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Hemolytic anemia

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https://www.readbyqxmd.com/read/28324773/haptoglobin-is-frequently-low-in-patients-with-myelofibrosis-clinical-relevance
#1
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/28304246/a-mouse-model-for-human-unstable-hemoglobin-santa-ana
#2
Samantha I Miyashiro, Silvia M G Massironi, Claudia M C Mori, Carolina C Cruz, Mitika K Hagiwara, Paulo C Maiorka
In the present study, we described the phenotype, histologic morphology, and molecular etiology of a mouse model of unstable hemoglobin Santa Ana. Hematologic evaluation of anemic mice (Anem/+) discovered after N-ethyl-N-nitrosourea mutagenesis revealed moderate anemia with intense reticulocytosis and polychromasia, followed by anisocytosis, macrocytosis, hypochromia, and intraerythrocytic inclusion and Heinz bodies. The mice also demonstrated hemoglobinuria, bilirubinemia, and erythrocytic populations with differing resistance to osmotic lysis...
December 1, 2016: Comparative Medicine
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
#3
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/28286630/a-fatal-case-of-immune-hyperhemolysis-with-bone-marrow-necrosis-in-a-patient-with-sickle-cell-disease
#4
Matthew S Karafin, Arun Singavi, Susan T Johnson, Joshua J Field
In patients with sickle cell disease, hyperhemolysis is a rare but life-threatening complication of transfusion. In this case report, we describe a 61 year-old woman with hemoglobin sickle cell (SC) disease and history of alloimmunization who developed hyperhemolysis associated with a transfusion. She was found to have a warm and a clinically-significant cold autoantibody. Severe anemia (Hb 2.7 g/dL) with reticulocytopenia and thrombocytopenia prompted a bone marrow biopsy, which demonstrated extensive bone marrow necrosis...
February 23, 2017: Hematology Reports
https://www.readbyqxmd.com/read/28285286/necrotizing-polyarteritis-nodosa-like-vasculitis-in-a-child-with-systemic-lupus-erythematosus
#5
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/28284610/association-of-ehrlichia-canis-hemotropic-mycoplasma-spp-and-anaplasma-platys-and-severe-anemia-in-dogs-in-thailand
#6
Gunn Kaewmongkol, Nicha Lukkana, Sarawut Yangtara, Sarawan Kaewmongkol, Naris Thengchaisri, Theerapol Sirinarumitr, Sathaporn Jittapalapong, Stanley G Fenwick
Canine tick-borne bacteria; Ehrlichia canis, hemotropic Mycoplasma spp. and Anaplasma spp., are organisms transmitted by Rhipicephalus sanguineus ticks. However, only a few clinical studies evaluating dogs infected with these organisms and anemia condition have been published. In this study, the potential tick-borne bacteria linked to anemia were investigated in eighty-one blood samples selected from anemic dogs using a broad range nested-PCR of the 16S rRNA gene. Positive results were shown in 12/81 blood specimens (14...
March 2017: Veterinary Microbiology
https://www.readbyqxmd.com/read/28283300/microangiopathic-hemolytic-anemia-in-pregnancy
#7
Mohammed Salhab, Andrew Hsu, Elizabeth Ryer, Juliet Appiah, Bradley Switzer
Microangipathic hemolytic anemia (MAHA) is a serious diagnosis and difficult to manage in pregnant patients as multiple life threatening conditions could present with MAHA. ADAMTS13 enzyme activity can be affected during pregnancy with multiple factors. A persistent extremely low ADAMTS13 enzyme activity levels, without an inhibitor, after the delivery was an important factor to establish the diagnosis. We present a case of likely congenital ADAMST13 deficiency that manifested for the first time in a pregnant woman at week 37 of pregnancy...
February 16, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28281830/imaging-of-musculoskeletal-manifestations-in-sickle-cell-disease-patients
#8
Vijaya Kosaraju, Alok Harwani, Sasan Partovi, Nicholas Bhojwani, Vasant Garg, Sabarish Ayyappan, Christos Kosmas, Mark Robbin
Sickle cell disease (SCD) is a hereditary red cell disorder with clinical manifestations secondary to sickling or crescent shaped distortion of the red blood cells. Major clinical manifestations of sickle cell disease include hemolytic anemia and vaso-occlusive phenomena resulting in ischemic tissue injury and organ damage. Chronic sequelae of the anemia and vaso-occlusive processes involving the musculoskeletal system include complications related to extramedullary hematopoiesis, osteonecrosis, myonecrosis, and osteomyelitis...
March 10, 2017: British Journal of Radiology
https://www.readbyqxmd.com/read/28277850/diagnosis-and-management-of-catastrophic-antiphospholipid-syndrome
#9
Or Carmi, Maya Berla, Yehuda Shoenfeld, Yair Levy
Catastrophic antiphospholipid syndrome (CAPS) is a rare, life-threatening disease. In 1992, Asherson defined it as a widespread coagulopathy related to the antiphospholipid antibodies (aPL). CAPS requires rapid diagnosis and prompt initiation of treatment. Areas covered: This paper discusses all aspects of CAPS, including its pathophysiology, clinical manifestations, diagnostic approaches, differential diagnoses, management and treatment of relapsing CAPS, and its prognosis. To obtain the information used in this review, scientific databases were searched using the key words antiphospholipid antibodies, catastrophic antiphospholipid syndrome, hemolytic anemia, lupus anticoagulant, and thrombotic microangiopathic hemolytic anemia...
March 1, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28277805/intrauterine-transfusion-and-non-invasive-treatment-options-for-hemolytic-disease-of-the-fetus-and-newborn-review-on-current-management-and-outcome
#10
Carolien Zwiers, Inge van Kamp, Dick Oepkes, Enrico Lopriore
Hemolytic disease of the fetus and newborn (HDFN) remains a serious pregnancy complication which can lead to severe fetal anemia, hydrops and perinatal death. Areas covered: This review focusses on the current prenatal management, treatment with intrauterine transfusion (IUT) and promising non-invasive treatment options for HDFN. Expert commentary: IUTs are the cornerstone in prenatal management of HDFN and have significantly improved perinatal outcome in the past decades. IUT is now a relatively safe procedure, however the risk of complications is still high when performed early in the second trimester...
March 20, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28275334/clinical-significance-of-an-alloantibody-against-the-kell-blood-group-glycoprotein
#11
Stella Maris Mattaloni, Carine Arnoni, Rosario Céspedes, Claudia Nonaka, Carolina Trucco Boggione, Melina Eliana Luján Brajovich, Andrea Trejo, Néstor Zani, Claudia Silvia Biondi, Lilian Castilho, Carlos Miquel Cotorruelo
BACKGROUND: Kell null (K0) individuals can produce anti-Ku, an antibody against many epitopes in the Kell glycoprotein, after transfusion and/or pregnancy. Since sensitized K0 patients are rare, little is known about anti-Ku clinical relevance and in particular about its association to hemolytic disease of the fetus and newborn. CASE REPORT: This work describes a case of neonatal hyperbilirubinemia due to immune-mediated erythrocyte destruction by an alloantibody directed against the Kell glycoprotein...
January 2017: Transfusion Medicine and Hemotherapy
https://www.readbyqxmd.com/read/28273010/post-babesiosis-warm-autoimmune-hemolytic-anemia
#12
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/28267201/a-case-of-anti-rd-causing-fetal-anemia
#13
Stefan Rauch, Jochen Ritgen, Matthias Wißkirchen, Ursula Bauerfeind, Elisabeth Kohne, Christof Weinstock
BACKGROUND: Rd (SC4) is a low-frequency antigen of the Scianna blood group system. Only very few reports on anti-Rd in pregnancy exist. Mild to moderate hemolytic disease of the newborn caused by anti-Rd has been reported. This report may add further information on the clinical significance of anti-Rd for the fetus. CASE REPORT: In a case of severe fetal anemia (hemoglobin concentration, 3.0 g/dL) repeated intrauterine transfusions were required. The strongly positive direct antiglobulin test (DAT) of the fetal red blood cells led to the diagnosis of hemolytic disease...
March 7, 2017: Transfusion
https://www.readbyqxmd.com/read/28267090/a-rare-cause-of-neonatal-hemolytic-anemia-glutathione-synthetase-deficiency
#14
Pembe Soylu Ustkoyuncu, Fatma Türkan Mutlu, Aslihan Kiraz, Zuhal Tag Balkis, Sibel Yel
BACKGROUND: Isolated hemolysis or hemolytic anemia and 5-oxoprolinuria are 2 distinct medical conditions in the clinical spectrum associated with glutathione synthetase deficiency. CLINICAL OBSERVATION: A 1-day-old female baby presented with anemia and respiratory distress. Her hemoglobin level was 9.5 g/dL and the total serum bilirubin level was 5.6 mg/dL. Metabolic acidosis was detected in her blood gas analysis. Metabolic acidosis recurred despite treatment and further investigation was required...
March 6, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28267085/hemolytic-anemia-due-to-right-ventricular-to-pulmonary-artery-conduit-stenosis
#15
Sudha Rao, Julie A Creaden, Shunyou Gong, Cynthia Rigsby, John M Costello
Hemolytic anemia is a well-recognized complication in patients with left-sided mechanical heart valves. It is rare to see hemolysis with a bioprosthetic valve in the right ventricular outflow tract. We report a 4-year-old-girl with history of truncus arteriosus status-post repair who developed hemolytic anemia as a result of a calcified and obstructed bioprosthetic right ventricular to pulmonary artery-valved conduit. The hemolysis was alleviated by replacing the obstructed conduit with a larger valved conduit...
March 6, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28266038/using-direct-antiglobulin-test-results-to-reduce-unnecessary-cold-agglutinin-testing
#16
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/28265287/pernicious-anemia-associated-cobalamin-deficiency-and-thrombotic-microangiopathy-case-report-and-review-of-the-literature
#17
Farhanah Yousaf, Bruce Spinowitz, Chaim Charytan, Marilyn Galler
A 43-year-old Hispanic male without significant previous medical history was brought to emergency department for syncope following a blood draw to investigate a 40 lbs weight loss during the past 6 months associated with decreased appetite and progressive fatigue. The patient also reported a 1-month history of jaundice. On examination, he was hemodynamically stable and afebrile with pallor and diffuse jaundice but without skin rash or palpable purpura. Normal sensations and power in all extremities were evident on neurological exam...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28261698/pediatric-cholecystectomy-for-symptomatic-gallstones-unrelated-to-hematologic-disorder
#18
Sang Gyun Suh, Yoo-Shin Choi, Kwi-Won Park, Seung Eun Lee
BACKGROUNDS/AIMS: Gallstones are being increasingly diagnosed in pediatric patients. The purpose of this study was to determine characteristics of pediatric patients who underwent cholecystectomy because of symptomatic gallstone disease unrelated to hemolytic disorder. METHODS: We reviewed cases of pediatric patients (under 18 years old) who underwent cholecystectomy between May 2005 and December 2015. RESULTS: A total 20 pediatric patients (under 18 years old) underwent cholecystectomy during the study period...
November 2016: Ann Hepatobiliary Pancreat Surg
https://www.readbyqxmd.com/read/28255478/myelodysplastic-syndrome-clinically-presenting-with-the-classic-ttp-pentad
#19
Santiago Fabián Moscoso Martínez, Evelyn Carolina Polanco Jácome, Elizabeth Guevara, Vijay Mattoo
The clinical presentation of myelodysplastic syndrome (MDS) is not specific. Many patients can be asymptomatic and can be detected only due to an abnormal complete blood cell count (CBC) on routine exam or for other reasons while others can be symptomatic as a consequence of underlying cytopenias. Thrombotic thrombocytopenic purpura (TTP) usually is suspected under the evidence of microangiopathic hemolytic anemia (MAHA) and thrombocytopenia and because it is a life-threatening condition (medical emergency) immediate initiation of plasmapheresis could be life-saving...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28254202/predictors-of-granulomatous-lymphocytic-interstitial-lung-disease-in-common-variable-immunodeficiency
#20
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
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