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Autoimmune hemolytic anemia

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https://www.readbyqxmd.com/read/28416507/thrombotic-thrombocytopenic-purpura
#1
Bérangère S Joly, Paul Coppo, Agnes Veyradier
Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic micro-angiopathy characterized by a microangiopathic hemolytic anemia, severe thrombocytopenia and organ ischemia linked to disseminated microvascular platelet rich-thrombi. TTP is specifically related to a severe deficiency in ADAMTS13 (A Disintegrin and Metalloprotease with ThromboSpondin type 1 repeats, member 13), the specific von Willebrand factor-cleaving pro-tease. ADAMTS13 deficiency is most frequently acquired via ADAMTS13 autoantibodies but rarely, it is inherited via mutations of ADAMTS13 gene...
April 17, 2017: Blood
https://www.readbyqxmd.com/read/28414687/disseminated-cryptococcal-disease-with-diffuse-pulmonary-infiltrates-in-a-non-hiv-host
#2
M Hughes, K Trivedi, M Rudrappa
INTRODUCTION: We present a case of disseminated Cryptococcus in a non-HIV host, where the primary manifestation was pulmonary with diffuse pulmonary infiltrates. This patient was on high dose corticosteroids for autoimmune hemolytic anemia. CASE: A 79 year old Caucasian man with a history of autoimmune hemolytic anemia on 100 mg of prednisone daily, coronary artery disease s/p bypass surgery, ischemic cardiomyopathy, chronic obstructive pulmonary disease, sleep apnea, chronic kidney disease, and history of bilateral pulmonary emboli presented to Hematology/Oncology clinic with symptoms of productive cough, worsening shortness of breath, hemoptysis...
March 2017: Journal of the Louisiana State Medical Society: Official Organ of the Louisiana State Medical Society
https://www.readbyqxmd.com/read/28405337/autoimmune-hemolytic-anemia-associated-with-babesiosis
#3
Roshni Narurkar, Aleksandra Mamorska-Dyga, John C Nelson, Delong Liu
BACKGROUND: Babesiosis is endemic in selected areas in North America. Babesia infection is commonly associated with anemia, thrombocytopenia, hyponatremia and elevated liver enzymes. Autoimmune hemolytic anemia (AIHA) is known to be caused by parasitic and viral infections but has not been well characterized. CASE PRESENTATION: We describe two cases diagnosed with babesiosis triggering severe AIHA. One case had history of splenectomy, and the other was an elderly patient...
2017: Biomarker Research
https://www.readbyqxmd.com/read/28401101/warm-autoimmune-hemolytic-anemia-experience-from-a-single-referral-center-in-mexico-city
#4
Hernandez-Company Alonso, Anguiano-Alvarez Victor Manuel, Carmona Gonzalez Carlos Amir, Rodriguez-Rodriguez Sergio, Pomerantz Allan, Lopez-Karpovitch Xavier, Tuna-Aguilar Elena Juventina
BACKGROUND: Autoimmune hemolytic anemia (AIHA) is characterized by an autoimmune-mediated destruction of red blood cells. Warm AIHA (wAIHA) represents 60% of AIHA cases and is associated with the positive detection of IgG and C3d in the direct antiglobulin test (DAT). This study aimed to assess the clinical and laboratorial differences between primary and secondary wAIHA patients from a referral center in Mexico City. METHODS: All patients diagnosed with wAIHA in our institution from January 1992 to December 2015 were included and received corticosteroids as the first-line treatment...
March 2017: Blood Research
https://www.readbyqxmd.com/read/28400547/anemia-in-thyroid-diseases
#5
Ewelina Szczepanek-Parulska, Aleksandra Hernik, Marek Ruchała
Anemia is a frequent, although often underestimated, clinical condition accompanying thyroid diseases. In spite of the fact that anemia and thyroid dysfunction often occur simultaneously, the causative relationship between these two disorders remains ambiguous. Thyroid hormones stimulate erythrocytes precursors proliferation directly, as well as via erythropoietin production enhancement, whereas iron-deficient anemia negatively influences thyroid hormonal status. Thus, different forms of anemia might emerge in the course of thyroid dysfunction...
March 28, 2017: Polish Archives of Internal Medicine
https://www.readbyqxmd.com/read/28397508/an-autoimmune-haemolytic-anaemia-secondary-to-ipilimumab-treatment
#6
B Ramos, G Gastal, R K Rovere
BACKGROUND: Melanoma is one of the fastest growing neoplasms worldwide. Treatment of metastatic disease has swiftly shifted in the last decade from generally ineffective chemotherapy regimens to highly effective targeted treatments or immunotherapy, with a range of side effects that differ completely from those of previous treatments for this disease. CASE: We present a case of a 71-year-old man with diagnosis metastatic melanoma. This patient was treated with anti-CTLA-4 antibody ipilimumab...
2017: Klinická Onkologie: Casopis Ceské a Slovenské Onkologické Spolecnosti
https://www.readbyqxmd.com/read/28394238/anti-ribosomal-p-antibody-a-multicenter-study-in-childhood-onset-systemic-lupus-erythematosus-patients
#7
C C M Valões, B C Molinari, A C G Pitta, N W S Gormezano, S C L Farhat, K Kozu, A M E Sallum, S Appenzeller, A P Sakamoto, M T Terreri, R M R Pereira, C S Magalhães, J C O A Ferreira, C M Barbosa, F H Gomes, E Bonfá, C A Silva
Objectives Anti-ribosomal P protein (anti-P) autoantibodies are highly specific for systemic lupus erythematosus (SLE). However, the evaluation of this autoantibody in childhood-onset SLE (cSLE) populations has been limited to a few small series, hampering the interpretation of the clinical and laboratorial associations. Therefore, the objective of this multicenter cohort study was to evaluate demographic, clinical/laboratorial features, and disease damage score in cSLE patients with and without the presence of anti-P antibody...
April 2017: Lupus
https://www.readbyqxmd.com/read/28389309/steroid-refractory-autoimmune-hemolytic-anemia-with-massive-splenomegaly
#8
Hiroshi Ureshino, Masaharu Miyahara
No abstract text is available yet for this article.
April 4, 2017: American Journal of Medicine
https://www.readbyqxmd.com/read/28371438/agranulocytosis-and-mixed-type-autoimmune-hemolytic-anemia-in-primary-sj%C3%A3-gren-s-syndrome-a-case-report-and-review-of-the-literature
#9
Lin Qiao, Jing Chen, Xiao-Mei Leng, Wen Zhang, Bing Han, Yan Zhao, Xiao-Feng Zeng
Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease that presents with sicca symptoms of the main mucosal surfaces. Patients with pSS have a broad spectrum of laboratory features, such as cytopenias and hypergammaglobulinemia. Although hematological abnormalities are usually seen in pSS patients, agranulocytosis and autoimmune hemolytic anemia (AIHA) are rare. Here we describe a 40-year-old woman with pSS who developed both agranulocytosis and mixed-type AIHA. An increased risk of malignancies has also been reported in pSS patients with hematological changes...
December 2016: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28369934/recurrent-donath-landsteiner-hemolytic-anemia-a-pediatric-case-report
#10
Sara D Prince, Lena E Winestone, Sandra J Nance, David F Friedman
BACKGROUND: Paroxysmal cold hemoglobinuria (PCH) is a form of autoimmune hemolytic anemia caused by the Donath-Landsteiner antibody (D-L antibody). In children, this is typically a transient immune-mediated hemolysis that follows a viral illness and does not recur. Recurrent acute or chronic PCH due to D-L antibody is very rare. CASE REPORT: We have reported a unique case of recurrent PCH in a 5-year-old boy with two acute episodes of hemolysis separated by 21 months of hematologic remission...
March 31, 2017: Transfusion
https://www.readbyqxmd.com/read/28367431/a-3-year-old-girl-with-recurrent-infections-and-autoimmunity-due-to-a-stat1-gain-of-function-mutation-the-expanding-clinical-presentation-of-primary-immunodeficiencies
#11
Juan Carlos Aldave Becerra, Enrique Cachay Rojas
We report a 3-year-old Peruvian girl, born to non-consanguineous parents. At the age of 8 months, she had a severe pneumonia complicated with empyema that required thoracic drainage and mechanical ventilation. Although no microorganisms were isolated, the patient recovered with broad-spectrum antibiotics. Since that date, she has presented multiple episodes of pneumonia and recurrent episodes of bronchospasm. At 1 year 5 months of age, the patient began with recurrent episodes of oropharyngeal, vaginal, and skin candidiasis, which improved transiently after using oral azole drugs...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28360039/how-i-treat-autoimmune-hemolytic-anemia
#12
Ronald S Go, Jeffrey L Winters, Neil E Kay
Autoimmune hemolytic anemia (AIHA) is an uncommon entity that presents diagnostic, prognostic and therapeutic dilemmas despite being a well-recognized entity for over 150 years. Because of significant differences in the rates of hemolysis as well as associated diseases and cause there is considerable clinical heterogeneity. In addition there is a lack of clinical trials required to refine and update standardized and evidence-based therapeutic approaches. To aid the clinician in AIHA management we present four vignettes that represent and highlight distinct clinical presentations with separate diagnostic and therapeutic pathways that we utilize in our clinical practice setting...
March 30, 2017: Blood
https://www.readbyqxmd.com/read/28324773/haptoglobin-is-frequently-low-in-patients-with-myelofibrosis-clinical-relevance
#13
Paolo Strati, Lucia Masarova, Prithviraj Bose, Naval Daver, Naveen Pemmaraju, Srdan Verstovsek
A recent study, showing the absence of paroxysmal nocturnal hemoglobinuria clones in myelofibrosis, has reopened the debate around the role of decreased haptoglobin in this disease. We present here a large prospective analysis of the clinical significance of low haptoglobin in 152 patients with myelofibrosis. Low haptoglobin (<32mg/dL) was observed in 50 patients (33%). Decreased haptoglobin did not associate with low hemoglobin levels, positive Coombs test or abnormal liver function tests, suggesting it is not result of autoimmune hemolytic anemia or liver cirrhosis...
March 8, 2017: Leukemia Research
https://www.readbyqxmd.com/read/28316442/alloimmunization-in-autoimmune-hemolytic-anemia-patient-the-differential-adsorption-approach
#14
Ravi C Dara, Aseem Kumar Tiwari, Dinesh Arora, Subhasis Mitra, Devi Prasad Acharya, Geet Aggarwal, Jyoti Sharma
Patients of β-thalassemia major are dependent on regular blood transfusions for their entire lifetime. Development of antibodies against red blood cell (RBC) antigen which may be alloantibody or autoantibody, several times as a result of frequent red cell component transfusions, further complicates the subsequent transfusion therapy. Among the autoantibodies, warm-reactive autoantibodies are commoner and interfere in the pretransfusion testing. These RBC autoantibodies present in patient's serum potentially react with all the cells of antibody identification panel giving "pan-reactive" picture and making alloantibody identification complex...
January 2017: Asian Journal of Transfusion Science
https://www.readbyqxmd.com/read/28298568/comparison-of-disease-characteristics-organ-damage-and-survival-in-patients-with-juvenile-onset-and-adult-onset-systemic-lupus-erythematosus-in-a-combined-cohort-from-2-tertiary-centers-in-turkey
#15
Bahar Artim-Esen, Sezgin Şahin, Erhan Çene, Yasemin Şahinkaya, Kenan Barut, Amra Adrovic, Yasemin Özlük, Işın Kılıçaslan, Ahmet Omma, Ahmet Gül, Lale Öcal, Özgür Kasapçopur, Murat İnanç
OBJECTIVE: Age at onset has been shown to affect the clinical course and outcome of systemic lupus erythematosus (SLE). Herein, we aimed to define the differences in clinical characteristics, organ damage, and survival between patients with juvenile-onset (jSLE) and adult-onset SLE (aSLE). METHODS: For the study, 719 patients (76.9%) with aSLE and 216 (23.1%) with jSLE were examined. Comparisons between the groups were made for demographic characteristics, clinical features, autoantibody profiles, damage, and survival rates...
March 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28285286/necrotizing-polyarteritis-nodosa-like-vasculitis-in-a-child-with-systemic-lupus-erythematosus
#16
Ritambhra Nada, Joseph L Matthews, Sagar Bhattad, Anju Gupta, Surjit Singh
A 10-year-old child presented with prolonged fever, lymphadenopathy, weight loss, oral ulcers, alopecia and parotitis. She later developed arterial thrombosis, poly-serositis, nephritis, myocarditis, sacro-ilitis, autoimmune hemolytic anemia and refractory thrombocytopenia. Though anti-dsDNA was negative, she was diagnosed to have systemic lupus erythematosus (SLE). Terminally, she had pulmonary symptoms and succumbed to her illness. The autopsy showed lupus nephritis-Class II, polyserositis, myocarditis, inflammatory myositis, immune mediated vasculitis involving renal, coronary, pancreatic, adrenal, dermal and intramuscular arteries, and pulmonary hemorrhages and edema...
February 15, 2017: Indian Pediatrics
https://www.readbyqxmd.com/read/28273010/post-babesiosis-warm-autoimmune-hemolytic-anemia
#17
Ann E Woolley, Mary W Montgomery, William J Savage, Maureen O Achebe, Kathleen Dunford, Sarah Villeda, James H Maguire, Francisco M Marty
Background Babesiosis, a tickborne zoonotic disease caused by intraerythrocytic protozoa of the genus babesia, is characterized by nonimmune hemolytic anemia that resolves with antimicrobial treatment and clearance of parasitemia. The development of warm-antibody autoimmune hemolytic anemia (also known as warm autoimmune hemolytic anemia [WAHA]) in patients with babesiosis has not previously been well described. Methods After the observation of sporadic cases of WAHA that occurred after treatment of patients for babesiosis, we conducted a retrospective cohort study of all the patients with babesiosis who were cared for at our center from January 2009 through June 2016...
March 9, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/28266038/using-direct-antiglobulin-test-results-to-reduce-unnecessary-cold-agglutinin-testing
#18
Craig B Wilen, Garrett S Booth, Brenda J Grossman, William J Lane, Penny C Szklarski, Ronald Jackups
BACKGROUND: Cold agglutinin disease (CAD) is a rare autoimmune hemolytic anemia mediated by autoantibodies that preferentially react at 4°C. Laboratory testing for cold-reactive autoantibodies is laborious and may not be ordered judiciously, particularly in patients with a negative direct antiglobulin test (DAT). We sought to determine whether a negative DAT using anti-human complement (anti-C3) rules out elevated cold agglutinin (CA) titers and the diagnosis of CAD. STUDY DESIGN AND METHODS: We performed a retrospective study of patients with a CA test performed at three major academic medical centers: Barnes-Jewish Hospital (2003-2014), Vanderbilt University Medical Center (2007-2009), and Massachusetts General Hospital (2009-2014)...
March 7, 2017: Transfusion
https://www.readbyqxmd.com/read/28254202/predictors-of-granulomatous-lymphocytic-interstitial-lung-disease-in-common-variable-immunodeficiency
#19
Stella Hartono, Megan S Motosue, Shakila Khan, Vilmarie Rodriguez, Vivek N Iyer, Rohit Divekar, Avni Y Joshi
BACKGROUND: A subset of patients with common variable immunodeficiency (CVID) develop granulomatous lymphocytic interstitial lung disease (GLILD), which is associated with early mortality. OBJECTIVE: To determine a set of clinical and/or laboratory parameters that correlate with GLILD. METHODS: A retrospective, nested case-control (patients with CVID diagnosed with GLILD compared with patients with CVID without a diagnosis of GLILD) medical record review was undertaken at Mayo Clinic, Rochester, MN...
February 18, 2017: Annals of Allergy, Asthma & Immunology
https://www.readbyqxmd.com/read/28252632/-autoimmune-hemolytic-anemia-associated-with-mesenteric-teratoma
#20
K I Ntanishyan, K R Sabirov, O V Shcherbakova, D E Vybornykh, I A Shupletsova, N V Tsvetaeva
The paper describes a case of autoimmune hemolytic anemia (AIHA) in a 27-year-old woman whose examination revealed mesenteric teratoma. AIHA was characterized by a hypertensive crisis and a temporary response to corticosteroid therapy that was complicated by the development of somatogenic psychosis and discontinued. A relapse of hemolysis developed 6 months later. The patient underwent laparoscopic splenectomy and removal of mesenteric root teratoma. Immediately after surgery, a hematological response was obtained as relief of hemolysis and restoration of a normal hemoglobin level...
2017: Terapevticheskiĭ Arkhiv
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