobulin heavy chain variable (IGHV) genes were mutated in 94/99 patients (95%) with a median somatic hypermutation rate of 6. Read More

Blood 2013 Mar 25;121(13):2522-8. Epub 2013 Jan 25.

Department of Hematology Oncology, Fondazione IRCCS Policlinico San Matteo, 27100 Pavia, Italy.

A study has shown that MYD88 (L265P) is a recurring somatic mutation in Waldenström's macroglobulinemia (WM). We developed an allele-specific polymerase chain reaction (PCR) for this mutation, and analyzed bone marrow or peripheral blood samples from 58 patients with WM, 77 with IgM monoclonal gammopathy of undetermined significance (IgM-MGUS), 84 with splenic marginal zone lymphoma (SMZL), and 52 with B-cell chronic lymphoproliferative disorders (B-CLPD). MYD88 (L265P) was detected in 58/58 (100%) patients with WM, 36/77 (47%) with IgM-MGUS, 5/84 (6%) with SMZL, and 3/52 (4%) with B-CLPD. Read More

Blood 2013 Mar 15;121(11):2051-8. Epub 2013 Jan 15.

Bing Center for Waldenström's Macroglobulinemia, Dana-Farber Cancer Institute, Boston, MA 02215, USA.

By whole-genome and/or Sanger sequencing, we recently identified a somatic mutation (MYD88 L265P) that stimulates nuclear factor κB activity and is present in >90% of Waldenström macroglobulinemia (WM) patients. MYD88 L265P was absent in 90% of immunoglobulin M (IgM) monoclonal gammopathy of undetermined significance (MGUS) patients. We therefore developed conventional and real-time allele-specific polymerase chain reaction (AS-PCR) assays for more sensitive detection and quantification of MYD88 L265P. Read More

Dermatol Online J 2012 Dec 15;18(12). Epub 2012 Dec 15.

The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, USA.

We report a case of a 33-year-old-woman with a one-year history of bilateral lower extremity vasculitis and laboratory evidence of hypergammaglobulinemia with otherwise unremarkable routine laboratory and rheumatologic studies. Her clinical picture, together with histopathologic evidence of leukocytoclastic vasculitis, favor a diagnosis of hypergammaglobulinemic purpura of Waldenström. Read More

Hematology Am Soc Hematol Educ Program 2012 ;2012:595-603

Amyloidosis Research and Treatment Center, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Policlinico San Matteo, Pavia, Italy.

Monoclonal gammopathy of undetermined significance (MGUS) is an asymptomatic plasma cell disorder occurring in 4.2% of adults > 50 years of age, which can progress into symptomatic diseases either through proliferation of the plasma cell clone, giving rise to multiple myeloma and other lymphoplasmacellular neoplasms, or through organ damage caused by the monoclonal protein, as seen in light-chain amyloidosis and related conditions. Differential diagnosis of asymptomatic and symptomatic monoclonal gammopathies is the determinant for starting therapy. Read More

Am J Clin Pathol 2012 Dec;138(6):867-76

Dept of Pathology, Medical College of Wisconsin, Milwaukee, WI 53226, USA.

The majority of plasma cell myelomas (PCMs) are positive for CD200, a membrane protein with immunosuppressive function. There are no flow cytometry data in the literature on plasma cell CD200 expression in other immunoproliferative disorders. Therefore we used flow cytometry to study the expression of CD200 on plasma cells in diagnostic bone marrow aspirates from 61 patients with monoclonal gammopathy of undetermined significance (MGUS) and 10 patients with lymphoplasmacytic lymphoma (LPL). Read More

Haematologica 2012 Jun 16;97(6):854-8. Epub 2011 Dec 16.

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

No comprehensive evaluation has been made to assess the risk of viral and bacterial infections among patients with monoclonal gammopathy of undetermined significance. Using population-based data from Sweden, we estimated risk of infections among 5,326 monoclonal gammopathy of undetermined significance patients compared to 20,161 matched controls. Patients with monoclonal gammopathy of undetermined significance had a 2-fold increased risk (P<0. Read More

Oncology (Williston Park) 2011 Jun;25(7):578-86

Monoclonal gammopathy of undetermined significance (MGUS) is defined as a serum M protein level of less than 3 g/dL, less than 10% clonal plasma cells in the bone marrow, and the absence of end-organ damage. The prevalence of MGUS is 3.2% in the white population but is approximately twice that high in the black population. Read More

Bol Asoc Med P R 2010 Oct-Dec;102(4):43-6

Hematopoietic Stem Cell Transplant Program, Audie L. Murphy Memorial Veterans Hospital, 7400 Merton Minter Blvd, San Antonio, TX, USA.

Monoclonal gammopathy of undetermined significance (MGUS) is frequently diagnosed in the elderly population. Most physicians will have to evaluate and manage patients with this condition. Recent studies have clarified risk factors, natural history, prognosis and complications of this common condition. Read More

Clin Vaccine Immunol 2011 Jun 20;18(6):969-77. Epub 2011 Apr 20.

Department of Infectious Diseases, NU Hospital Organization, Trollhättan/Uddevalla, Sweden.

Whereas patients with multiple myeloma (MM) have a well-documented susceptibility to infections, this has been less studied in other B-cell disorders, such as Waldenstrom's macroglobulinemia (WM) and monoclonal gammopathy of undetermined significance (MGUS). We investigated the humoral immunity to 24 different pathogens in elderly patients with MM (n = 25), WM (n = 16), and MGUS (n = 18) and in age-matched controls (n = 20). Antibody titers against pneumococci, staphylococcal alpha-toxin, tetanus and diphtheria toxoids, and varicella, mumps, and rubella viruses were most depressed in MM patients, next to lowest in WM and MGUS patients, and highest in the controls. Read More

Clin Lymphoma Myeloma Leuk 2011 Feb;11(1):93-5

University Hospital of Salamanca, Cancer Research Center (IBMCC-CSIC), University of Salamanca, Spain.

The distinction between IgM monoclonal gammopathy of undetermined significance (MGUS), asymptomatic Waldenstrom's macroglobulinemia (WM; aWM), and symptomatic WM (sWM) relies on two features: the presence of infiltration by lymphoplasmacytic lymphoma in the bone marrow (BM) biopsy and the existence of signs or symptoms attributable to the disease. Nevertheless, some patients lack a BM biopsy or it is not conclusive for diagnosis. In this study we have investigated 94 patients with IgM monoclonal gammopathies, in which a BM trephine biopsy and morphological and flow cytometry (FCM) evaluation of BM aspirate were available at diagnosis. Read More

Clin Lymphoma Myeloma Leuk 2011 Feb;11(1):82-4

Faculty of Medicine, University of Iceland, Department of Clinical Haematology, Landspítali University Hospital, Reykjavík, Iceland.

The prevalence of paraproteinemias or monoclonal gammopathies increases with age. No other major risk factors have been recognized, but significant associations have been reported with chronic antigen exposure, agricultural environment, and family history. In around 130 families reported worldwide, IgG or IgA monoclonal gammopathy of undetermined significance (MGUS) occurs with multiple myeloma (MM) whereas Waldenström's macroglobulinemia (WM) is linked to IgM MGUS. Read More