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hemolytic anemias

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https://www.readbyqxmd.com/read/29788810/clinical-and-immunological-pattern-and-outcome-of-egyptian-systemic-lupus-erythematosus-patients-a-single-center-experience
#1
G A Mahmoud, A A Shahin, H S Zayed, A Moghazy, B M Eissa
Objective The objective of this study was to describe the clinical and immunological pattern and disease outcome in Egyptian systemic lupus erythematosus patients. Patients and methods The medical records of 770 systemic lupus erythematosus patients who were followed from 2002-2015 at Kasr Alainy Hospital, Cairo University, were retrospectively reviewed. Results There were 707 (91.8%) females. The mean age at disease onset was 22.1 ± 8.6 and the disease duration was 6.1 ± 4.5 years. The main clinical manifestations were mucocutaneous (90...
January 1, 2018: Lupus
https://www.readbyqxmd.com/read/29783824/-clinical-analysis-of-seven-cases-of-rare-hemolytic-disease-of-the-newborn
#2
S Gu, H X Wang, C Y Yang, X F Yang, Y Lin, Y Zhong, J P He, Y J Wang
Objective: To summarize the clinical features of 7 rare cases of hemolytic disease of newborn (HDN), and to improve the understanding of rare HDN. Methods: Data of clinical information, laboratory findings, treatments and outcomes were collected and analyzed for four cases with HDN due to anti-M, two cases due to anti-Kidd, and one case due to anti-Duffy. All of them were admitted to the Department of Neonatology, Beijing Children's Hospital Affiliated to Capital Medial University from July 2007 to June 2017...
May 2, 2018: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29779353/-using-target-next-generation-sequencing-assay-in-diagnosing-of-46-patients-with-suspected-congenital-anemias
#3
Y Li, G X Peng, Q Y Gao, Y Li, L Ye, J P Li, L Song, H H Fan, Y Yang, Y Z Xiong, Z J Wu, W R Yang, K Zhou, X Zhao, L P Jing, F K Zhang, L Zhang
Objective: To evaluate the impact of the targeted next-generation sequencing (NGS) assay for difficult congenital anemias. Methods: Blood Disease Hospital Anemia Panel 2014 (BDHAP-2014) including 217 known genes of congenital anemias was developed. NGS and parental verification were performed for patients who were suspected diagnosed with congenital anaemia from August 2014 to July 2017. Results: A total of 46 patients were enrolled in this study, the clinical suspection were 11 cases Fanconi anemia (FA), 8 cases congenital dyserythropoietic anemia (CDA), 6 cases congenital sideroblast anemia (CSA), 12 cases congenital hemolytic anemia (CHA), 1 case dyskeratosis congenital (DC), 4 cases iron-refractory iron deficiency anemia and 4 cases unexplained cytopenia (Uc), respectively...
May 14, 2018: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/29779325/-comparison-of-hemolytic-characteristics-among-paroxysmal-nocturnal-hemoglobinuria-autoimmune-hemolytic-anemia-and-hereditary-spherocytosis
#4
W W Li, J Shi, Z D Huang, N Nie, Y Q Shao, X X Li, M L Ge, J Zhang, J B Huang, P Jin, M Wang, Y Z Zheng
Objective: To determine the valuable hemolytic characteristics in differential diagnosis of paroxysmal nocturnal hemoglobinuria (PNH), autoimmune hemolytic anemia (AIHA) and hereditary spherocytosis (HS). Method: The clinical and hemolytic characteristics of 108 PNH patients, 127 AIHA patients and 172 HS patients diagnosed from January 1998 to April 2017 were compared. Results: ①Reticulocyte percentage (Ret%) of PNH patients [6.70% (0.14%-22.82%)] was significantly lower than that of AIHA [14.00%(0.10%-55...
April 14, 2018: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/29777547/tacrolimus-induced-asymptomatic-thrombotic-microangiopathy-diagnosed-by-laboratory-tests-in-pig-to-rhesus-corneal-xenotransplantation-a-case-report
#5
Jong-Min Kim, Jaeyoung Kim, Se-Hyun Choi, Jun-Seop Shin, Byoung-Hoon Min, Won Young Jeong, Ga-Eul Lee, Min-Sun Kim, Seeun Kwon, Mee Kum Kim, Chung-Gyu Park
Tacrolimus-associated thrombotic microangiopathy (TA-TMA) is a rare complication. TA-TMA is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ damage due to thrombus. We report asymptomatic TA-TMA diagnosed by laboratory tests in pig-to-rhesus corneal xenotransplantation. Corneal transplantation had been conducted from a wild-type SNU miniature pig to a rhesus macaque. The veterinary records were retrospectively reviewed in this case. The immunosuppressive regimen consisted of rituximab, basiliximab, and IVIg as inductive therapies, and steroids with tacrolimus (0...
May 18, 2018: Xenotransplantation
https://www.readbyqxmd.com/read/29772969/case-control-study-of-plasma-mean-platelet-component-concentration-and-survival-analysis-for-dogs-with-immune-mediated-hemolytic-anemia
#6
Andrea Zoia, Magda Gerou-Ferriani, Michele Drigo, Marco Caldin
OBJECTIVE To determine whether dogs with immune-mediated hemolytic anemia (IMHA) had a low plasma mean platelet component (MPC) concentration and whether MPC was associated with outcome. DESIGN Retrospective case-control study and survival analysis. ANIMALS 95 dogs with IMHA (cases) as well as 95 healthy dogs and 95 sick dogs without IMHA (controls) matched to cases by age, reproductive status, and breed. PROCEDURES Plasma MPC concentration at initial examination was compared among groups. For dogs with IMHA only, sex, age, serum urea and bilirubin concentrations, Hct, platelet count, and plasma fibrinogen, D-dimer, and MPC concentrations were evaluated for associations with survival to 42 days after initial examination...
June 1, 2018: Journal of the American Veterinary Medical Association
https://www.readbyqxmd.com/read/29771863/upshaw-schulman-syndrome-with-c-2728c-t-mutation-in-adamts13-gene
#7
Shahzadi Resham, Zehra Fadoo, Bushra Moiz
Congenital thrombotic thrombocytopenic purpura is a rare autosomal recessive disorder presenting with hemolytic anemia, thrombocytopenia, micro vascular thrombosis, and end organ damage. Here, we present a case of a 7-year-old girl having recurrent neonatal hemolysis, developmental delay, frequent seizures, and thrombocytopenia. Characteristic clinical picture and gene sequencing of a disintegrin and metalloproteinase with thrombospondin motifs 13 confirmed the diagnosis of Upshaw-Schulman syndrome. She was treated successfully with plasma infusion...
May 16, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29768958/bevacizumab-induced-atypical-hemolytic-uremic-syndrome-and-treatment-with-eculizumab
#8
Anusha Vakiti, Daulath Singh, Ravi Pilla, Muhamad A Moustafa, Kelly W Fitzpatrick
Bevacizumab (Avastin) is a recombinant humanized monoclonal antibody used for the management of various solid malignancies including colorectal, lung, brain, renal, and ovarian cancers as well as age-related macular degeneration of the eye. It is a vascular endothelial growth factor inhibitor which exhibits its action by blocking the growth of blood vessels in cancerous tissue. Common side effects include hypertension, fatigue, headaches, and increased risk of infections. Atypical hemolytic uremic syndrome is a serious side effect associated with bevacizumab due to its anti-angiogenic effect...
January 1, 2018: Journal of Oncology Pharmacy Practice
https://www.readbyqxmd.com/read/29767893/-hemolytic-anemia-in-cancer
#9
Maria Anastasiou, Yves Chalandon, Sandro Anchisi
Hemolysis (from the Greek word: hemolysis: hema = blood + lysis = liberation) is a medical term that describes red blood cell's destruction by mechanisms of lysis of the membrane. An hemolytic anemia occurs when excessive destruction of red blood cells overwhelms bone marrow's capacity of regeneration. Although anemia is frequently associated with an oncologic disease, hemolytic anemia is rarely diagnosed in oncologic patients. Consequently, a massive hemolysis can be quickly deleterious and often fatal. An early diagnosis can improve survival and can be made with inexpensive tests...
May 16, 2018: Revue Médicale Suisse
https://www.readbyqxmd.com/read/29766398/hepatosplenic-t-cell-lymphoma-a-unifying-entity-in-a-patient-with-hemolytic-anemia-massive-splenomegaly-and-liver-dysfunction
#10
Marianna Mavilia, Agnes McAuliffe, Safina Hafeez, Haleh Vaziri
Hepatosplenic T cell lymphoma (HSTCL) is a rare subtype of peripheral non-Hodgkin lymphoma, of which 20% of cases are associated with chronic immunosuppression. It often occurs in patients with inflammatory bowel disease treated with immunomodulating medications such a thiopurines or TNF-alpha inhibitors. Cytopenias are commonly seen but autoimmune hemolytic anemia (AIHA) is rare. Here we present a young male with longstanding ulcerative colitis on chronic azathioprine, exhibiting several rare features of HSTCL...
May 15, 2018: Clinical Journal of Gastroenterology
https://www.readbyqxmd.com/read/29756358/echocardiographic-parameters-to-identify-sickle-cell-patients-with-cardio-pathology
#11
Simbo Chiadika, Mary Lim-Fung, Fiorella Llanos-Chea, Astrid Serauto Canache, Wei Yang, Christina Paruthi, Xu Zhang, David D McPherson, Modupe Idowu
BACKGROUND: Sickle cell disease (SCD) affects millions of people and causes chronic hemolytic anemia leading to vasculopathies such as pulmonary hypertension and abnormalities in cardiac function that increase complications and mortality. It is therefore crucial to identify cardiac abnormalities in SCD. We aimed to assess the prevalence of echocardiographic parameters in SCD to help identify cardiopulmonary risk. METHODS: Ninety-one patients (53% male), median age of 30, body surface area (BSA) of 1...
May 14, 2018: Echocardiography
https://www.readbyqxmd.com/read/29755372/use-of-laser-assisted-optical-rotational-cell-analyzer-lorrca-maxsis-in-the-diagnosis-of-rbc-membrane-disorders-enzyme-defects-and-congenital-dyserythropoietic-anemias-a-monocentric-study-on-202-patients
#12
Anna Zaninoni, Elisa Fermo, Cristina Vercellati, Dario Consonni, Anna P Marcello, Alberto Zanella, Agostino Cortelezzi, Wilma Barcellini, Paola Bianchi
Chronic hemolytic anemias are a group of heterogeneous diseases mainly due to abnormalities of red cell (RBC) membrane and metabolism. The more common RBC membrane disorders, classified on the basis of blood smear morphology, are hereditary spherocytosis (HS), elliptocytosis, and hereditary stomatocytoses (HSt). Among RBC enzymopathies, the most frequent is pyruvate kinase (PK) deficiency, followed by glucose-6-phosphate isomerase, pyrimidine 5' nucleotidase P5'N, and other rare enzymes defects. Because of the rarity and heterogeneity of these diseases, diagnosis may be often challenging despite the availability of a variety of laboratory tests...
2018: Frontiers in Physiology
https://www.readbyqxmd.com/read/29753419/severe-renal-failure-and-thrombotic-microangiopathy-induced-by-malignant-hypertension-successfully-treated-with-spironolactone
#13
H M Villafuerte Ledesma, J M Peña Porta, P Iñigo Gil, P Martin Azara, J E Ruiz Lalglesia, A Tomás LaTorre, A Martínez Burillo, P Vernet Perna, R Álvarez Lipe
Malignant hypertension can cause thrombotic microangiopathy (TMA) characterized by hemolytic anemia and thrombocytopenia. On the other hand, severe hypertension is sometimes associated with hemolytic uremic syndrome (HUS) or thrombotic thrombocytopenic purpura (TTP). Distinguishing these entities is important because of therapeutic implications. Plasmapheresis should be initiated as soon as possible if we are dealing with TTP. We describe the case of a 30-year-old man referred to our hospital with malignant hypertension, severe renal failure and TMA: haemoglobin=9g/dL, total bilirubin=0...
May 9, 2018: Annales de Cardiologie et D'angéiologie
https://www.readbyqxmd.com/read/29750742/an-atypical-case-of-atypical-hemolytic-uremic-syndrome
#14
Tine Francois, Johan Vande Walle, Evelyn Dhont, Werner Keenswijk
We present the case of a 2-month-old infant presenting with pallor and laboratory results showing: hemoglobin 5.1 (10 to 1.5) g/dL, MCV 94.7 (75 to 105) fL, leukocytes 17.4 (7 to 15) ×10/μL, platelets 259 (150 to 450) ×10/μL, hyperbilirubinemia and renal dysfunction. A hemolytic anemia with tubular injury secondary to hemoglobinuria was suspected. Hyperhydration and packed cells were given but she deteriorated. Fluid overload with anuria further complicated the course necessating hemodialysis. Atypical hemolytic uremic syndrome was suspected and eculizumab was administered resulting in rapid improvement...
May 10, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29744115/thrombotic-thrombocytopenic-purpura-possibly-triggered-by-graves-disease
#15
Saurabh D Chitnis, Tuoyo O Mene-Afejuku, Amandeep Aujla, Ahmed Shady, Gaby S Gil, Eder Hans Cativo, Andrea Popescu-Martinez
Thrombotic thrombocytopenic purpura (TTP) is a part of a spectrum of thrombotic microangiopathy syndromes which are mainly characterized by platelet aggregation causing microangiopathic hemolytic anemia, thrombocytopenia and microvascular occlusion. In literature, very few cases expressing a direct association between pre-existing Grave's disease and TTP have been described. A 37-year-old African-American woman with past medical history of Grave's disease and polysubstance abuse who presented with complaints of dyspnoea at rest and chest pain was diagnosed to have TTP on further evaluation...
October 2017: Oxford Medical Case Reports
https://www.readbyqxmd.com/read/29743397/-transplantation-associated-thrombotic-microangiopathy-confirmed-by-renal-biopsy
#16
Satoshi Miyamoto, Shunsuke Kimura, Yosuke Hosoya, Daisuke Hasegawa, Hisashi Ishida, Atsuro Daida, Toshihiro Matsui, Yuri Yoshimoto, Shinsuke Hirabayashi, Takuya Fujimaru, Tadashi Kumamoto, Shin-Ichiro Mori, Koyu Suzuki, Atsushi Manabe
An eight-year-old girl with myelodysplastic syndrome (refractory cytopenia) received a bone marrow transplant (BMT) from an unrelated donor because of immunosuppressive therapy failure. Following administration of foscarnet for cytomegalovirus reactivation at day40 post-BMT, serum creatinine increased, and proteinuria, hematuria, and hypertension gradually exacerbated and became prolonged. However, neither schistocytosis nor other organ damage was evident. At six months post-BMT, renal biopsy revealed diffuse glomerular damage with glomerular lobulation, a double contour of the glomerular basement membrane, erythrocyte congestion and thrombi in the glomerular endocapillaries, and mesangiolysis, confirming the diagnosis of transplantation-associated thrombotic microangiopathy (TA-TMA)...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/29741259/primary-red-cell-hydration-disorders-pathogenesis-and-diagnosis
#17
A Caulier, R Rapetti-Mauss, H Guizouarn, V Picard, L Garçon, C Badens
Hydration status is critical for erythrocyte survival and is mainly determined by intracellular cation content. Active pumps, passive transporters, and ion channels are the key components of volume homeostasis, whereas water passively fits ionic movements. Whenever cation content increases, erythrocyte swells, whereas it shrinks when cation content decreases. Thus, inappropriate cation leak causes erythrocyte hydration disorders, hemolytic anemia, and characteristic red cell shape abnormalities named stomatocytosis...
May 2018: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/29739088/thrombotic-microangiopathy-like-hemolysis-in-vitamin-b12-deficiency-related-macrocytic-anemia
#18
Masayoshi Yamanishi, Shigeru Koba, Takaaki Jo, Tohru Kotera, Shinsaku Imashuku
BACKGROUND: Hemolytic features in patients with pernicious anemia have not been emphasized. METHODS: Seven Japanese patients at 60 - 88 years of age with vitamin B12 deficiency-related hemolytic anemia were assessed. RESULTS: Serum vitamin B12 levels in these cases were 46 - 89 pg/mL (normal reference range: 233 - 914 pg/mL). Clinically, the patients presented with thrombotic microangiopathy (TMA)-like hemolytic features (including macrocytic anemia, schistocytes on blood smears, high serum lactate dehydrogenase, hyperbilirubinemia, and low serum haptoglobin)...
April 1, 2018: Clinical Laboratory
https://www.readbyqxmd.com/read/29732576/transfusions-of-least-incompatible-blood-with-intravenous-immunoglobulin-plus-steroids-cover-in-two-patients-with-rare-antibody
#19
Nay Win, Malcolm Needs, Nicole Thornton, Robert Webster, Cherry Chang
BACKGROUND: The therapeutic value of the use of intravenous immunoglobulin (IVIG) to correct anemia and thrombocytopenia as a result of immunologic causes (hemolytic disease of the fetus and newborn and fetal or neonatal alloimmune thrombocytopenia) have been well established. Few published papers exist regarding the use of IVIG in adult settings. We report two patients with clinically significant antibodies against high-incidence antigens, who were successfully transfused with incompatible red blood cells (RBCs), in conjunction with IVIG plus steroids and IVIG...
May 6, 2018: Transfusion
https://www.readbyqxmd.com/read/29728803/acquired-thrombotic-thrombocytopenic-purpura-with-isolated-cfhr3-1-deletion-rapid-remission-following-complement-blockade
#20
Martin Bitzan, Rawan M Hammad, Arnaud Bonnefoy, Watfa Shahwan Al Dhaheri, Catherine Vézina, Georges-Étienne Rivard
BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is caused by the abundance of uncleaved ultralarge von Willebrand factor multimers (ULvWF) due to acquired (autoantibody-mediated) or congenital vWF protease ADAMTS13 deficiency. Current treatment recommendations include plasma exchange therapy and immunosuppression for the acquired form and (fresh) frozen plasma for congenital TTP. CASE-DIAGNOSIS/TREATMENT: A previously healthy, 3-year-old boy presented with acute microangiopathic hemolytic anemia, thrombocytopenia, erythrocyturia and mild proteinuria, but normal renal function, and elevated circulating sC5b-9 levels indicating complement activation...
May 4, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
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