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plasma cells disorders guidelines

Uday Yanamandra, Navin Khattry, Shaji Kumar, Noopur Raje, Arihant Jain, Sundar Jagannath, Hari Menon, Lalit Kumar, Neelam Varma, Subhash Varma, Tapan Saikia, Pankaj Malhotra
The science of multiple myeloma (MM) and related plasma cell disorders is rapidly evolving with increased understanding of the disease biology and recent approval of the newer drugs widening the therapeutic armamentarium. Despite multiple international guidelines regarding the management of this disease, the practice of managing MM is not uniform amongst Indian physicians. There are challenges in management which are unique to the Indian patients. This review discusses these challenges and the consensus of the nation-wide experts in dealing with the same...
March 2017: Indian Journal of Hematology & Blood Transfusion
Eric B Hamill, Preeti J Thyparampil, Michael T Yen
Current guidelines recommend a thorough systemic workup in patients with newly diagnosed immunoglobulin amyloid light chain (AL) amyloidosis because of a close association with an underlying plasma cell proliferative disorder. Rarely, however, AL amyloidosis may present as a localized, benign disease without systemic involvement. Few previous reports have documented localized AL amyloid of the conjunctiva, and all these used immunohistochemistry for amyloid typing, which can be inaccurate. The authors identified 2 cases of AL amyloidosis confirmed by mass spectrometry localized to the conjunctiva and without evidence of systemic disease as determined by thorough systemic workup...
November 15, 2016: Ophthalmic Plastic and Reconstructive Surgery
Krystal Bergin, Elizabeth Moore, Zoe McQuilten, Erica Wood, Bradley Augustson, Hilary Blacklock, Joy Ho, Noemi Horvath, Tracy King, John McNeil, Peter Mollee, Hang Quach, Christopher M Reid, Brian Rosengarten, Patricia Walker, Andrew Spencer
BACKGROUND: Plasma cell dyscrasias (PCD) are a spectrum of disorders resulting from the clonal expansion of plasma cells, ranging from the pre-malignant condition monoclonal gammopathy of undetermined significance (MGUS) to multiple myeloma (MM). MM generates a significant burden of disease on the community and it is predicted that it will increase in both incidence and prevalence owing to an ageing population and longer survival secondary to new therapeutic options. Robust and comprehensive clinical data are currently lacking but are required to define current diagnostic, investigational and management patterns in Australia and New Zealand (ANZ) for comparison to both local and international guidelines for standards of care...
November 9, 2016: BMC Medical Research Methodology
Walter H Reinhart
The hematocrit (Hct) determines the oxygen carrying capacity of blood, but also increases blood viscosity and thus flow resistance. From this dual role the concept of an optimum Hct for tissue oxygenation has been derived. Viscometric studies using the ratio Hct/blood viscosity at high shear rate showed an optimum Hct of 50-60% for red blood cell (RBC) suspensions in plasma. For the perfusion of an artificial microvascular network with 5-70μm channels the optimum Hct was 60-70% for high driving pressures. With lower shear rates or driving pressures the optimum Hct shifted towards lower values...
2016: Clinical Hemorheology and Microcirculation
Zhiming Zhu
Management of hypertension in diabetes is critical for reducing cardiovascular mortality and morbidity. Dietary approaches for controlling high blood pressure have historically focused on sodium. Thus, many guidelines recommend that patients with type 2 diabetes reduce high sodium intake. Nonetheless, the potential benefits of sodium reduction are debatable. The kidney has a crucial role in glucose filtration and reabsorption in addition to its regulation of fluid and electrolyte homeostasis. A key factor linking sodium uptake and glucose transport is the sodium-glucose cotransporter 2 (SGLT2) in renal proximal tubular cells...
September 2016: Journal of Hypertension
P Pardes-Chavanes, M Afanetti, C Boyer, M Poirée
Intracerebral hemorrhage (ICH) remains a cause of death in hematologic malignancies. Asparaginase represents a key agent in the treatment of acute lymphoblastic leukemia (ALL). The toxicity of asparaginase includes coagulopathy such as thrombotic or bleeding tendency. We report a case of fatal cerebral hemorrhage in a 12-year-old girl treated for ALL. Cerebral hemorrhage occurred after three injections of L-asparaginase. The patient presented with hypofibrinogenemia (0.36g/L), associated with thrombocytopenia (24,000/mm(3))...
December 2016: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
Huy P Pham, Joseph Schwartz
PURPOSE OF REVIEW: Therapeutic apheresis can be used to treat many diseases. The American Society for Apheresis (ASFA) publishes Guidelines on the use of therapeutic apheresis every 3 years with the goal of providing the best available evidence for apheresis practice as well as clinical expertise. The 2016 (7th ed.) ASFA Guidelines contain 87 diseases (up from 78 in the 6th ed.) and 179 indications. This review outlines three new therapeutic apheresis indications for hematological disorders...
November 2016: Current Opinion in Hematology
Martha Grogan, Morie Gertz, Arleigh McCurdy, Lindsey Roeker, Robert Kyle, Sudhir Kushwaha, Richard Daly, Joseph Dearani, Richard Rodeheffer, Robert Frantz, Martha Lacy, Suzanne Hayman, Christopher McGregor, Brooks Edwards, Angela Dispenzieri
AIM: To determine the outcome of orthotopic heart transplantation (OHT) in immunoglobulin light chain (AL) amyloidosis. METHODS: The medical records of patients with AL who underwent orthotopic heart transplantation at the Mayo Clinic in Rochester Minnesota from 1992 to 2011 were reviewed. Patients met at least one of the following at: New York Heart Association class IV heart failure, ventricular thickness > 15 mm, ejection fraction < 40%. Selection guidelines for heart transplant included age < 60 years, absence of multiple myeloma and significant extra-cardiac organ involvement...
June 24, 2016: World Journal of Transplantation
Marleen H van Coevorden-Hameete, Maarten J Titulaer, Marco W J Schreurs, Esther de Graaff, Peter A E Sillevis Smitt, Casper C Hoogenraad
Autoimmune encephalitis (AIE) is a group of disorders in which autoantibodies directed at antigens located on the plasma membrane of neurons induce severe neurological symptoms. In contrast to classical paraneoplastic disorders, AIE patients respond well to immunotherapy. The detection of neuronal surface autoantibodies in patients' serum or CSF therefore has serious consequences for the patients' treatment and follow-up and requires the availability of sensitive and specific diagnostic tests. This mini-review provides a guideline for both diagnostic and research laboratories that work on the detection of known surface autoantibodies and/or the identification of novel surface antigens...
2016: Frontiers in Molecular Neuroscience
Saskia Tamminga, Merel van Maarle, Lidewij Henneman, Cees B M Oudejans, Martina C Cornel, Erik A Sistermans
Cell-free DNA (cfDNA) testing has recently become indispensable in diagnostic testing and screening. In the prenatal setting, this type of testing is often called noninvasive prenatal testing (NIPT). With a number of techniques, using either next-generation sequencing or single nucleotide polymorphism-based approaches, fetal cfDNA in maternal plasma can be analyzed to screen for rhesus D genotype, common chromosomal aneuploidies, and increasingly for testing other conditions, including monogenic disorders. With regard to screening for common aneuploidies, challenges arise when implementing NIPT in current prenatal settings...
2016: Advances in Clinical Chemistry
Rohit K Mishra, Vani Mishra, Anand Pandey, Amit K Tiwari, Himanshu Pandey, Shivesh Sharma, Avinash C Pandey, Anupam Dikshit
BACKGROUND: Malassezia commensal yeasts along with multitude of antigens have been found to be associated with various skin disorders including Pityriasis versicolor (PV). Amongst them Mala s1, a 37 kDa protein has been proved to be a major allergen reacting with a large panel of sera. However, there exists no therapeutic alternative to combat such problems in form of plant based natural compounds. The purpose of this study is in the first place, to determine the anti-Malassezia activity of Nyctanthes arbor-tristis L...
March 31, 2016: BMC Complementary and Alternative Medicine
Ellen Brisse, Maya Imbrechts, Karen Put, Anneleen Avau, Tania Mitera, Nele Berghmans, Omer Rutgeerts, Mark Waer, Marisa Ninivaggi, Hilde Kelchtermans, Louis Boon, Robert Snoeck, Carine H Wouters, Graciela Andrei, Patrick Matthys
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening immunological disorder that is characterized by systemic inflammation, widespread organ damage, and hypercytokinemia. Primary HLH is caused by mutations in granule-mediated cytotoxicity, whereas secondary HLH occurs, without a known genetic background, in a context of infections, malignancies, or autoimmune and autoinflammatory disorders. Clinical manifestations of both HLH subtypes are often precipitated by a viral infection, predominantly with Herpesviridae...
April 1, 2016: Journal of Immunology: Official Journal of the American Association of Immunologists
Srinath Sundararajan, Abhijeet Kumar, Neha Korde, Amit Agarwal
Smoldering multiple myeloma (SMM) is a pre-malignant condition with an inherent risk for progression to multiple myeloma (MM). The 2014 IMWG guidelines define smoldering multiple myeloma as a monoclonal gammopathy disorder with serum monoclonal protein (IgG or IgA) ≥30 g/L or urinary monoclonal protein ≥500 mg per 24 h and/or clonal bone marrow plasma cells 10-60 % without any myeloma-defining events or amyloidosis. The risk for progression of SMM to MM vary based on clinical, laboratory, imaging, and molecular characteristics...
April 2016: Current Hematologic Malignancy Reports
Joannes F M Jacobs, Jillian R Tate, Giampaolo Merlini
The serum free light chain (FLC) assay has proven to be an important complementary test in the management of patients with monoclonal gammopathies. The serum FLC assay has value for patients with plasma cell disorders in the context of screening and diagnosis, prognostic stratification, and quantitative monitoring. Nonetheless, serum FLC measurements have analytical limitations which give rise to differences in FLC reporting depending on which FLC assay and analytical platform is used. As the FLC measurements are incorporated in the International Myeloma Working Group guidelines for the evaluation and management of plasma cell dyscrasias, this may directly affect clinical decisions...
June 1, 2016: Clinical Chemistry and Laboratory Medicine: CCLM
Michael J R Desborough, Brian Hockley, Mallika Sekhar, Andrew K Burroughs, Simon J Stanworth, Vipul Jairath
BACKGROUND & AIMS: Cirrhosis is a complex acquired disorder of coagulation and frequent indication for transfusion of blood components. We characterised blood component use in patients with cirrhosis and compared this to transfusion guidelines. METHODS: All National Health Service trusts with representation on the British Society of Gastroenterology membership list were invited to take part. Data were collected prospectively on consecutive, unselected, hospitalised admissions with cirrhosis over 28 days...
April 2016: Liver International: Official Journal of the International Association for the Study of the Liver
Prabhat Khakural, Ranjan Sapkota, Uttam K Shrestha, Prakash Sayami
Inflammatory myofibroblastic tumour (IMT) is an uncommon mesenchymal tumour, which can occur anywhere in the body, rarely in esophagus. Mostly, the diagnosis is postoperative, after the hispathological evaluation of the specimen. There are no definite guidelines regarding the diagnosis and management. Here, we report a 60 year old lady presenting with dysphagia, diagnosed to have a submucosal esophageal tumor with Barium esophagogram and contrast enhanced computed tomography. She was managed successfully with surgical enucleation with the final histopathological diagnosis of IMT...
2015: Journal of Cardiothoracic Surgery
Massimiliano Pino, Filippo Farri, Pietro Garofalo, Fausto Taranto, Andrea Toso, Paolo Aluffi
Extramedullary plasmacytoma (EMP) is a rare variant of plasma cell myeloma that affects soft tissues. The head and neck region are the most affected sites, although others have also been described. Herein we report an uncommon case of EMP of the larynx in a 65-year-old male who presented with a history of progressive dysphonia and hoarseness. Laryngeal fiberscopy evidenced a reddish pedicled voluminous mass in the left false cords and ventricle. Microscopic suspension laryngoscopy was performed under general anaesthesia and a 4 W Acublade CO2 Laser was used for transoral resection of the lesion...
2015: Case Reports in Otolaryngology
A Driessen, N Schäfer, V Albrecht, M Schenk, M Fröhlich, E K Stürmer, M Maegele
PURPOSE: Early detection and management of post-traumatic haemorrhage and coagulopathy have been associated with improved outcomes, but local infrastructures, logistics and clinical strategies may differ. METHODS: To assess local differences in infrastructure, logistics and clinical management of trauma-associated haemorrhage and coagulopathy, we have conducted a web-based survey amongst the delegates to the 15th European Congress of Trauma and Emergency Surgery (ECTES) and the 2nd World Trauma (WT) Congress held in Frankfurt, Germany, 25-27 May 2014...
August 2015: European Journal of Trauma and Emergency Surgery: Official Publication of the European Trauma Society
John R Mills, David R Barnidge, David L Murray
Established guidelines from the International Myeloma Working Group recommend diagnostic screening for patients suspected of plasma cell proliferative disease using protein electrophoresis (PEL), free light chain measurements and immunofixation electrophoresis (IFE) of serum and urine in certain cases. Plasma cell proliferative disorders are generally classified as monoclonal gammopathies given most are associated with the excess secretion of a monoclonal immunoglobulin or M-protein. In clinical practice, the M-protein is detected in a patients' serum by the appearance of a distinct protein band migrating within regions typically occupied by immunoglobulins...
June 15, 2015: Methods: a Companion to Methods in Enzymology
Jie Ding, Fei Huang, Gaoyi Wu, Tao Han, Fuqiang Xu, Dan Weng, Chengli Wu, Xiaodong Zhang, Yuanqing Yao, Xiaoming Zhu
Our study was approved by the Medical Ethics Committee of Tang Du Hospital, Fourth Military Medical University and complied strictly with national ethical guidelines. Preeclampsia (PE) is a specific clinical disorder characterized by gestational hypertension and proteinuria and is a leading cause of maternal and perinatal mortality worldwide. The miR-519d-3p is upregulated in the maternal plasma of patients with PE which indicates a possible association between this microRNA and the pathogenesis of PE. No studies to date have addressed the effect of miR-519d-3p on the invasion and migration of trophoblast cells...
2015: PloS One
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