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hemolytic anemia guidelines

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https://www.readbyqxmd.com/read/28680366/fatal-pulmonary-embolism-following-splenectomy-in-a-patient-with-evan-s-syndrome-case-report-and-review-of-the-literature
#1
Varun Monga, Seth M Maliske, Usha Perepu
BACKGROUND: Evans syndrome (ES) is a rare disease characterized by simultaneous or sequential development of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP) with or without immune neutropenia. Splenectomy is one of the treatment options for disease refractory to medical therapy. Venous thromboembolism (VTE) following splenectomy for hematological diseases has an incidence of 10%. CASE PRESENTATION: Here we describe a case report of a young patient hospitalized with severe hemolytic anemia with Hgb 4...
2017: Thrombosis Journal
https://www.readbyqxmd.com/read/28626212/a-huge-subcutaneous-hematoma-in-an-adult-with-kasabach-merritt-syndrome
#2
Kuan-Lin Wu, Chiung-Ying Liao, Chen-Kuang Chang, Shang-Yun Ho, Yeu-Sheng Tyan, Yuan-Chun Huang
BACKGROUND Kasabach-Merritt syndrome is a potentially fatal disease that consists of hemangioma(s) with thrombocytopenia, microangiopathic hemolytic anemia, and coagulopathy. Extensive hemangiomatosis is rare. We present the radiological features and treatment strategy of a young adult suffering from Kasabach-Merritt syndrome with widespread hemangiomas and an infected huge hematoma in the right thigh. CASE REPORT A 33-year-old Taiwanese male presented with a painful 20-cm mass over his right thigh and gross hematuria for 2 days...
June 19, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28550351/diagnostic-and-treatment-guidelines-for-thrombotic-thrombocytopenic-purpura-ttp-2017-in-japan
#3
Masanori Matsumoto, Yoshihiro Fujimura, Hideo Wada, Koichi Kokame, Yoshitaka Miyakawa, Yasunori Ueda, Satoshi Higasa, Takanori Moriki, Hideo Yagi, Toshiyuki Miyata, Mitsuru Murata
Thrombotic thrombocytopenic purpura (TTP) can rapidly progress into a life-threatening condition, thus the importance of appropriate diagnosis and treatment cannot be overstated. Until recently, TTP has mainly been diagnosed by clinical findings such as thrombocytopenia and non-immune hemolytic anemia. In addition to these clinical findings, however, reduced activity of a disintegrin-like and metalloprotease with thrombospondin type 1 motif 13 (ADAMTS13) below 10% has been accepted internationally as a diagnostic criterion for TTP...
July 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28550188/recommendations-for-splenectomy-in-hereditary-hemolytic-anemias
#4
Achille Iolascon, Immacolata Andolfo, Wilma Barcellini, Francesco Corcione, Loïc Garçon, Lucia De Franceschi, Claudio Pignata, Giovanna Graziadei, Dagmar Pospisilova, David C Rees, Mariane de Montalembert, Stefano Rivella, Antonella Gambale, Roberta Russo, Leticia Ribeiro, Jules Vives-Corrons, Patricia Aguilar-Martinez, Antonis Kattamis, Beatrice Gulbis, Maria Domenica Cappellini, Irene Roberts, Hannah Tamary
Hereditary hemolytic anemias are a group of disorders including red cell membrane defects, red blood cells enzyme disorders, congenital dyserythropoietic anemias, thalassemia syndromes and haemoglobinopathies. As damaged red blood cells passing through the spleen red pulp are efficiently removed by splenic macrophages, splenectomy is one possible therapeutic approach to the management of severely affected patients. However, except for hereditary spherocytosis for which the effectiveness of splenectomy has been well documented, the efficacy of splenectomy in other anemias within this group has yet to be determined and there are concerns regarding short- and long-term infectious and thrombotic complications...
May 26, 2017: Haematologica
https://www.readbyqxmd.com/read/28503958/neonatal-management-and-outcome-in-alloimmune-hemolytic-disease
#5
Isabelle M C Ree, Vivianne E H J Smits-Wintjens, Johanna G van der Bom, Jeanine M M van Klink, Dick Oepkes, Enrico Lopriore
Hemolytic disease of the fetus and newborn (HDFN) occurs when fetal and neonatal erythroid cells are destroyed by maternal erythrocyte alloantibodies, it leads to anemia and hydrops in the fetus, and hyperbilirubinemia and kernicterus in the newborn. Postnatal care consists of intensive phototherapy and exchange transfusions to treat severe hyperbilirubinemia and top-up transfusions to treat early and late anemia. Other postnatal complications have been reported such as thrombocytopenia, iron overload and cholestasis requiring specific management...
June 5, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28406545/diagnosing-nocturnal-paroxysmal-hemoglobinuria-a-single-center-4-year-experience
#6
T Mercier, T Devos, M Mukovnikova, N Boeckx
INTRODUCTION: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disease and can present as a wide range of signs and symptoms. As such, the indication for diagnostic testing for PNH is not always straightforward. Therefore, we analyzed all first-time samples tested over a 56-month period to determine the clinical settings with a high probability of detecting a PNH clone. METHODS: We retrospectively analyzed 323 first-time PNH flow cytometry tests, including LDH, cytopenias, direct antiglobulin test (DAT), and clinical indication for testing as available at the time of testing...
June 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/27777394/systemic-lupus-erythematosus-presenting-as-refractory-thrombotic-thrombocytopenic-purpura-a-diagnostic-and-management-challenge-a-case-report-and-concise-review-of-the-literature
#7
REVIEW
Mohammad Abu-Hishmeh, Alamgir Sattar, Fnu Zarlasht, Mohamed Ramadan, Aisha Abdel-Rahman, Shante Hinson, Caroline Hwang
BACKGROUND Thrombotic thrombocytopenic purpura (TTP) is one of the thrombotic microangiopathic (TMA) syndromes, caused by severely reduced activity of the vWF-cleaving protease ADAMTS13. Systemic lupus erythematosus (SLE), on the other hand, is an autoimmune disease that affects various organs in the body, including the hematopoietic system. SLE can present with TMA, and differentiating between SLE and TTP in those cases can be very challenging, particularly in patients with no prior history of SLE. Furthermore, an association between these 2 diseases has been described in the literature, with most of the TTP cases occurring after the diagnosis of SLE...
October 25, 2016: American Journal of Case Reports
https://www.readbyqxmd.com/read/27550478/clinical-practice-guidelines-for-the-management-of-atypical-hemolytic-uremic-syndrome-in-korea
#8
Hae Il Cheong, Sang Kyung Jo, Sung Soo Yoon, Heeyeon Cho, Jin Seok Kim, Young Ok Kim, Ja Ryong Koo, Yong Park, Young Seo Park, Jae Il Shin, Kee Hwan Yoo, Doyeun Oh
Atypical hemolytic uremic syndrome (aHUS) is a rare syndrome characterized by micro-angiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. The major pathogenesis of aHUS involves dysregulation of the complement system. Eculizumab, which blocks complement C5 activation, has recently been proven as an effective agent. Delayed diagnosis and treatment of aHUS can cause death or end-stage renal disease. Therefore, a diagnosis that differentiates aHUS from other forms of thrombotic microangiopathy is very important for appropriate management...
October 2016: Journal of Korean Medical Science
https://www.readbyqxmd.com/read/27397673/hemolytic-disease-of-the-fetus-and-newborn-modern-practice-and-future-investigations
#9
REVIEW
Jeanne E Hendrickson, Meghan Delaney
Red blood cell (RBC) sensitization occurs in some women in response to exposure to paternally derived RBC antigens during pregnancy or to nonself antigens on transfused RBCs during their lifetime. Once sensitized, future pregnancies may be at risk for hemolytic disease of the fetus and newborn. Although great strides have been made over the past few decades in terms of identifying blood group antigens and in predicting fetal anemia through the use of noninvasive monitoring, many questions remain in terms of understanding RBC alloimmunization risk factors, preventative therapies, and treatment strategies...
October 2016: Transfusion Medicine Reviews
https://www.readbyqxmd.com/read/26807378/acute-hepatic-phenotype-of-wilson-disease-clinical-features-of-acute-episodes-and-chronic-lesions-remaining-in-survivors
#10
Hisao Hayashi, Yasuaki Tatsumi, Shinsuke Yahata, Hiroki Hayashi, Kenji Momose, Ryohei Isaji, Youji Sasaki, Kazuhiko Hayashi, Shinya Wakusawa, Hidemi Goto
BACKGROUND AND AIMS: Wilson disease (WD) is an inherited disorder of copper metabolism, and an international group for the study of WD (IGSW) has proposed three phenotypes for its initial presentation: acute hepatic, chronic hepatic, and neurologic phenotypes. Characterization of the acute hepatic phenotype may improve our understanding of the disease. METHODS: Clinical features of 10 WD patients with the acute hepatic phenotype and characteristics of chronic lesions remaining in survivors were assessed by the European Association for the Study of the Liver (EASL) guidelines...
December 28, 2015: Journal of Clinical and Translational Hepatology
https://www.readbyqxmd.com/read/26634125/therapeutic-dilemma-in-the-management-of-a-patient-with-the-clinical-picture-of-ttp-and-severe-b12-deficiency
#11
Kara Walter, Jennifer Vaughn, Daniel Martin
BACKGROUND: Idiopathic thrombotic thrombocytopenic purpura (TTP) is a rare hematological emergency characterized by the pentad of microangiopathic hemolytic anemia, thrombocytopenia, neurological symptoms, renal injury, and fever that is invariably fatal if left untreated. Prompt intervention with plasma exchange minimizes mortality and is the cornerstone of therapy. Rare reports have described "pseudo-TTP" driven by extreme hematologic abnormalities resulting from deficiency of vitamin B12...
2015: BMC Hematology
https://www.readbyqxmd.com/read/26251141/-management-of-pregnancy-and-delivery-in-patients-with-paroxysmal-nocturnal-hemoglobinuria
#12
REVIEW
Kensuke Usuki
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hemolytic anemia, which generally affects women of childbearing age. PNH hemolysis increases the risk of complications such as thrombosis during pregnancy and the postpartum period. Management of a pregnant woman with PNH remains a challenge due to the high incidence of thrombotic complications and the difficulty of differentiating a PNH crisis from the complications of pregnancy. PNH is associated with an increased rate of premature labor and fetal loss. Eculizumab, a C5 complement inhibitor, is a potential therapeutic option for such patients...
July 2015: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/26009624/a-pediatrician-s-practical-guide-to-diagnosing-and-treating-hereditary-spherocytosis-in-neonates
#13
REVIEW
Robert D Christensen, Hassan M Yaish, Patrick G Gallagher
Newborn infants who have hereditary spherocytosis (HS) can develop anemia and hyperbilirubinemia. Bilirubin-induced neurologic dysfunction is less likely in these neonates if the diagnosis of HS is recognized and appropriate treatment provided. Among neonates listed in the USA Kernicterus Registry, HS was the third most common underlying hemolytic condition after glucose-6-phosphate dehydrogenase deficiency and ABO hemolytic disease. HS is the leading cause of direct antiglobulin test (direct Coombs) negative hemolytic anemia requiring erythrocyte transfusion in the first months of life...
June 2015: Pediatrics
https://www.readbyqxmd.com/read/25790109/icsh-guidelines-for-the-laboratory-diagnosis-of-nonimmune-hereditary-red-cell-membrane-disorders
#14
M-J King, L Garçon, J D Hoyer, A Iolascon, V Picard, G Stewart, P Bianchi, S-H Lee, A Zanella
INTRODUCTION: Hereditary spherocytosis (HS), hereditary elliptocytosis (HE), and hereditary stomatocytosis (HSt) are inherited red cell disorders caused by defects in various membrane proteins. The heterogeneous clinical presentation, biochemical and genetic abnormalities in HS and HE have been well documented. The need to raise the awareness of HSt, albeit its much lower prevalence than HS, is due to the undesirable outcome of splenectomy in these patients. METHODS: The scope of this guideline is to identify the characteristic clinical features, the red cell parameters (including red cell morphology) for these red cell disorders associated, respectively, with defective cytoskeleton (HS and HE) and abnormal cation permeability in the lipid bilayer (HSt) of the red cell...
June 2015: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/25710019/mechanisms-linking-red-blood-cell-disorders-and-cardiovascular-diseases
#15
REVIEW
Ioana Mozos
The present paper aims to review the main pathophysiological links between red blood cell disorders and cardiovascular diseases, provides a brief description of the latest studies in this area, and considers implications for clinical practice and therapy. Anemia is associated with a special risk in proatherosclerotic conditions and heart disease and became a new therapeutic target. Guidelines must be updated for the management of patients with red blood cell disorders and cardiovascular diseases, and targets for hemoglobin level should be established...
2015: BioMed Research International
https://www.readbyqxmd.com/read/25696892/thrombotic-microangiopathy-and-indications-for-therapeutic-plasma-exchange
#16
REVIEW
Jill Adamski
Thrombotic microangiopathy (TMA) is a clinicopathological condition associated with a wide variety of medical conditions. TMA is classically characterized by microangiopathic hemolytic anemia, thrombocytopenia, and microvascular thrombi that cause end-organ damage. The most prominent diagnoses associated with TMA are thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). Although TTP and HUS can have similar clinical and laboratory features and are often lumped together as a combined entity referred to as "TTP/HUS," the pathologic processes causing TMA and optimal therapies for these conditions are different...
December 5, 2014: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/25656421/a-new-preparation-method-for-red-blood-cells-for-intrauterine-transfusion-enabling-reduction-of-donor-exposure
#17
Ido J Bontekoe, John Scharenberg, Henk Schonewille, Jaap Jan Zwaginga, Anneke Brand, Pieter F van der Meer, Dirk de Korte
BACKGROUND: Severe hemolytic anemia of the fetus, caused by maternal red blood cell (RBC) alloantibodies, is treated with intrauterine transfusion (IUT) of RBCs. Because IUT is associated with additional antibody formation, RBCs with the closest match between donor and mother are preferred. Because one fetus needs a median of three IUTs, finding such RBCs is complicated. Collection of repeated low-volume donations from one selected donor during the entire IUT treatment period would reduce donor exposure and possibly IUT-associated alloimmunization...
July 2015: Transfusion
https://www.readbyqxmd.com/read/25551305/prevalence-of-maternal-red-cell-alloimmunisation-a-population-study-from-queensland-australia
#18
Manika Pal, Bronwyn Williams
The aim of this study was to determine the current prevalence of red cell antigen alloimmunisation in Australia. Blood group (ABO and RhD) and red cell antibody screen results of pregnant women who presented at public hospitals in Queensland between the period of January 2011 and June 2013 were evaluated retrospectively. Antibody prevalence in pregnancy was compared to other published studies. A total of 482 positive antibody screens from 66,354 samples (0.73%) were identified. The prevalence of antibodies was: anti-E 27...
February 2015: Pathology
https://www.readbyqxmd.com/read/25520957/occurrence-of-hemolytic-anemia-in-patients-with-gbs-treated-with-high-dose-ivig
#19
Thy P Nguyen, Suur Biliciler, Amer Wahed, Kazim Sheikh
OBJECTIVE: We describe an underrecognized side effect of high-dose IV immunoglobulin (IVIg), hemolytic anemia. BACKGROUND: There are no established guidelines on treating patients with Guillain-Barré syndrome (GBS) who relapse or do not improve after a standard course of treatment (IVIg or plasma exchange). Some centers will opt for a second course of the initial treatment. There is an ongoing trial of a second course of IVIg in patients with severe GBS. METHODS: We retrospectively reviewed 4 patients with severe GBS who received high-dose IVIg...
December 2014: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/25491600/appropriate-provision-of-anti-d-prophylaxis-to-rhd-negative-pregnant-women-a-scoping-review
#20
REVIEW
Trina M Fyfe, M Jane Ritchey, Christorina Taruc, Daniel Crompton, Brian Galliford, Rose Perrin
BACKGROUND: The purpose of this scoping review was to review the literature on healthcare provider provision of anti-D prophylaxis to RhD negative pregnant women in appropriate clinical situations in various healthcare settings. METHODS: A scoping review framework was used to structure the process. The following databases were searched: CINAHL (EBSCO), EBM Reviews (OvidSP), Embase (OvidSP), Medline (OvidSP), and Web of Science (ISI). In addition, hand searching of article references was conducted...
December 10, 2014: BMC Pregnancy and Childbirth
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