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Hematology

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9 papers 0 to 25 followers
By Faye Kehler Family Physician and GP Anesthetist since 1987 interested in all aspects of Medicine
https://www.readbyqxmd.com/read/27527836/immunoglobulin-light-chain-amyloidosis-2016-update-on-diagnosis-prognosis-and-treatment
#1
Morie A Gertz
DISEASE OVERVIEW: Immunoglobulin light chain amyloidosis is a clonal, nonproliferative plasma cell disorder in which fragments of immunoglobulin light chain are deposited in tissues. Clinical features depend on organs involved but can include restrictive cardiomyopathy, nephrotic syndrome, hepatic failure, peripheral/autonomic neuropathy, and atypical multiple myeloma. DIAGNOSIS: Tissue biopsy stained with Congo red demonstrating amyloid deposits with applegreen birefringence is required for diagnosis...
September 2016: American Journal of Hematology
https://www.readbyqxmd.com/read/26408108/how-we-diagnose-and-treat-iron-deficiency-anemia
#2
REVIEW
Michael Auerbach, John W Adamson
It is estimated that one-third of the world's population is anemic, the majority being due to iron deficiency (ID). In adults, ID is associated with fatigue in the absence of anemia, restless legs syndrome, pica and, in neonates, delayed growth and development. In adolescents, ID is associated with decrements in learning and behavioral abnormalities. In the absence of a clear cause, search for a source of bleeding is indicated. No single test is diagnostic of ID unless the serum ferritin is low or the percent transferrin saturation is low with an elevated total iron binding capacity...
January 2016: American Journal of Hematology
https://www.readbyqxmd.com/read/27296954/the-effect-of-bone-marrow-plasma-cell-burden-on-survival-in-patients-with-light-chain-amyloidosis-undergoing-high-dose-melphalan-and-autologous-stem-cell-transplantation
#3
Christopher Dittus, Nsabimana Uwumugambi, Fangui Sun, J Mark Sloan, Vaishali Sanchorawala
The prognosis in light chain (AL) amyloidosis has been linked to several variables, which are primarily related to end-organ damage. Recently, bone marrow plasma cell (BMPC) burden >10% has also been described as an adverse prognostic factor. We reviewed data pertaining to 546 patients with AL amyloidosis who underwent high-dose melphalan (HDM) and stem cell transplantation (SCT) to determine if BMPC > 10% was a negative prognostic factor. Of these patients, 445 had a BMPC burden ≤ 10% and 101 had a BMPC burden > 10%...
September 2016: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/26819490/clinical-applications-of-hemolytic-markers-in-the-differential-diagnosis-and-management-of-hemolytic-anemia
#4
REVIEW
W Barcellini, B Fattizzo
Several hemolytic markers are available to guide the differential diagnosis and to monitor treatment of hemolytic conditions. They include increased reticulocytes, an indicator of marrow compensatory response, elevated lactate dehydrogenase, a marker of intravascular hemolysis, reduced haptoglobin, and unconjugated hyperbilirubinemia. The direct antiglobulin test is the cornerstone of autoimmune forms, and blood smear examination is fundamental in the diagnosis of congenital membrane defects and thrombotic microangiopathies...
2015: Disease Markers
https://www.readbyqxmd.com/read/26769228/myelodysplastic-syndromes-contemporary-review-and-how-we-treat
#5
REVIEW
Naseema Gangat, Mrinal M Patnaik, Ayalew Tefferi
Myelodysplastic syndromes (MDS) are a heterogeneous group of clonal stem cell disorders with an inherent tendency for leukemic transformation. Diagnosis is currently based on the presence of peripheral blood cytopenias, peripheral blood and bone marrow dysplasia/blasts, and clonal cytogenetic abnormalities. With the advent of next generation sequencing, recurrent somatic mutations in genes involved in epigenetic regulation (TET2, ASXL1, EZH2, DNMT3A, IDH1/2), RNA splicing (SF3B1, SRSF2, U2AF1, ZRSR2), DNA damage response (TP53), transcriptional regulation (RUNX1, BCOR, ETV6) and signal transduction (CBL, NRAS, JAK2) have been identified in MDS...
January 2016: American Journal of Hematology
https://www.readbyqxmd.com/read/26294090/myelodysplastic-syndromes-2015-update-on-diagnosis-risk-stratification-and-management
#6
REVIEW
Guillermo Garcia-Manero
DISEASE OVERVIEW: The myelodysplastic syndromes (MDS) are a very heterogeneous group of myeloid disorders characterized by peripheral blood cytopenias and increased risk of transformation to acute myelogenous leukemia (AML). MDS occurs more frequently in older males and in individuals with prior exposure to cytotoxic therapy. DIAGNOSIS: Diagnosis of MDS is based on morphological evidence of dysplasia upon visual examination of a bone marrow aspirate and biopsy. Information obtained from additional studies such as karyotype, flow cytometry, or molecular genetics is complementary but not diagnostic...
September 2015: American Journal of Hematology
https://www.readbyqxmd.com/read/26732417/monoclonal-gammopathy-the-good-the-bad-and-the-ugly
#7
REVIEW
Siobhan V Glavey, Nelson Leung
Monoclonal gammopathy of undetermined significance (MGUS) is a condition characterized by the presence of a monoclonal gammopathy (MG) in which the clonal mass has not reached a predefined state in which the condition is considered malignant. It is a precursor to conditions such as multiple myeloma or lymphoma at a rate of ~1%/year. Thus, from a hematologic standpoint, MGUS is a fairly benign condition. However, it is now recognized that organ damage resulting from just the MG without the need MM or lymphoma can occur...
May 2016: Blood Reviews
https://www.readbyqxmd.com/read/26728842/myelodysplastic-syndrome
#8
Richard A Wells, Rena Buckstein, Jeremy Rezmovitz
No abstract text is available yet for this article.
July 12, 2016: CMAJ: Canadian Medical Association Journal, Journal de L'Association Medicale Canadienne
https://www.readbyqxmd.com/read/26499177/current-management-of-primary-immune-thrombocytopenia
#9
REVIEW
Drew Provan, Adrian C Newland
Primary immune thrombocytopenia is an autoimmune disorder of unknown cause affecting both children and adults. The low peripheral blood platelet count is caused by premature platelet destruction by self-reacting antibodies in addition to an impairment of platelet production. The disease is heterogeneous in its pathophysiology, clinical features and responses to treatment. To date, most of the treatments used have been immune-modulating drugs and these contribute to increased morbidity and mortality in patients...
October 2015: Advances in Therapy
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