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Ann Hematol 2018 Dec 4. Epub 2018 Dec 4.

Department of Hematology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, People's Republic of China.

Light chain-associated Fanconi syndrome (LCFS) is a disorder of renal proximal tubule due to immunoglobulin light chains. Cases of LCFS are rare and mostly sporadically reported, and treatment of this entity is still controversial. This single-center retrospective study included 22 patients diagnosed with LCFS in Peking Union Medical College Hospital. Read More

Can J Neurol Sci 2018 Nov 29:1-3. Epub 2018 Nov 29.

3Department of Medical Imaging,The Ottawa Hospital,Ottawa,Ontario,Canada.

J Clin Lab Anal 2018 Nov 28:e22827. Epub 2018 Nov 28.

The Central Laboratory, Fujian Medical University Union Hospital, Fuzhou, China.

Background: IgM monoclonal gammopathy can be present in a broad spectrum of diseases. We evaluated the value of serum markers in the differential diagnosis of Waldenstrom macroglobulinemia (WM) and other types of IgM monoclonal gammopathies.

Methods: We included patients who were first admitted to hospital and identified as having IgM monoclonal gammopathy by serum immunofixation electrophoresis (sIFE). Read More

Clin Neuropsychol 2018 Nov 23:1-13. Epub 2018 Nov 23.

a Clinical Neuropsychology, Cognitive Disorders and Dyslexia Unit, Department of Neurology , Arcispedale Santa Maria Nuova-IRCCS , Reggio Emilia , Italy.

Objective: A case report of a 74-year-old male presenting with an atypical multimodal semantic impairment. The patient was diagnosed with Waldenström macroglobulinemia (WM) for which he received allogeneic bone marrow transplantation (BMT) due to disease progression. Following BMT, he developed a sudden onset of semantic difficulties that have remained unchanged for eight years. Read More

Br J Haematol 2018 Nov 20. Epub 2018 Nov 20.

Division of Infectious Diseases, Department of Medicine, Brigham and Women's Hospital, Boston, MA, USA.

Blood Adv 2018 Nov;2(22):3102-3111

Service d'Hématologie Clinique, Centre Hospitalier Schaffner, Lens, France.

Few reports assess prognosis during follow-up of patients with symptomatic Waldenström macroglobulinemia (WM). In 121 WM patients treated between 1993 and 2016, we analyzed the prognostic role during the clinical course of the initial International Prognostic Scoring System for WM (IPSSWM). Then, we assessed onset of response, progression, and second treatment initiation coded as time-dependent covariates. Read More

J Food Sci 2018 Nov 15. Epub 2018 Nov 15.

Dept. of Processing Technology, Nofima AS, P.O. Box 327, NO-4002, Stavanger, Norway.

A comparative evaluation on the effect of carbon dioxide (CO ) on quality and shelf life of Atlantic salmon loins pasteurized with microwave and conventional technology was conducted. The experimental design allowed CO to enter the salmon muscle before (soluble gas stabilization [SGS] + vacuum) or after pasteurization (CO emitter + vacuum), whereas the control samples (vacuum only) were not presented for CO . This setup resulted in six different groups; three heated with microwaves and three with conventional pasteurization. Read More

N Engl J Med 2018 11;379(20):1974-5

University of Ferrara, Ferrara, Italy

N Engl J Med 2018 11;379(20):1973-4

Bing Center for Waldenström’s Macroglobulinemia, Boston, MA

N Engl J Med 2018 11;379(20):1975-1976

University Hospital of Ulm, Ulm, Germany

PLoS One 2018 9;13(11):e0206502. Epub 2018 Nov 9.

Facultad de Ciencias Veterinarias, Universidad de Concepción, Chillán, Chile.

A lack of knowledge of naturally occurring pathogens is limiting our ability to use the Antarctic to study the impact human-mediated introduction of infectious microorganisms have on this relatively uncontaminated environment. As no large-scale coordinated effort to remedy this lack of knowledge has taken place, we rely on smaller targeted efforts to both study present microorganisms and monitor the environment for introductions. In one such effort, we isolated Campylobacter species from fecal samples collected from wild birds in the Antarctic Peninsula and the sub-Antarctic island of South Georgia. Read More

Br J Haematol 2018 Nov 8. Epub 2018 Nov 8.

Bing Center for Waldenstrom Macroglobulinemia, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA.

Br J Haematol 2018 Nov 8. Epub 2018 Nov 8.

National Clinical Research Center for Cancer, Key Laboratory of Cancer Prevention and Therapy, Tianjin Medical University Cancer Institute and Hospital, Tianjin, China.

Biomed Res Int 2018 4;2018:6728128. Epub 2018 Oct 4.

Department of Hematology, National Cancer Center Hospital, Tsukiji, Tokyo 104-0045, Japan.

Background: Waldenström macroglobulinemia (WM) is a rare, indolent B-cell lymphoma. Clinically, chromosome 6q deletion (6q del) including loss of the B lymphocyte-induced maturation protein 1 gene (BLIMP-1) is reported to be associated with poor prognosis. However, it remains unclear how the underlying biological mechanism contributes to the aggressiveness of WM with 6q del. Read More

Blood Adv 2018 Nov;2(21):2937-2946

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

Activating mutations are present in 95% of Waldenström macroglobulinemia (WM) patients, and trigger NF-κB through BTK and IRAK. The BTK inhibitor ibrutinib is active in mutated ) WM patients, but shows lower activity in wild-type ( ) disease. patients also show shorter overall survival, and increased risk of disease transformation in some series. Read More

Drugs 2018 Nov;78(16):1653-1663

Department of Hematology, Rigshospitalet, Blegdamsvej 9, 2100, Copenhagen, Denmark.

Bruton's tyrosine kinase (BTK) is crucial in B-cell development and survival. The role of BTK as a downstream kinase in the B-cell receptor (BCR) signaling pathway is well described. As a key player in the pathogenesis of B-cell malignancies, targeting of dysregulated BCR signaling has been explored by development of inhibitors of downstream mediators. Read More

Nephrol Dial Transplant 2018 Oct 31. Epub 2018 Oct 31.

Division of Kidney Diseases and Hypertension, Zucker School of Medicine at Hofstra/Northwell, Hempstead, NY, USA.

Waldenström macroglobulinemia (WM) is a rare B-cell lymphoma characterized by lymphoplasmacytic cell infiltration in the bone marrow and other organs and the presence of a monoclonal immunoglobulin M protein in the serum. Although uncommon, several kidney diseases have been associated with WM. In addition to kidney diseases related to lymphoplasmacytic lymphoma infiltration, a variety of glomerular and tubular lesions have been described in patients with WM. Read More

J Oral Microbiol 2018 17;10(1):1527655. Epub 2018 Oct 17.

Department of Oral Microbiology and Immunology, Institute of Odontology, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden.

: To investigate oral diseases and microbiological conditions, such as the presence of ureolytic bacteria in dental plaque, in relation to experience of stomach pain in a remote adult Asian population. : Ninety-three adults, 40-60-years old, from the Karen Hill tribe in Northern Thailand with no regular access to dental care were examined. Clinical registrations were performed and interproximal gingival plaque samples were collected and analyzed with the checkerboard (CKB) method for the presence of 14 oral bacterial species. Read More

Hum Pathol 2018 Oct 17. Epub 2018 Oct 17.

Department of Pathology and Laboratory Medicine, Hematology/Oncology Division, University of California Irvine (UCI) Medical Center, Orange, California. Electronic address:

Although rare cases of prolymphocytic transformation from splenic B-cell lymphomas and follicular lymphoma have been reported, prolymphocytic transformation from lymphoplasmacytic lymphoma has not been previously reported. We report a case of 76-year old male patient with a history of Waldenström's Macroglobulinemia diagnosed in 2010 and treated with infusion chemotherapy. He was in clinical remission for 5years. Read More

Br J Haematol 2018 Oct 18. Epub 2018 Oct 18.

Haematology, Concord Repatriation General Hospital, Sydney, Australia.

J Nephrol 2018 Dec 17;31(6):907-918. Epub 2018 Oct 17.

Department of Pathology, Stanford University Medical Center, 300 Pasteur Drive, L235, Stanford, CA, 94305, USA.

IgM secreting myelomas or lymphomas, including Waldenström macroglobulinemia, are associated with a varied spectrum of renal pathology, including intracapillary hyaline deposits, cryoglobulin, membranoproliferative glomerulonephritis, amyloid, monoclonal immunoglobulin deposition disease, cast nephropathy, and lymphoma infiltration. We report our single institution experience, and describe five cases with distinctive glomerular pathology: intracapillary IgM pseudothrombi and thrombotic microangiopathic change, with glomerular intracellular crystals in two biopsies. Two patients were hypocomplementemic at presentation. Read More

Am J Hematol 2018 Oct 17. Epub 2018 Oct 17.

Division of Hematology, Mayo Clinic, Rochester, Minnesota.

Disease Overview: Waldenström macroglobulinemia (WM) is a lymphoplasmacytic lymphoma with immunoglobulin M (IgM) monoclonal protein. Clinical features include anemia, thrombocytopenia, hepatosplenomegaly, lymphadenopathy, and rarely hyperviscosity.

Diagnosis: Presence of IgM monoclonal protein associated with ≥10% clonal lymphoplasmacytic cells in bone marrow confirms the diagnosis. Read More

Arch Soc Esp Oftalmol 2018 Oct 11. Epub 2018 Oct 11.

Servicio de Oftalmología, Hospital Universitario de Gran Canaria Doctor Negrín, Las Palmas de Gran Canaria, España.

A 66 year-old patient, monitored for diabetic retinopathy refractory to multiple treatment methods despite a good metabolic control, referred to progressive weight loss. For this reason, a systemic study was performed, detecting anaemia, elevation of the erythrocyte sedimentation rate, and hyperproteinaemia due to elevated serum levels of monoclonal IgM. Subsequently, by performing a bone marrow biopsy and genetic study, the diagnosis of Waldenström macroglobulinaemia was made. Read More

Leukemia 2018 Oct 12. Epub 2018 Oct 12.

Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, MN, USA.

Blood Res 2018 Sep 28;53(3):189-197. Epub 2018 Sep 28.

Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Background: The incidence of lymphoplasmacytic lymphoma (LPL) is lower in Asian than in Western populations. Few studies have described the clinical features and treatment outcomes of patients with LPL, including non-IgM LPL, in East Asia.

Methods: We retrospectively analyzed patients diagnosed with LPL at Asan Medical Center between January 2001 and March 2016. Read More

Nat Commun 2018 10 10;9(1):4182. Epub 2018 Oct 10.

Division of Cancer Epidemiology and Genetics, National Cancer Institute, Bethesda, 20892, MD, USA.

Waldenström macroglobulinemia (WM)/lymphoplasmacytic lymphoma (LPL) is a rare, chronic B-cell lymphoma with high heritability. We conduct a two-stage genome-wide association study of WM/LPL in 530 unrelated cases and 4362 controls of European ancestry and identify two high-risk loci associated with WM/LPL at 6p25.3 (rs116446171, near EXOC2 and IRF4; OR = 21. Read More

Blood Cancer J 2018 Oct 9;8(10):97. Epub 2018 Oct 9.

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

MYD88 mutations are one of the most recurrent mutations in hematologic malignancies. However, recent mouse models suggest that MYD88 alone may not be sufficient to induce tumor formation. Interplay between MYD88 and other genetic events is further supported by the fact that TNFAIP3 (A20) inactivation often accompanies MYD88. Read More

PLoS One 2018 4;13(10):e0204589. Epub 2018 Oct 4.

Department of Medical Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston MA, United States of America.

Waldenström Macroglobulinemia (WM) is a low-grade B-cell lymphoma characterized by disease progression from IgM MGUS to asymptomatic and then symptomatic disease states. We profiled exosomes from the peripheral blood of patients with WM at different stages (30 smoldering/asymptomatic WM, 44 symptomatic WM samples and 10 healthy controls) to define their role as potential biomarkers of disease progression. In this study, we showed that circulating exosomes and their miRNA content represent unique markers of the tumor and its microenvironment. Read More

BMJ Case Rep 2018 Oct 2;2018. Epub 2018 Oct 2.

Tripler Army Medical Center, Honolulu, Hawaii, USA.

The authors present a case of an elderly man with a history of Waldenstrom macroglobulinaemia in remission who presented with progressively worsening gait abnormalities and falls for several months. His examination was notable for bilateral lower extremity weakness and an unsteady gait. Brain and spinal MRI showed focal leptomeningeal enhancement in the brain and spinal column. Read More

J Eur Acad Dermatol Venereol 2018 Sep 29. Epub 2018 Sep 29.

Hôpitaux de Paris, Saint-Louis Hospital, Department of Immuno-hematology, Paris, France.

The presence of a serum monoclonal immunoglobulin can lead to complications related to the physiochemical properties of circulating immunoglobulin and/or its deposition in different tissues, such as kidney, peripheral nervous system and skin. Specific cutaneous manifestations are rare. They are referred to as a Monoclonal Gammopathy of Cutaneous Significance (MGCS). Read More

JAAD Case Rep 2018 Sep 14;4(8):784-787. Epub 2018 Sep 14.

Division of Dermatology, University of Calgary, Richmond Road Diagnostic and Treatment Centre, Calgary, Canada.

J Glob Oncol 2018 Sep 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

J Thorac Imaging 2018 Nov;33(6):W57-W59

Departments of Diagnostic Imaging and Nuclear Medicine.

Ann Biol Clin (Paris) 2018 Sep 21. Epub 2018 Sep 21.

Laboratoire d'hématologie, CHU de Toulouse, IUC-T, Toulouse, France.

Lymphoplasmacytic lymphoma is rare. Although most cases of LPL are Waldenström macroglobulinemia associated with an immunoglobulin M, there are exceptions. Indeed, few cases are immunoglobulin A-secreting or immunoglobulin G-secreting. Read More

Case Rep Hematol 2018 28;2018:8573105. Epub 2018 Aug 28.

Center for Lymphoid Malignancies, Columbia University Medical Center-College of Physicians and Surgeons, New York, NY, USA.

Bing-Neel syndrome is a rare manifestation of Waldenström macroglobulinemia characterized by lymphoplasmacytic cells' infiltration into the central nervous system. We present a case of a 74-year-old patient with a known diagnosis of Waldenström macroglobulinemia and newly depressed consciousness. Flow cytology of his cerebral spinal fluid demonstrated a lambda light chain-restricted population of B-cells consistent with a CD5+ CD10+ B-cell lymphoma. Read More

Leuk Lymphoma 2018 Sep 19:1-7. Epub 2018 Sep 19.

b Department of Lymphoma/Myeloma , The University of Texas MD Anderson Cancer Center , Houston , TX , U.S.A.

Multiple myeloma (MM) and Waldenström's macroglobulinemia (WM) are plasma cell disorders often treated with proteasome inhibitors. Recently, several studies evaluated carfilzomib as an initial treatment for these diseases and reported outstanding clinical outcomes. We conducted a retrospective study to report the efficacy and safety of frontline carfilzomib-based combinations in a standard of care setting. Read More

Acta Haematol 2018 18;140(2):87-96. Epub 2018 Sep 18.

Waldenström macroglobulinemia (WM), also known as lymphoplasmacytic lymphoma, is rare but a clinicopathologically distinct B-cell malignancy. This study assessed differentially expressed genes (DEGs) to identify potential WM biomarkers and uncover the underlying the molecular mechanisms of WM progression using gene expression profiles from the Gene Expression Omnibus database. DEGs were identified using the LIMMA package and their potential functions were then analyzed by using the gene ontology (GO) and Kyoto Encyclopedia of Genes and Genomes pathway enrichment analyses and the protein-protein interaction (PPI) network analysis by using the Search Tool for the Retrieval of Interacting Genes/Proteins database. Read More

Ann Biol Clin (Paris) 2018 Oct;76(5):575-578

GCS de Saintonge, Centres hospitaliers de Saint-Jean-d'Angély, Jonzac, Saintes et Royan, Saint-Jean-d'Angély, France.

The presence of serum monoclonal IgM is often associated with the diagnosis of Waldenström macroglobulinemia (WM) or other chronic lymphoproliferative disorders. IgM myeloma is a rare entity (0.5%). Read More

Clin Lymphoma Myeloma Leuk 2018 Aug 23. Epub 2018 Aug 23.

Department of Oncology, Alexandra Hospital, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece; Department of Clinical Therapeutics, Alexandra Hospital, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece.

Background: Waldenström's macroglobulinemia (WM) is a rare malignancy characterized by bone marrow infiltration by lymphoplasmacytic cells and the presence of a monoclonal IgM paraprotein. The interactions of lymphoplasmacytic cells with other cells in their microenvironment, including mast cells and endothelial cells, support their survival and proliferation and can induce resistance to therapy. von Willebrand factor (vWF) plays a key role in primary hemostasis but is also a marker of endothelial "stimulation. Read More

Am Fam Physician 2018 Jun;97(11):741-748

Novant health Family Medicine Residency, Charlotte, NC, USA.

Most oncologic emergencies can be classified as metabolic, hematologic, structural, or treatment related. Tumor lysis syndrome is a metabolic emergency that presents as severe electrolyte abnormalities. Stabilization is focused on vigorous rehydration, maintaining urine output, and lowering uric acid levels. Read More

Clin Cancer Res 2018 Sep 11. Epub 2018 Sep 11.

LeBow Institute for Myeloma Therapeutics and Jerome Lipper Multiple Myeloma Center, Dana-Farber Cancer Institute, Harvard Medical School, Boston, Massachusetts.

p21-activated kinase 4 (PAK4) plays a significant biological and functional role in a number of malignancies, including multiple myeloma (MM). On the basis of our promising findings in MM, we here characterize PAK4 expression and role in WM cells, as well effect of dual PAK4-NAMPT inhibitor (KPT-9274) against WM cell growth and viability. We have analyzed mRNA and protein expression levels of PAK4 in WM cells, and used loss-of-function approach to investigate its contribution to WM cell viability. Read More

Br J Haematol 2018 Sep 10. Epub 2018 Sep 10.

Service d'Hématologie-Immunologie-Transfusion, Hôpitaux Universitaires Paris Ile De France Ouest, Université Versailles Saint Quentin, Boulogne, France.

The diagnosis of Waldenström Macroglobulinaemia (WM)/lymphoplasmacytic lymphoma (LPL) remains one of exclusion because other B-cell lymphoproliferative disorders (B-LPD), such as marginal zone lymphoma (MZL), can fulfil similar criteria, including MYD88 L265P mutation. It has been suggested that expression of the myeloid marker CD13 (also termed ANPEP) is more frequent in LPL than in other B-LPD and has also been described on normal and malignant plasma cells. Here, CD13 expression was tested in a cohort of 1037 B-LPD patients from 3 centres by flow cytometry. Read More

Br J Haematol 2018 Nov 10;183(4):564-577. Epub 2018 Sep 10.

Department of Haematology, Karolinska Institute, Stockholm, Sweden.

We present a nationwide prospective Swedish registry-based study of Waldenström macroglobulinaemia (WM), that focuses on incidence and survival in relation to clinical prognostic factors and primary systemic therapies. A total of 1511 patients with WM and lymphoplasmocytic lymphoma (LPL) were registered in the Swedish Lymphoma Registry (SLR) between 1 January 2000 and 31 December 2014. The age-adjusted incidence of WM/LPL was 11·5 per million persons per year, three times higher than the reported incidence worldwide. Read More

Hematol Oncol 2018 Sep 7. 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 Oct;32(5):xiii-xiv

Bing Center for Waldenström's Macroglobulinemia, Dana-Farber Cancer Institute, Harvard Medical School, 450 Brookline Avenue, M548, Boston, MA 02215, USA. Electronic address:

Hematol Oncol Clin North Am 2018 Oct 19;32(5):875-890. Epub 2018 Jul 19.

Division of Oncology, Department of Medicine, Stanford University, Stanford Cancer Institute, 875 Blake Wilbur Drive, Stanford, CA 94305, USA. Electronic address:

Recent advances in the understanding of Waldenström macroglobulinemia (WM) biology have paved the way for development of a plethora of novel therapeutic strategies. The success of ibrutinib in WM has shifted treatment paradigms away from conventional chemoimmunotherapy approaches. Recognition of high-risk genomic subgroups as well as mechanisms of acquired resistance to ibrutinib have led to targeting of additional pathways. Read More

Hematol Oncol Clin North Am 2018 Oct 27;32(5):865-874. Epub 2018 Jul 27.

Department of Haematology, University College London, Huntley Street, London WC1E 6AG, UK. Electronic address:

Waldenström macroglobulinemia (WM) is an indolent low-grade non-Hodgkin lymphoma characterized by bone marrow infiltration by lymphoplasmacytic cells and associated clonal IgM paraproteinemia. Recent insights into the biology and genomic characteristics of WM have provided a further platform for more targeted therapies. Despite the high response rates and better depth and duration of responses, the disease remains incurable. Read More

Hematol Oncol Clin North Am 2018 Oct 19;32(5):853-864. Epub 2018 Jul 19.

Department of Medicine, Lymphoma Service, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, NY 10065, USA. Electronic address:

Waldenström macroglobulinemia (WM) is an indolent B-cell lymphoma that is heavily dependent on Bruton tyrosine kinase (BTK) hyperactivation. Ibrutinib is a first-generation BTK inhibitor that has shown high activity and durable responses in patients with relapsed/refractory WM. Newer and more selective BTK inhibitors are currently being tested in several clinical trials and are expected to address the toxicity and the acquired resistance observed in patients receiving ibrutinib. Read More

Hematol Oncol Clin North Am 2018 Oct 21;32(5):841-852. Epub 2018 Jul 21.

Bing Center for Waldenstrom Macroglobulinemia, Dana-Farber Cancer Institute, Harvard Medical School, 450 Brookline Avenue, Mayer 221, Boston, MA 02215, USA. Electronic address:

For the last 2 decades, anti-CD20 monoclonal antibodies have revolutionized the treatment of patients with B-cell lymphomas. These agents have shown efficacy when used as single agents and also have improved response and survival rates when added to chemotherapy. Monoclonal antibodies are safe and effective as well in patients with Waldenström macroglobulinemia (WM). Read More

Hematol Oncol Clin North Am 2018 Oct 25;32(5):829-840. Epub 2018 Jul 25.

Department of Clinical Therapeutics, National and Kapodistrian University of Athens School of Medicine, 80 Vassilisis Sofias Avenue, Athens 11528, Greece.

Waldenström macroglobulinemia (WM) remains an incurable B-cell lymphoproliferative disorder, yet therapy is only considered for patients with symptomatic disease. Primary therapy options for WM include combinations based on anti-CD20 monoclonal antibodies, mainly rituximab. However, proteasome inhibitors have become an important part of WM therapy both as primary therapy and as salvage option. Read More