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management of anemia

Maria Domenica Cappellini, John Porter, Raffaella Origa, Gian Luca Forni, Ersi Voskaridou, Frédéric Galactéros, Ali T Taher, Jean-Benoît Arlet, Jean-Antoine Ribeil, Maciej Garbowski, Giovanna Graziadei, Chantal Brouzes, Michaela Semeraro, Abderrahmane Laadem, Dimana Miteva, Jun Zou, Victoria Sung, Tatiana Zinger, Kenneth M Attie, Olivier Hermine
β-thalassemia, a hereditary blood disorder caused by defective synthesis of hemoglobin β globin chains, leads to ineffective erythropoiesis and chronic anemia that may require blood transfusions. Sotatercept (ACE-011) acts as a ligand trap to inhibit negative regulators of late-stage erythropoiesis in the transforming growth factor beta superfamily, correcting ineffective erythropoiesis. In this phase II, open-label, dose-finding study, 16 patients with transfusion-dependent β-thalassemia and 30 patients with non-transfusion-dependent β thalassemia were enrolled at 7 centers in 4 countries from November 2012 to November 2014...
October 18, 2018: Haematologica
Raquel Ballester-Clau, Gisela Torres Vicente, Tania Voltà-Pardo, Laura López-Barroso, Mercedes Cucala-Ramos, Josep M Reñé-Espinet, Montse Planella de Rubinat
OBJECTIVE: The aim of this study was to assess the efficacy and safety of intravenous ferric carboxymaltose (FCM) following hospitalization for acute gastrointestinal bleeding (AGIB) in the context of a restrictive transfusion strategy. PATIENTS AND METHODS: A retrospective single-center study analyzed patients with AGIB (excluding AGIB secondary to portal hypertension) administered a single FCM dose with or without blood transfusion. RESULTS: Eighty-six episodes in 84 patients were analyzed...
October 17, 2018: European Journal of Gastroenterology & Hepatology
Sandra E Kurtin, Jean A Ridgeway, Sara Tinsley
CASE STUDY A male patient aged 67 years with a 2-year history of refractory anemia and myelodysplastic syndromes (MDS) with del(5q) started lenalidomide (Revlimid) treatment as a participant in the MDS-001 trial (List et al., 2005). At the time of the study, this patient had been transfusion-dependent since 2001, and at study entry he had received a total of 12 units of red blood cells (RBCs). The patient started lenalidomide at 25 mg daily for 21 days of each 28-day cycle on April 2, 2002. (Please note that as a result of subsequent trials, the approved starting dose for lenalidomide in patients with del[5q] MDS is 10 mg...
November 2017: Journal of the Advanced Practitioner in Oncology
Valentine de A C de Castro Lima, Ana Luisa Figueira Gouvêa, Paulo Menezes, Jacqueline da F Santos, Mayra Carrijo Rochael, Fabiana Rabe Carvalho, Jorge Reis Almeida, Jocemir Ronaldo Lugon
Hemophagocytic lymphohistiocytosis (HLH) is an uncommon and life-threating condition characterized by major immune activation and massive cytokine production by mononuclear inflammatory cells, due to defects in cytotoxic lymphocyte function. It is even more unusual in renal transplant recipients, in which it is often associated with uncontrolled infection. The mortality is high in HLH and differential diagnosis with sepsis is a challenge. The approach and management depend on the underlying trigger and comorbidities...
October 11, 2018: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
Morie A Gertz
DISEASE OVERVIEW: Waldenström macroglobulinemia (WM) is a lymphoplasmacytic lymphoma with immunoglobulin M (IgM) monoclonal protein. Clinical features include anemia, thrombocytopenia, hepatosplenomegaly, lymphadenopathy, and rarely hyperviscosity. DIAGNOSIS: Presence of IgM monoclonal protein associated with ≥10% clonal lymphoplasmacytic cells in bone marrow confirms the diagnosis. The L265P mutation in MYD88 is detectable in >90% of patients and is found in the majority of IgM monoclonal gammopathy of undetermined significance patients...
October 17, 2018: American Journal of Hematology
Pedro S Argoti, Giancarlo Mari
The diagnosis and management of fetal anemia has been at the forefront of advances in the fields of fetal physiology, immunology, fetal imaging, and fetal therapy among others. Alloimmunization and parvovirus infection are the leading cause of fetal anemia in the United States. The middle cerebral artery peak systolic velocity (MCA-PSV)diagnoses fetal anemia. Its discovery is considered one of the most important achievements in fetal medicine. Accumulation of experience in recent years as well as refinement of surgical techniques have led to safer invasive procedures...
October 11, 2018: Minerva Ginecologica
John E Strobeck, Jonathan Feldschuh, Wayne L Miller
OBJECTIVES: This study performed a retrospective outcome analyses of a large cohort of mixed ejection fraction patients admitted for acute heart failure (HF), whose inpatient care was guided by individual quantitative blood volume analysis (BVA) results. BACKGROUND: Decongestion strategies in patients hospitalized for HF are based on clinical assessment of volume and have not integrated a quantitative intravascular volume metric. METHODS: Propensity score control matching analysis was performed in 245 consecutive HF admissions to a community hospital (September 2007 to April 2014; 78 ± 10 years of age; 50% with HF with reduced ejection fraction [HFrEF]; and 30% with Stage 4 chronic kidney disease)...
October 4, 2018: JACC. Heart Failure
Jean Dubuisson
Uterine leiomyomas are the most common benign tumors in women of reproductive age. Most of leiomyomas are asymptomatic. They are often found incidentally, and require neither monitoring nor treatment. For symptomatic women who wish to become pregnant, surgical myomectomy remains the conservative treatment of choice. It can be performed in various routes depending on the location and the number of leiomyomas and the experience of the surgeon. A minimally invasive procedure should always be the preferred option so as to improve woman satisfaction and to decrease perioperative morbidity...
October 11, 2018: Journal of Gynecology Obstetrics and Human Reproduction
Florence Thierry, Marisa F Ferreira, Gavin K Paterson, Tiziana Liuti, Jorge Del-Pozo
Gastric pneumatosis is an imaging finding defined as the presence of gas foci in the gastric wall. In humans, this imaging feature can result from one of two separate clinical entities: life-threatening emphysematous gastritis or clinically benign gastric emphysema. This retrospective case series study describes the clinical and imaging features in five animals diagnosed with spontaneous gastric pneumatosis without gastric dilatation-volvulus. Three canine and two feline cases of spontaneous gastric pneumatosis were identified on radiographic and ultrasonographic examinations...
October 11, 2018: Veterinary Radiology & Ultrasound
Hui Zhao, Baojun Yang, Haixia Li, Yun Xu, Limin Feng
STUDY OBJECTIVE: To evaluate the feasibility, effectiveness, and reproductive outcome of hysteroscopic management using the Hysteroscopy Endo Operative system (HEOS) in patients with diffuse uterine leiomyomatosis (DUL). DESIGN: Retrospective study (Canadian Task Force Classification III). SETTING: Beijing Tiantan Hospital, Capital Medical University, Beijing, China. PATIENTS: Eight women of reproductive age suffering from menorrhagia and anemia or infertility were diagnosed with DUL by ultrasonography and hysteroscopy...
October 8, 2018: Journal of Minimally Invasive Gynecology
Takayuki Ikezoe
The clinical features of transplant-associated thrombotic microangiopathy (TA-TMA) include microangiopathic hemolytic anemia, consumptive thrombocytopenia, and organ dysfunction caused by thrombi in microvessels. The pathogenesis of TA-TMA is based on vascular endothelial insults caused by various factors, including chemotherapy and total-body irradiation used for transplant pre-conditioning, calcineurin inhibitors, cytokines release associated with infection, and complement factors. The incidence of TA-TMA is approximately 15-30% among allogeneic transplant patients, and the mortality rate reaches 100% in severe cases with multi-organ dysfunction...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Hiroshi Moritake
Recently, the modern technique of comprehensive genomic analysis has identified both somatic mutations originating from tumor cells and germline mutations as causative genes of inherited familial leukemias among which Fanconi anemia and Li-Fraumeni syndrome are well known. Pathogenic germline mutations occur in various pathways, affecting DNA repair, ribosome biogenesis, telomere biology, hematopoietic transcription factors, tumor suppressors, neutrophil development, and other critical cellular processes. The clinical manifestations of germline mutations present a wide phenotypic spectrum of patients displaying congenital anomalies, early-onset myelodysplastic syndrome, or no medical problems until the developing leukemia...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Hideki Makishima
Aplastic anemia (AA) is an autoimmune-mediated bone marrow failure syndrome. While AA is not a malignant disease, clonal hematopoiesis is commonly detected via next-generation sequencing and single nucleotide polymorphism (SNP) array. Clonal hematopoiesis in AA has been confirmed by the detection of classic X chromosome skewing, PNH clones, UPD6p, and various mutations. The most frequent genetic events in AA are UPD6p and somatic mutations in BCOR/BCORL1, PIGA, DNMT3A, and ASXL1. While some mutations are common between patients with AA and healthy elderly donors, UPD6p and PIGA mutations are specific to clonal cells in AA, which need to manage their highly autoimmune extrinsic environment...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Olivia Allen, Ahmed Edhi, Adam Hafeez, Alexandra Halalau
Hepatitis A is a common viral infection with a benign course but in rare cases can progress to acute liver failure. It usually presents with abdominal pain, nausea, vomiting, diarrhea, jaundice, anorexia, or asymptomatically, but it can also present atypically with relapsing hepatitis and prolonged cholestasis. In addition, extrahepatic manifestations have been reported, including urticarial and maculopapular rash, acute kidney injury, autoimmune hemolytic anemia, aplastic anemia, acute pancreatitis, mononeuritis, reactive arthritis, glomerulonephritis, cryoglobulinemia, Guillain-Barre syndrome, and pleural or pericardial effusion...
2018: Case Reports in Medicine
Sophie Gatineau-Sailliant, Karine Turcotte, Marie-Claude Quintal, Sophie Turpin, Josette Champagne, Tony Petrella, Mathieu Roussy, Sonia Cellot, Dorothée Bouron-Dal Soglio
Gray zone lymphoma is an aggressive disease for which appropriate management is still debated. We report a 15-year-old girl with a cervical mass, an enlarged ipsilateral tonsil, and anemia. Both sites showed hypermetabolism on F18-FG positron emission tomography/CT. Surgical resection was diagnostic of Epstein-Barr virus-negative gray zone lymphoma cervical and tonsillar involvement. No abnormality was found in cytogenetic analysis on tumor cells. However, exome sequencing in peripheral blood DNA revealed a germline mutation in TP53...
October 5, 2018: Journal of Pediatric Hematology/oncology
Maria Stefania Lepanto, Luigi Rosa, Antimo Cutone, Maria Pia Conte, Rosalba Paesano, Piera Valenti
The discovery of the ferroportin-hepcidin complex has led to a critical review on the treatment of anemia and anemia of inflammation (AI). Ferroportin, the only known mammalian iron exporter from cells to blood, is negatively regulated by hepcidin, a hormone peptide able to bind to ferroportin, leading to its degradation. Therefore, new efficient therapeutic interventions acting on hepcidin and ferroportin are imperative to manage anemia and AI. Bovine milk derivative lactoferrin (bLf), a glycoprotein able to chelate two ferric ions per molecule, is emerging as a natural anti-inflammatory substance able to modulate hepcidin and ferroportin synthesis through the down-regulation of interleukin-6 (IL-6)...
2018: Frontiers in Immunology
Rupesh Raina, Vinod Krishnappa, Taryn Blaha, Taylor Kann, William Hein, Linda Burk, Arvind Bagga
Atypical hemolytic uremic syndrome (aHUS), a rare variant of thrombotic microangiopathy, is characterized by microangiopathic hemolytic anemia, thrombocytopenia and renal impairment. The condition is associated with poor clinical outcomes with high morbidity and mortality. Atypical HUS predominantly affects the kidneys but has the potential to cause multi-organ system dysfunction. This uncommon disorder is caused by a genetic abnormality in the complement alternative pathway resulting in over-activation of the complement system and formation of microvascular thrombi...
October 8, 2018: Therapeutic Apheresis and Dialysis
Clelia Madeddu, Giulia Gramignano, Giorgio Astara, Roberto Demontis, Elisabetta Sanna, Vinicio Atzeni, Antonio Macciò
Cancer-related anemia (CRA) is a common sign occurring in more than 30% of cancer patients at diagnosis before the initiation of antineoplastic therapy. CRA has a relevant influence on survival, disease progression, treatment efficacy, and the patients' quality of life. It is more often detected in patients with advanced stage disease, where it represents a specific symptom of the neoplastic disease, as a consequence of chronic inflammation. In fact, CRA is characterized by biological and hematologic features that resemble those described in anemia associated to chronic inflammatory disease...
2018: Frontiers in Physiology
Yi Ji, Siyuan Chen, Lizhi Li, Kaiying Yang, Chunchao Xia, Li Li, Gang Yang, Feiteng Kong, Guoyan Lu, Xingtao Liu
PURPOSE: We sought to characterize the clinical features and management of patients diagnosed as Kaposiform hemangioendothelioma (KHE) without cutaneous involvement. METHODS: The electronic patient chats at six Triple A hospitals in China were searched to find all patient diagnoses with KHE without cutaneous involvement. RESULTS: Of 30 patients (mean age at diagnosis, 55.6 months), 17 (56.7%) were male. Fourteen (46.7%) patients were associated with Kasabach-Merritt phenomenon (KMP)...
October 6, 2018: Journal of Cancer Research and Clinical Oncology
Eveline Thobias Konje, Moke Tito Nyambita Magoma, Jennifer Hatfield, Susan Kuhn, Reginald S Sauve, Deborah Margret Dewey
BACKGROUND: Despite the significant benefits of early detection and management of pregnancy related complications during antenatal care (ANC) visits, not all pregnant women in Tanzania initiate ANC in a timely manner. The primary objectives of this research study in rural communities of Geita district, Northwest Tanzania were: 1) to conduct a population-based study that examined the utilization and availability of ANC services; and 2) to explore the challenges faced by women who visited ANC clinics and barriers to utilization of ANC among pregnant women...
October 5, 2018: BMC Pregnancy and Childbirth
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