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https://www.readbyqxmd.com/read/28390781/clinical-disorders-responsible-for-plasma-hyperviscosity-and-skin-complications
#1
REVIEW
Gregorio Caimi, Melania Carlisi, Caterina Urso, Rosalia Lo Presti, Eugenia Hopps
In this brief review, we have examined some clinical disorders which are associated to an altered hemorheological profile and at times accompanied by skin ulcers. This skin condition may be, in fact, observed in patients with primary plasma hyperviscosity such as multiple myeloma, Waldenstrom macroglobulinemia, cryoglobulinemia, cryofibrinogenemia, dysfibrinogenemia and connective tissue diseases. It must be underlined that the altered hemorheological pattern is not the only responsible for this skin complication but, as it worsens the microcirculatory flow, it contributes to determine the occurrence of the skin ulcers...
April 5, 2017: European Journal of Internal Medicine
https://www.readbyqxmd.com/read/28333850/multimodality-ocular-imaging-in-a-case-report-of-hyperviscosity-syndrome-associated-with-lymphoplasmacytic-leukemia-the-images-tell-the-story
#2
Rene Y Choi, Rachel Jacoby, Akbar Shakoor
PURPOSE: To report the clinical course of a patient with ocular manifestations of hyperviscosity syndrome associated with Waldenström macroglobulinemia, and for the first time, video imaging of mobile emboli in the conjunctival and retinal vasculature. METHODS AND PATIENT: A 60-year-old woman with newly diagnosed Waldenström macroglobulinemia, with no visual complaints was evaluated by the Ophthalmology service for a baseline ocular examination. RESULTS: At presentation, ocular examination revealed a visual acuity of 20/25 in each eye...
March 22, 2017: Retinal Cases & Brief Reports
https://www.readbyqxmd.com/read/28294689/genomics-signaling-and-treatment-of-waldenstr%C3%A3-m-macroglobulinemia
#3
Zachary R Hunter, Guang Yang, Lian Xu, Xia Liu, Jorge J Castillo, Steven P Treon
Next-generation sequencing has revealed recurring somatic mutations in Waldenström macroglobulinemia (WM). Commonly recurring mutations include MYD88 (95% to 97%), CXCR4 (30% to 40%), ARID1A (17%), and CD79B (8% to 15%). Diagnostic discrimination of WM from overlapping B-cell malignancies is aided by MYD88 mutation status. Transcription is affected by MYD88 and CXCR4 mutations and includes overexpression of genes involved in VDJ recombination, CXCR4 pathway signaling, and BCL2 family members. Among patients with MYD88 mutations, those with CXCR4 mutations show transcriptional silencing of tumor suppressors associated with acquisition of mutated MYD88...
February 13, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28199189/presence-of-psa-antibodies-in-seminal-plasma-of-infertile-men
#4
Vanessa A Cuppett, Suresh C Sikka, Rajesh K Naz
Immunoinfertility due to antisperm antibodies and semen hyperviscosity are among major causes of male infertility. Although the modulation of prostate-specific antigen (PSA) has been investigated in prostate abnormalities, its role and the effect of its dysfunction in male fertility/infertility have not been extensively examined. The present study was conducted to examine the presence of PSA antibodies locally in the seminal plasma of men having immunoinfertility and semen hyperviscosity. Seminal plasma samples from immunoinfertile men (n=25), men with hyperviscous semen (n=25), and normal men (n=24) were collected and analyzed for immunoreactivity with PSA in ELISA and Western blot...
March 1, 2017: Frontiers in Bioscience (Elite Edition)
https://www.readbyqxmd.com/read/28190444/management-of-neuro-oncologic-emergencies
#5
REVIEW
J T Jo, D Schiff
Patients with brain tumors and systemic malignancies are subject to diverse neurologic complications that require urgent evaluation and treatment. These neurologic conditions are commonly due to the tumor's direct effects on the nervous system, such as cerebral edema, increased intracranial pressure, seizures, spinal cord compression, and leptomeningeal metastases. In addition, neurologic complications can develop as a result of thrombocytopenia, coagulopathy, hyperviscosity syndromes, infection, immune-related disorders, and adverse effects of treatment...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28146331/plasma-exchange-in-the-intensive-care-unit-technical-aspects-and-complications
#6
Aurélie Lemaire, Nathalie Parquet, Lionel Galicier, David Boutboul, Rémi Bertinchamp, Marion Malphettes, Guillaume Dumas, Eric Mariotte, Marie-Noëlle Peraldi, Virginie Souppart, Benoit Schlemmer, Elie Azoulay, Emmanuel Canet
BACKGROUND: Data on plasma exchange therapy in the intensive care unit (ICU) setting are scarce. We aimed to describe the technical aspects and the adverse events associated with the procedure in critically ill patients. METHODS: All adult patients treated by plasma exchange in the medical ICU of the Saint-Louis university hospital between January 1, 2013 and March 31, 2015 were prospectively included. RESULTS: We report on 260 plasma exchange procedures performed in 50 patients...
February 1, 2017: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/28116769/capillary-electrophoresis-analysis-of-n-glycosylation-changes-of-serum-paraproteins-in-multiple-myeloma
#7
Zsuzsanna Kovacs, Adam Simon, Zoltan Szabo, Zsolt Nagy, Laszlo Varoczy, Ildiko Pal, Eszter Csanky, Andras Guttman
Multiple myeloma (MM) is an immedicable malignancy of the human plasma cells producing abnormal antibodies (also referred to as paraproteins) leading to kidney problems and hyperviscosity syndrome. In this paper we report on the N-glycosylation analysis of paraproteins from total human serum as well as the Fc and Fab κ/λ light chain fractions of papain digested immunoglobulins from multiple myeloma patients. Capillary electrophoresis with laser induced fluorescence detection (CE-LIF) was used for the analysis of the N-glycans after endoglycosidase (PNGase F) mediated sugar release and fluorophore labeling (APTS)...
January 24, 2017: Electrophoresis
https://www.readbyqxmd.com/read/28094456/waldenstr%C3%A3-m-macroglobulinemia-2017-update-on-diagnosis-risk-stratification-and-management
#8
Morie A Gertz
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. The L265P mutation in MYD88 is detectable in more than 90% of patients. Risk Stratification: Age, hemoglobin level, platelet count, β2 microglobulin, and monoclonal IgM concentrations are characteristics required for prognosis...
February 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28092215/therapeutic-implications-from-the-pathogenesis-of-raynaud-s-phenomenon
#9
Ariane L Herrick
Raynaud's phenomenon (RP) can be either primary (idiopathic) or secondary to a number of different diseases/conditions, when vasopasm can be superimposed upon structural vascular abnormality or a hyperviscosity state and may then lead to severe ischaemia with tissue damage. Treatment must be tailored to the individual. Areas covered: This review discusses how increased understanding of the pathogenesis of RP has driven and is driving new approaches to therapy, and how we are now better able to predict which patients presenting with RP are likely to have an underlying disease requiring specific intervention...
February 3, 2017: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/28056114/diagnosis-and-management-of-waldenstr%C3%A3-m-macroglobulinemia-mayo-stratification-of-macroglobulinemia-and-risk-adapted-therapy-msmart-guidelines-2016
#10
Prashant Kapoor, Stephen M Ansell, Rafael Fonseca, Asher Chanan-Khan, Robert A Kyle, Shaji K Kumar, Joseph R Mikhael, Thomas E Witzig, Michelle Mauermann, Angela Dispenzieri, Sikander Ailawadhi, A Keith Stewart, Martha Q Lacy, Carrie A Thompson, Francis K Buadi, David Dingli, William G Morice, Ronald S Go, Dragan Jevremovic, Taimur Sher, Rebecca L King, Esteban Braggio, Ann Novak, Vivek Roy, Rhett P Ketterling, Patricia T Greipp, Martha Grogan, Ivana N Micallef, P Leif Bergsagel, Joseph P Colgan, Nelson Leung, Wilson I Gonsalves, Yi Lin, David J Inwards, Suzanne R Hayman, Grzegorz S Nowakowski, Patrick B Johnston, Steven J Russell, Svetomir N Markovic, Steven R Zeldenrust, Yi L Hwa, John A Lust, Luis F Porrata, Thomas M Habermann, S Vincent Rajkumar, Morie A Gertz, Craig B Reeder
Importance: Waldenström macroglobulinemia (WM), an IgM-associated lymphoplasmacytic lymphoma, has witnessed several practice-altering advances in recent years. With availability of a wider array of therapies, the management strategies have become increasingly complex. Our multidisciplinary team appraised studies published or presented up to December 2015 to provide consensus recommendations for a risk-adapted approach to WM, using a grading system. Observations: Waldenström macroglobulinemia remains a rare, incurable cancer, with a heterogeneous disease course...
January 5, 2017: JAMA Oncology
https://www.readbyqxmd.com/read/28024494/-pet-ct-manifestation-and-clinical-analysis-of-waldenstrom-macroglobulinemia
#11
Chong-Yang Ding, Hong-Yu Liu, Zhe Guo, Tian-Nu Li
OBJECTIVE: To investigate the PET-CT manifestation and clinical features of patients with Waldenstrom macroglobulinemia(WM) . METHODS: The clinical features, laboratorial examination results and PET-CT manifestation of 12 patients with WM were analyzed retrospectively. RESULTS: The average age of 12 patients with WM was 63.5 years old, the most common incipient symptoms were fatigue and hyperviscosity syndrome. The median SUVmax of bone marrow was 4...
December 2016: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/27851348/1713-jelly-blood-the-importance-of-a-clinical-diagnosis-of-hyperviscosity-syndrome
#12
Kathryn Waksmundzki, Simon Mucha
No abstract text is available yet for this article.
December 2016: Critical Care Medicine
https://www.readbyqxmd.com/read/27836860/prospective-multicenter-clinical-trial-of-everolimus-as-primary-therapy-in-waldenstrom-macroglobulinemia-wmctg-09-214
#13
Steven P Treon, Kirsten Meid, Christina Tripsas, Leonard T Heffner, Herbert Eradat, Ashraf Z Badros, Lian Xu, Zachary R Hunter, Guang Yang, Christopher J Patterson, Joshua Gustine, Jorge J Castillo, Jeffrey Matous, Irene M Ghobrial
Purpose: Everolimus inhibits mTOR, a component of PI3K/AKT prosurvival signaling triggered by MYD88 and CXCR4-activating mutations in Waldenstrom macroglobulinemia.Experimental design: We evaluated everolimus in a prospective, multicenter study of 33 symptomatic, previously untreated Waldenstrom macroglobulinemia patients. Intended therapy consisted of everolimus (10 mg/day) until progression or unacceptable toxicity. Dose deescalation was permitted. The study was registered at www.clinicaltrials.gov (NCT01470196)...
November 11, 2016: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/27827323/sequence-variation-at-klk-and-wfdc-clusters-and-its-association-to-semen-hyperviscosity-and-other-male-infertility-phenotypes
#14
Patrícia Isabel Marques, Filipa Fonseca, Ana Sofia Carvalho, Diana A Puente, Isabel Damião, Vasco Almeida, Nuno Barros, Alberto Barros, Filipa Carvalho, Mikel Azkargorta, Felix Elortza, Hugo Osório, Rune Matthiesen, Victor Quesada, Susana Seixas
STUDY QUESTION: Are kallikreins (KLKs), the whey-acidic-protein four-disulfide core domain (WFDCs) and their neighbors, semenogelins (SEMGs), known to play a role in the cascade of semen coagulation and liquefaction, associated with male infertility? SUMMARY ANSWER: Several KLK and SEMG variants are overrepresented among hyperviscosity, asthenozoospermia and oligozoospermia, supporting an effect of abnormal semen liquefaction on the loss of semen quality and in lowering male reproductive fitness...
November 7, 2016: Human Reproduction
https://www.readbyqxmd.com/read/27825464/prognostic-factors-and-indications-for-treatment-of-waldenstr%C3%A3-m-s-macroglobulinemia
#15
REVIEW
Robert A Kyle, Stephen M Ansell, Prashant Kapoor
Waldenström's Macroglobulinemia (WM) is characterized by the presence of an IgM monoclonal protein regardless of its size, 10% or more bone marrow infiltration by small lymphocytes with a plasmacytoid or plasma cell differentiation. These cells usually have the following markers: IgM+, CD5(-), CD10(-), CD19(+), CD20(+) and CD23(-). Chronic lymphocytic leukemia as well as other lymphoproliferative disorders such as mantle cell, marginal zone and mucosa-associated lymphoid tissue (MALT) lymphoma must be excluded...
June 2016: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/27825460/bone-marrow-microenvironment-in-waldenstrom-s-macroglobulinemia
#16
REVIEW
Shahrzad Jalali, Stephen M Ansell
Waldenstrom Macroglobulinemia (WM) is a low-grade B-cell lymphoma defined firstly by infiltration of lymphoplasmacytic cells into the bone marrow (BM), the milieu where the cells acquire signals that promote malignant growth and proliferation. A second characteristic associated with WM is the increased synthesis of monoclonal immunoglobulin M (IgM) by lymphoplasmacytic cells, which is secreted in the serum and often results in hyperviscosity. Advanced genomic tools have improved our understanding of the genetic events that contribute to malignant transformation in WM, but the role of BM microenvironment is also emerging as having an essential role in WM disease progression...
June 2016: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/27818812/cns-vasculitis-associated-with-waldenstr%C3%A3-m-macroglobulinemia
#17
Tanawan Riangwiwat, Chris Y Wu, Alberto S Santos-Ocampo, Randal J Liu, Aaron M McMurtray, Beau K Nakamoto
Waldenström macroglobulinemia (WM) is an indolent B cell lymphoproliferative disorder with monoclonal IgM secretion. We present a patient with WM who presented with multifocal acute cortical ischemic strokes and was found to have central nervous system (CNS) vasculitis. Workup was negative for cryoglobulins and hyperviscosity syndrome. Immunosuppression with intravenous steroids and cyclophosphamide stabilized the patient's mental status and neurologic deficits. On followup over 7 years, patient gained independence from walking aids and experienced no recurrences of CNS vasculitis...
2016: Case Reports in Neurological Medicine
https://www.readbyqxmd.com/read/27796129/-diagnosis-and-treatment-of-polycythaemia-vera-state-of-the-art
#18
Imelda Marton, Zsófia Simon, Zita Borbényi
Polycythaemia vera (PV), a condition characterized by blood hyperviscosity due to the expansion of the erythrocyte mass is the most common entity among all Philadelphia chromosome-negative myeloproliferative neoplasms. Arterial and venous thrombotic events are leading determinants of morbidity and mortality but impairment of quality of life due to vasomotor symptoms (erythromelalgia, pruritus) and disease-associated symptoms (tiredness, fatigue, pruritus, night sweats, vision problems, headache, concentration loss, abdominal discomfort, early satiety, fever, weight loss) are also present...
October 2016: Orvosi Hetilap
https://www.readbyqxmd.com/read/27637720/high-oxygen-affinity-hemoglobins
#19
O Mangin
High oxygen affinity hemoglobins are responsible for rare and heterogeneous autosomic dominant genetic diseases. They cause pure erythrocytosis, sometimes accountable for hyperviscosity and thrombosis, or hemolysis. Differential diagnoses must be first ruled out. The diagnosis is based on the identification of a decreased P50, and their possible characterization by cation exchange-high performance liquid chromatography and capillary electrophoresis. Finally, genetic studies of the responsible globin chain gene will confirm the mutation...
September 13, 2016: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/27557753/erythrocyte-oxidative-stress-is-associated-with-cell-deformability-in-patients-with-retinal-vein-occlusion
#20
M Becatti, R Marcucci, A M Gori, L Mannini, E Grifoni, A Alessandrello Liotta, A Sodi, R Tartaro, N Taddei, S Rizzo, D Prisco, R Abbate, C Fiorillo
Essentials Retinal vein occlusion (RVO), characterized by blood hyperviscosity, has an unclear pathogenesis. We aimed to find out if hemorheological profile is altered by oxidative stress in RVO patients. Red blood cell (RBC) oxidative stress is associated to whole blood viscosity and RBC deformability. Reactive oxygen species alter RBC membrane rigidity, playing a key role in RVO pathogenesis. SUMMARY: Background Retinal vein occlusion (RVO) is characterized by vision loss resulting from hypoperfusion and hypoxia of the retina...
August 25, 2016: Journal of Thrombosis and Haemostasis: JTH
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