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JOURNAL ARTICLE
(no author information available yet)
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare clonal disease with abnormal hematopoietic stem cells that causes intravascular hemolytic anemia, thrombosis, and peripheral blood cytopenia. It has a chronic progressive course and can be fatal in severe cases if not treated aggressively. Complement inhibitors are the first-line recommended treatment for hemolysis-related symptoms of PNH. With the rapid development of new complement inhibitors, it is critical to quickly screen and confirm the diagnosis, identify patients with complement inhibitor indications, and monitor breakthrough hemolysis and extravascular hemolysis during complement inhibitor therapy...
February 14, 2024: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
#2
Yasser Abouelkheer, Luisa Ladel, Daniel Boxer, Seaf Shafique
Hereditary hemolytic anemia associated with pyruvate kinase deficiency is a rare hematological disorder that affects the glycolic pathway within red blood cells. The standard of care includes splenectomy, transfusions, and hematopoietic stem cell transplantation. However, these treatments can lead to common iatrogenic side effects such as infections, surgical complications, and iron overload. The novel drug therapy Mitapivat has shown promising results in terms of both efficacy and safety, but it can cause rare side effects such as fractures...
March 2024: Curēus
#3
Shun Yasuda, Miho Ono-Okutsu, Toma Fukuda, Hyo Kyozuka, Keiya Fujimori
Hemolytic disease is a common cause of fetal morbidity and mortality. The anti-M blood cell alloantibodies are one of the most severe causes of fetal anemia and intrauterine death. Since no standard treatment method has been established for pregnant women, the management of this pathology is through conventional methods used for treating Rh blood-type alloimmunization. For the first time, we report a unique case wherein a pregnant woman who had intrauterine fetal death in two previous pregnancies with very low titers of anti-M antibodies had negative effects during very early pregnancy, which were successfully managed in her third pregnancy with a novel protocol...
April 2024: American Journal of Perinatology Reports
#4
Tanja Fetter, Simon Fietz, Maya Bertlich, Christine Braegelmann, Luka de Vos-Hillebrand, Joerg Wenzel, Annkristin Heine, Jennifer Landsberg, Philipp Jansen
INTRODUCTION: Over the past decade, immune checkpoint inhibitors such as antibodies against cytotoxicity T-lymphocyte-associated protein 4 (CTLA-4) and programmed cell death protein 1 (PD-1) have become an important armamentarium against a broad spectrum of malignancies. However, these specific inhibitors can cause adverse autoimmune reactions by impairing self-tolerance. Hematologic side effects of immune checkpoint inhibitors, including autoimmune hemolytic anemia (AIHA), are rare but can be life-threatening...
2024: Frontiers in Immunology
#5
JOURNAL ARTICLE
Johanne Kodal Breinholt, Andreas Glenthøj, Mustafa Vakur Bor
Hemoglobin (Hb) Volga is a rare, unstable β-chain hemoglobin variant (β27 Ala→Asp), causing chronic hemolytic anemia. This study presents two members of a Danish family, splenectomized due to Hb Volga at and with multiple thrombotic events. The proband was diagnosed with Hb Volga 9 years old and splenectomy was performed as a part of treatment. Throughout his life, he experienced multiple superficial thrombophlebitis, two episodes of distal deep venous thrombosis (DVT) on lower extremities (age 32 and 33) and a transient ischemic attack (TIA) presented as amaurosis fugax (age 51)...
April 2, 2024: Hemoglobin
#6
JOURNAL ARTICLE
Yoshifumi Ubara, Shigekazu Kurihara, Yoshiki Tsuchiya, Yuki Oba, Daisuke Ikuma, Hiroki Mizuno, Masayuki Yamanouchi, Tatsuya Suwabe, Atsuko Imase, Nobumitsu Shibata, Kei Kono, Keiichi Kinowaki, Kenichi Ohashi, Kentaro Ogata, Naoki Sawa
A 28-year-old woman with a 5-year history of untreated hypertension was admitted for respiratory distress, hemoptysis, and retinopathy. Computed tomography showed diffuse plaques in both lung fields. Acute kidney injury, hemolytic anemia, and thrombocytopenia were noted. Kidney biopsy showed thrombosis with fibrinoid necrosis and edematous intimal thickening and luminal narrowing of the small renal artery, indicating thrombotic microangiopathy; the majority of glomeruli were collapsed. After 8 weeks of treatment with antihypertensive drugs, serum creatinine decreased to 1...
March 28, 2024: CEN Case Reports
#7
JOURNAL ARTICLE
Mohamed Hbibi, Mounira El Alaoui El Hanafi, Zakaria Kasmi, Hind Ouair, Sarra Benmiloud, Fatima Ailal, Moustapha Hida, Ahmed Aziz Bousfiha
Autoimmune cytopenias are defined by autoantibodies' immune destruction of one or more blood elements. Most often it is autoimmune hemolytic anemia or immune thrombocytopenia or both that define Evans syndrome. It may be secondary to infection or to underlying pathology such as systemic autoimmune disease or primary immunodeficiency, especially when it becomes chronic over several years. Primary Immunodeficiencies or inborn errors of immunity (IEI) are no longer defined solely by infections: autoimmunity is part of the clinical features of several of these diseases...
January 5, 2024: La Tunisie Médicale
#8
Tsuyoshi Hirata, Naoko Kubota, Kazuaki Fukushima, Erika Takami, Tsuyoshi Kato, Tomomi Okamoto
An 80-year-old man presented in December with the main complaint of jaundice. Blood tests revealed hemolytic anemia and renal dysfunction. Positive syphilis serology results led to a diagnosis of untreated latent syphilis. A positive direct Coombs test led to a diagnosis of autoimmune hemolytic anemia (AIHA). Antibiotics were started for the syphilis, with improvement in the anemia and renal dysfunction observed. However, paroxysmal intravascular hemolysis occurred after his discharge. Based on a positive Donath-Landsteiner (D-L) test, paroxysmal cold hemoglobinuria (PCH) diagnosis was made...
March 2024: Oxford Medical Case Reports
#9
JOURNAL ARTICLE
Vanda P Peixoto, Cristina Prudêncio, Mónica Vieira, Sérgio F Sousa
Complement C5 is the target of the monoclonal antibody eculizumab, used in complement dysregulating disorders, like the rare disease Paroxysmal Nocturnal Hemoglobinuria (PNH). PNH is an acquired hematopoietic stem cell condition characterized by aberrant destruction of erythrocytes, chronic hemolytic anemia, and thromboembolism propensity. C5 is a protein component of the complement system which is part of the immune system of the body and plays a prominent role in the destruction of red blood cells, misidentifying them as a threat...
March 26, 2024: Journal of Biomolecular Structure & Dynamics
#10
Esmirna Perez, Nehemias Guevara, Jordan Smith, Ricardo Velasquez
Altered mental status (AMS) is a common condition encountered in daily practice. Finding the cause is essential for treatment, but sometimes this may be challenging. Spontaneous coronary artery dissection (SCAD) is frequently underdiagnosed and is a potentially fatal cause of acute coronary syndrome. Clinical presentation depends on the extent of SCAD, ranging from unstable angina to sudden death. AMS has not been reported with this condition, but it may be possible in hypoperfusion states. Thrombotic thrombocytopenic purpura (TTP) is part of the microangiopathic hemolytic anemia (MAHA) spectrum, presenting with AMS as the cardinal symptom...
February 2024: Curēus
#11
REVIEW
Bora Gülhan, Fatih Özaltın, Kibriya Fidan, Zeynep Birsin Özçakar, Oğuz Söylemezoğlu
Classical clinical triad of hemolytic uremic syndrome (HUS) is microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury associated with endothelial cell injury. Several situations, including infections, medications, malignancies, and transplantation can trigger endothelial damage. On the HUS spectrum, atypical hemolytic uremic syndrome (aHUS) deserves special attention in pediatric patients, as it can cause endstage kidney disease and mortality. A dysfunction in the alternative complement pathway, either acquired or genetic, has been shown to be the main underlying cause...
2024: Turkish Journal of Pediatrics
#12
JOURNAL ARTICLE
Yuanlin Yang, Xiaoming Fei, Fang Lei, Lixia Wang, Xianqiu Yu, Yu Tang
RATIONALE: Neuroblastoma amplified sequence (NBAS)-associated disease is an autosomal recessive disorder and a broad spectrum of clinical symptoms has been reported. However, autoimmune mediated hemolytic anemia (AIHA) is rarely reported in NBAS disease. PATIENT CONCERNS: A now 21-year-old male harbors heterozygous variants of c.6840G>A and c.335 + 1G>A and was found had retarded growth, hypogammaglobulinemia, B lymphopenia, optic atrophy, horizontal nystagmus, slight splenomegaly and hepatomegaly since childhood...
March 22, 2024: Medicine (Baltimore)
#13
JOURNAL ARTICLE
Marit Jalink, Chaja F Jacobs, Jahanzaib Khwaja, Dorothea Evers, Coty Bruggeman, Bruno Fattizzo, Marc Michel, Etienne Crickx, Quentin A A Hill, Ulrich Jaeger, Arnon P Kater, Anja B U Mäkelburg, Anouk Breedijk, Peter A W Te Te Boekhorst, Marlijn P A Hoeks, Masja de de Haas, Shirley P D'Sa, Josephine M I M I Vos
Autoimmune hemolytic anemia (AIHA) is a rare autoantibody-mediated disease. For steroid and/or rituximab-refractory AIHA, there is no consensus on optimal treatment. Daratumumab, a monoclonal antibody targeting CD38, could be beneficial by suppression of CD38+ plasmacells and thus autoantibody secretion. In addition, since CD38 is also expressed by activated T-cells, daratumumab may also act via immunomodulatory effects. We evaluated efficacy and safety of daratumumab monotherapy in an international retrospective study including 19 adult patients with heavily pretreated refractory AIHA...
March 20, 2024: Blood Advances
#14
Pablo Demelo-Rodríguez, Sabela Castañeda-Pérez, Leyre Alonso-Gonzalo, Sergio Moragón-Ledesma, Francisco Galeano-Valle
Warm antibody autoimmune hemolytic anemia (WAIHA) is a rare disease that leads to the destruction of red blood cells in the reticuloendothelial system through the mediation of agglutinins (immunoglobulin G (IgG) type in most cases) that attach to the erythrocyte wall at 37 °C. The association of WAIHA and venous thromboembolism (VTE) seems to be higher than other hemolytic disorders classically associated with VTE and there is a current investigation aimed at clarifying this association and establishing some criteria to use anticoagulant treatment in patients with WAIHA...
February 2024: Curēus
#15
JOURNAL ARTICLE
Lokesh Koumar Sivanandam, H Arunkumar, Pranay Marlecha, Varsha Madamanchi, Chanchal Maheshwari, Mohammed Quader Naseer, Vivek Sanker, Tirth Dave
INTRODUCTION: This case study reports on a suicide attempt involving indoxacarb and vitamin C. Indoxacarb is a neurotoxic insecticide used in agriculture and as a flea controller in pets. Cotton, vegetables, and fruits are treated with indoxacarb, an insecticide that can be applied both indoors and outdoors. It causes skin allergies, methemoglobinemia, and hemolytic anemia. It is also attributed to allergic reactions through ingestion, inhalation, physical contact, and translaminar action...
March 17, 2024: Journal of Medical Case Reports
#16
JOURNAL ARTICLE
Cansu Yilmaz Yegit, Beril Yasa, Elmas Zeynep Ince, Tugba Sarac Sivrikoz, Asuman Coban
BACKGROUND: The objectives were to evaluate the descriptive features of newborns with a diagnosis of Rhesus (Rh) hemolytic disease, to determine the morbidity and mortality rates, to evaluate the treatment methods and the factors affecting treatment requirements and clinical outcomes during a ten-year period at a tertiary center. METHODS: Newborn infants who had a positive direct Coombs test and/or had a history of intrauterine transfusion (IUT) due to Rh hemolytic disease were included...
March 7, 2024: Pediatrics and Neonatology
#17
REVIEW
Yanping Zheng, Siyang Lin, Meihuan Chen, Liangpu Xu, Hailong Huang
In eukaryotic RNA, N6 -methyladenosine (m6 A) is a prevalent form of methylation modification. The m6 A modification process is reversible and dynamic, written by m6 A methyltransferase complex, erased by m6 A demethylase, and recognized by m6 A binding proteins. Through mediating RNA stability, decay, alternative splicing, and translation processes, m6 A modification regulates gene expression at the post-transcriptional level. Erythropoiesis is the process of hematopoietic stem cells undergoing proliferation, a series of differentiation and maturation to form red blood cells (RBCs)...
March 15, 2024: Clinical Genetics
#18
JOURNAL ARTICLE
Régis Peffault de Latour, Alexander Röth, Austin G Kulasekararaj, Bing Han, Phillip Scheinberg, Jaroslaw P Maciejewski, Yasutaka Ueda, Carlos M de Castro, Eros Di Bona, Rong Fu, Li Zhang, Morag Griffin, Saskia M C Langemeijer, Jens Panse, Hubert Schrezenmeier, Wilma Barcellini, Vitor A Q Mauad, Philippe Schafhausen, Suzanne Tavitian, Eloise Beggiato, Lee Ping Chew, Anna Gaya, Wei-Han Huang, Jun Ho Jang, Toshio Kitawaki, Abdullah Kutlar, Rosario Notaro, Vinod Pullarkat, Jörg Schubert, Louis Terriou, Michihiro Uchiyama, Lily Wong Lee Lee, Eng-Soo Yap, Flore Sicre de Fontbrune, Luana Marano, Ferras Alashkar, Shreyans Gandhi, Roochi Trikha, Chen Yang, Hui Liu, Richard J Kelly, Britta Höchsmann, Cécile Kerloeguen, Partha Banerjee, Rafael Levitch, Rakesh Kumar, Zhixin Wang, Christine Thorburn, Samopriyo Maitra, Shujie Li, Aurelie Verles, Marion Dahlke, Antonio M Risitano
BACKGROUND: Persistent hemolytic anemia and a lack of oral treatments are challenges for patients with paroxysmal nocturnal hemoglobinuria who have received anti-C5 therapy or have not received complement inhibitors. Iptacopan, a first-in-class oral factor B inhibitor, has been shown to improve hemoglobin levels in these patients. METHODS: In two phase 3 trials, we assessed iptacopan monotherapy over a 24-week period in patients with hemoglobin levels of less than 10 g per deciliter...
March 14, 2024: New England Journal of Medicine
#19
JOURNAL ARTICLE
I Reffo, M Domini, M Cevolani, G Del Fabro, D Rufolo, S Venturini, L Pinciroli, D Tonin, M Avolio, M Crapis, G Basaglia, M Balbi, G Nadalin
Clostridium perfringens can rarely cause severe systemic infections, usually from an abdominal source, associated with massive hemolysis, which is usually fatal. Hemolytic anemia and acute renal injury resulting from toxin action are critical for the development of multiple organ dysfunction syndrome (MODs), making this condition a real emergency, requiring multispecialty skills and aggressive multimodal therapies. We herein describe a case of septic shock from acute cholecystitis with massive hemolysis caused by C...
March 4, 2024: CEN Case Reports
#20
Shunya Yokota, Masashi Yuki
An 11-year-old neutered male Jack Russell Terrier was presented to Yuki Animal Hospital for regenerative anemia during the treatment of hypoadrenocorticism. A blood smear examination showed spherocytes, polychromatic erythrocytes, and erythrocyte ghosts. The direct agglutination test was positive at 37°C. The dog was then diagnosed with immune-mediated hemolytic anemia (IMHA). Although prednisolone and mycophenolate mofetil were administered, the hematocrit and reticulocyte count decreased, and nonregenerative anemia developed...
March 2024: Veterinary Clinical Pathology
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