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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
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
Subina Narang, Panchmi Gupta, Anuj Sharma, Sunandan Sood, Anshu Palta, Shilpa Goyal
Central retinal vein occlusion (CRVO) is a common pathology of the retinal vasculature. Patients with CRVO usually present with a drop in visual acuity. The condition bears no specific therapy; treatment is aimed at the management of potentially blinding complications, of which there are many. With majority of cases being unilateral, bilateral CRVO is usually associated with an underlying systemic illness such as a hyperviscosity syndrome. Here, we present a case of a patient, who presented with a bilateral drop in vision diagnosed as bilateral CRVO on ophthalmic evaluation...
July 2016: Middle East African Journal of Ophthalmology
Ruzicic Dusan, Kovacevic Relja, Mirkovic Marija, Radovanovic Jelena, Krstevska Vesna, Terzic Milijana, Pantelic Vladimir, Matic Irena, Hrncic Dragan
Metanephric adenoma (MA) is a rare neoplasm that acounts for 0.2% of adult renal neoplasms. MAs are typically discover incidentally during detailed examinations for nonspecific symptoms such as abdominal or flank pain, hematuria, fever and palpable abdominal mass. Additionally, polycythemia has occasionally been reported as well. Herein we describe a case of metanephric adenoma which was an incidental finding in the course of a clinical autopsy in a patient with complete AV block and polycythemia. Histologically, the tumor was composed of small and uniform tubular structures reminiscent of renal tubuli, without signs of cellular atypia and pleomorphism...
July 2016: Acta Cardiol Sin
Xue-Lian Gong, Xiao-Lei Gu, Yong-Chun Chen, Hai Zhu, Zhen-Na Xia, Jian-Zhong Li, Guo-Cai Lu
EPO-018B, a synthetic peptide-based erythropoiesis stimulating agent (ESA), is mainly designed for treatment of anemia caused by chronic renal failure and chemotherapy against cancer. It overcomes the deficiencies of currently approved ESA, including the frequent administration of temperature-sensitive recombinant protein and anti-EPO antibody-mediated pure red cell aplasia (PRCA). This study was designed to evaluate the potential chronic toxicity of EPO-018B. Subcutaneous administration doses were designed as 0, 0...
September 15, 2016: Toxicology and Applied Pharmacology
Suvro Sankha Datta, Somnath Mukherjee, Biplabendu Talukder, Prasun Bhattacharya
Therapeutic plasma exchange (TPE) is a conjunctive modality of treatment along with rituximab to decrease paraproteinemia associated with hyperviscosity. Here we narrate our experience in treating a diagnosed case of Waldenstrom's macroglobulinemia in 70 years old male patient with moderate anemia and severe features of hyperviscosity syndrome by serial TPE and rituximab combined with bortezomib. The patient was relieved of his symptoms after initial two TPE procedures performed on alternative day. However he again developed signs and symptoms of the disease within 6 weeks following second TPE and starting of rituximab (375 mg/m(2) weekly for 4 weeks) therapy with bortezomib...
June 2016: Indian Journal of Hematology & Blood Transfusion
Pawani Sachar, Kumar Rajamani
BACKGROUND: Ovarian hyperstimulation syndrome (OHSS) is a serious and potentially fatal complication of medical ovarian stimulation. Uncommonly, it is associated with thromboembolic complications with venous thrombosis being more common than arterial thromboembolic events. We present a case of cerebral infarction in the setting of severe OHSS secondary to in vitro fertilization treatment with no residual neurological deficits. MATERIALS AND METHODS: We also performed a review of previously published ischemic cerebral infarction and cerebral venous sinus thrombosis (CVST) cases associated with OHSS to evaluate common patterns in presentations, commonly affected central nervous system sites, trends for therapeutic options in these cases, and outcomes...
September 2016: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
Jorge J Castillo, Ramon Garcia-Sanz, Evdoxia Hatjiharissi, Robert A Kyle, Xavier Leleu, Mary McMaster, Giampaolo Merlini, Monique C Minnema, Enrica Morra, Roger G Owen, Stephanie Poulain, Marvin J Stone, Constantine Tam, Marzia Varettoni, Meletios A Dimopoulos, Steven P Treon, Efstathios Kastritis
The diagnosis of Waldenström macroglobulinaemia (WM) can be challenging given the variety of signs and symptoms patients can present. Furthermore, once the diagnosis of WM is established, the initial evaluation should be thorough as well as appropriately directed. During the 8th International Workshop for WM in London, United Kingdom, a multi-institutional task force was formed to develop consensus recommendations for the diagnosis and initial evaluation of patients with WM. In this document, we present the results of the deliberations that took place to address these issues...
October 2016: British Journal of Haematology
Rebecca L King, Wilson I Gonsalves, Stephen M Ansell, Patricia T Greipp, Lori A Frederick, David S Viswanatha, Rong He, Robert A Kyle, Morie A Gertz, Prashant Kapoor, William G Morice, Matthew T Howard
OBJECTIVES: Lymphoplasmacytic lymphoma (LPL) with non-immunoglobulin M (IgM) paraproteinemia remains poorly understood. The goal of this study was to investigate the clinicopathologic features of LPL in the bone marrow in patients with immunoglobulin G (IgG) or immunoglobulin A (IgA) paraproteins and evaluate MYD88 L265P mutation status to determine the relationship of these cases to Waldenström macroglobulinemia (WM). METHODS: Bone marrows from LPL cases with IgG or IgA paraproteins diagnosed between January 1, 2007, and June 30, 2014, were retrieved from the clinical archive...
June 2016: American Journal of Clinical Pathology
Ceyhun Varım, Hasan Ergenc, Mehmet Sevki Uyanık, Tezcan Kaya, Ahmet Nalbant, Cengiz Karacaer, Cenk Sunu, Ali Tamer
Multiple myeloma (MM), the second most common hematological malignancy, is caused by the accumulation of monoclonal plasma cells in bone marrow. It accounts for 10-15% of deaths from hematological malignancies and approximately 2% of deaths from cancer. The median age at presentation is 70 years old. The diagnosis is incidental in 30% of cases. MM is often discovered through routine blood screening with a large gap between the total protein and the albumin levels. Two thirds of patients complain of bone pain, especially lower back pain...
September 15, 2015: Open Access Macedonian Journal of Medical Sciences
Michael J Simmonds, Surendran Sabapathy, Kevin R Serre, Luke J Haseler, Gregory C Gass, Sonya M Marshall-Gradisnik, Clare L Minahan
BACKGROUND: The purpose of the present study was to investigate the effects of regular treadmill walking on plasma factors that increase low-shear blood viscosity and red blood cell aggregation in older women with type 2 diabetes. METHODS: Eighteen women with type 2 diabetes (age: 69±3 yr; body mass index: 30.5±5.0 kg⋅m-2) performed 12-wk of 120 min⋅wk-1 of supervised treadmill walking at an intensity equivalent to the gas-exchange threshold. Peak exercise values, anthropometry and blood indices of diabetic status, markers of inflammation, and plasma fibrinogen were analysed during a 6-wk pre-training 'control' period, and then after 6 and 12-wk of regular walking...
May 30, 2016: Clinical Hemorheology and Microcirculation
Elizabeth Turner
Management of a right-to-left ("reversed") patent ductus arteriosus (PDA) focuses on control of clinical signs associated with hyperviscosity due to erythrocytosis. Pentoxifylline therapy is presented as an adjunct to routine phlebotomies for the long-term clinical management of reversed PDA in a 10-year-old Chihuahua.
June 2016: Canadian Veterinary Journal. la Revue Vétérinaire Canadienne
Christine H Yang, Saurabh Gombar, Clare J Twist, Dita Gratzinger, Carlos O Esquivel, Audrey H Lau
No abstract text is available yet for this article.
May 26, 2016: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
Siba Prasad Dalai, Lalit Kumar Meher, Samir Kumar Behera, Sachidananda Nayak, Sujit Kumar Tripathy
Cryoglobulins are monoclonal or polyclonal immunoglobulins that undergo reversible precipitation at low temperatures. Cryoglobulinemia is associated with HCV infection in more than 90% cases, the remaining 10% being called as Essential Cryoglobulinemia which is generally associated with a severe course and suboptimal response to conventional therapies. As the digital vessels are more prone to colder temperatures, hyperviscosity in those vessels can initiate local thrombosis and may manifest as ischemic ulceration and gangrene...
April 2016: Journal of Clinical and Diagnostic Research: JCDR
Daniel Halperin, Simon Hallam, Athar Haroon, Tom Butler, Samir Agrawal
Waldenstrom's macroglobulinaemia is the most commonly reported subtype of lymphoplasmacytic lymphoma (LPL); it is characterised by IgM secretion. Neurological complications are common usually as a result of hyperviscosity. In rare cases, cells can infiltrate the central nervous system; this is known as Bing-Neel syndrome. We report the case of a 57-year-old male with lymphoplasmacytic lymphoma of the IgG-subtype with neurological symptoms and the consequent finding of lymphoplasmacytoid cells in his cerebrospinal fluid as well as deposits on MRI and PET-CT imaging...
2016: Case Reports in Hematology
Christopher J Borgman
Multiple myeloma is a neoplastic plasma-cell disorder resulting from malignant plasma cells in the bone marrow. It can cause a hyperviscosity syndrome secondary to the paraproteinaemia associated with the disease. The increased hyperviscosity can lead to retinal vein occlusions and other ocular problems that may challenge clinicians. In patients with multiple myeloma and hypertension and/or diabetes mellitus, retinal changes appear similar and changes due to one disease or the other may be difficult to determine...
July 2016: Clinical & Experimental Optometry: Journal of the Australian Optometrical Association
Sadia Sultan, Syed Mohammed Irfan, Sania Murad
BACKGROUND: Polycythaemia rubra vera (PV) is a Philadelphia chromosome negative myeloproliferative neoplasm characterized by increased red cell production, independent of the mechanisms that regulate normal erythropoiesis. The aim of this study was to analyze the clinico-epidemiological profile of Pakistani patients with PV. MATERIALS AND METHODS: In this retrospective cross sectional study, 26 patients with PV were enrolled from January 2010 to December 2014. They were diagnosed based on WHO criteria...
2016: Asian Pacific Journal of Cancer Prevention: APJCP
Emanuele Cecchi, Guido Parodi, Serena Fatucchi, Paola Angelotti, Cristina Giglioli, Anna Maria Gori, Brunella Bandinelli, Benedetta Bellandi, Elena Sticchi, Ilaria Romagnuolo, Lucia Mannini, David Antoniucci, Rosanna Abbate
Takotsubo cardiomyopathy (TTC) pathophysiology is still unclear. A transient intracoronary thrombosis dissolved at the time of angiography has been hypothesized. We aimed to evaluate the prevalence of thrombophilic disorders in TTC patients. In 75 TTC women, 75 age- and sex-matched acute coronary syndrome (ACS) patients, both enrolled during the acute phase, and in 75 control subjects, we compared the prevalence of congenital and acquired thrombophilic alterations and the values of clotting and endothelial activation biomarkers...
September 2016: Clinical Research in Cardiology: Official Journal of the German Cardiac Society
Zdeněk Adam, Luděk Pour, Marta Krejčí, Sabina Ševčíková, Eva Pourová, Eva Ševčíková, Zdeněk Král, Jiří Mayer
Waldenström macroglobulinemia is defined by the presence of monoclonal immunoglobulin IgM type (M-IgM) and evidence of lymphoplasmacytic bone marrow infiltration. The disease has an indolent course, the treatment is only initiated when the disease has begun to damage its carrier. The following symptoms are regarded as proven indications for initiating therapy: B symptoms, symptomatic lymphadenopathy, splenomegaly, anemia with hemoglobin below 100 g / l or thrombocytopenia < 100 × 10(9)/l, caused by lymphoplasmacytic bone marrow infiltration...
January 2016: Vnitr̆ní Lékar̆ství
Prashant Kapoor, Jonas Paludo, Stephen M Ansell
Waldenstrom macroglobulinemia (WM) is a non-Hodgkin lymphoma (NHL) characterized by the presence of a CD20 + lymphoplasmacytic bone marrow (BM) infiltrate and serum immunoglobulin M monoclonal protein. Both sporadic and familial forms exist. A remarkable improvement in outcome of nearly all age groups of WM patients may be primarily a consequence of successful integration of anti-CD20 monoclonal antibody, rituximab, to the conventional chemotherapy. However, the seminal discoveries of MYD88 (L265P) mutation, present in the vast majority (85-100 %), and CXCR4 (WHIM) mutations, identified in nearly a third of patients (who almost exclusively harbor the MYD88 (L265P) variant), have laid a solid foundation for a paradigm shift in our diagnostic and therapeutic approaches towards this rare hematologic malignancy...
March 2016: Current Treatment Options in Oncology
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