China
Africa
Explore chapters and articles related to this topic
Paediatric and neonatal transfusions
Published in Jennifer Duguid, Lawrence Tim Goodnough, Michael J. Desmond, Transfusion Medicine in Practice, 2020
There are a variety of rare constitutional bone marrow failure syndromes that may present in children with anaemia or pancytopenia. The treatment of choice for idiopathic aplastic anaemia is bone marrow transplantation. As soon as this diagnosis has been made, a search should begin for an HLA-compatible donor, preferably a sibling. Transfusion of red cells and other products should be minimized to prevent HLA sensitization, although this is much less likely if all the cellular products are leukodepleted. Donors should be cytomegalovirus (CMV)-negative. Children for whom a marrow donor cannot be found will be transfusion-dependent.
The Hematologic System and its Disorders
Published in Walter F. Stanaszek, Mary J. Stanaszek, Robert J. Holt, Steven Strauss, Understanding Medical Terms, 2020
Walter F. Stanaszek, Mary J. Stanaszek, Robert J. Holt, Steven Strauss
White blood cells may also increase or decrease in number. Neutropenia is a reduction in the number of neutrophils in the blood. It occurs in a wide variety of diseases, including certain hereditary defects, aplastic anemia, bone marrow tumors, and acute leukemias. Granulocytopenia is a more general term describing a reduction in the number of granulocytes (leukocytes), including neutrophils. Agranulocytosis applies to diminished or absent granulocyte production, essentially the same disorder. Whichever term applies, granulocytopenic conditions all result in increased susceptibility to bacterial infection and mucous membrane ulcerations,: usually in relation to the degree of the deficiency.
Unexplained Fever In Hematologic Disorders Section 1. Benign Hematologic Disorders
Published in Benedict Isaac, Serge Kernbaum, Michael Burke, Unexplained Fever, 2019
A distinct form of severe anemia, usually normocytic or mildly macrocytic, is aplastic anemia,51 due to bone marrow failure. The peripheral blood usually shows anemia associated with leukopenia and thrombocytopenia (pancytopenia). The reticulocyte count is low. The bone marrow biopsy will reveal absent or reduced cellularity. The serum iron is elevated. The clinical manifestations vary with the severity of the pancytopenia. Bleeding and infection are the most common complications. Infection is usually associated with fever, but local signs of inflammation may be absent. The differential diagnosis is important because pancytopenia may occur in a variety of disorders, potentially evolving with fever, including lymphoma, pre-leukemia, metastatic carcinoma to the marrow, granulomatous diseases, parenchymal liver diseases with hypersplenism, and lipidoses. Two other diseases, usually febrile, SLE and tuberculosis due to atypical mycobacteria, may be associated with pan-yctopenia; some viral diseases, especially hepatitis and infectious mononucleosis, have also been implicated. The main causes of aplastic anemia are ionizing irradiation and chemical agents. The latter include chloramphenicol, phenylbutazone, hydantoin, sulfa drugs, phe-nothiazines, quinacrune, colchicine, sulfonylureas, gold compounds, benzene, insecticides, organic arsenicals, antineoplastic, and immunosuppressive drugs.
Decreased bone marrow regulatory innate lymphoid cells show a distinctive miRNA profiling in aplastic anemia
Published in Hematology, 2021
Yingmei Li, Dingming Wan, Rong Guo, Fang Wang, Lijie Han, Danfeng Zhang, Haizhou Xing, Weijie Cao, Yu Liu, Xinsheng Xie, Jifeng Yu, Zhongxing Jiang
Bone marrow samples from 30 patients with AA were obtained with informed consent from patients and/or their legal guardians prior to the treatment at the First Affiliated Hospital of Zhengzhou University from September 2018 to December 2019. Protocols were approved by Ethic Committee of The First Affiliated Hospital of Zhengzhou University, Henan, China, and based on the ethical principles of the Helsinki Declaration. Diagnosis was done based on the guidelines for the diagnosis and management of adult aplastic anemia [10]. All clinical data regarding patients are summarized in Table 1. Bone marrow samples from 30 HD (16 males and 14 females) with the age of 20- to 55-year-old were obtained with informed consent from stem cell transplantation donors, who passed the physical and laboratory testing, as the control group and tested side by side with AA patient samples.
Eltrombopag, oral immunosuppressant and androgen combination therapy in twelve patients with refractory severe aplastic anemia
Published in Hematology, 2020
Qingyan Gao, Li Zhang, Xin Zhao, Yangmin Zhu, Guangxin Peng, Yang Li, Yuan Li, Jianping Li, Lin Song, Lei Ye, Huihui Fan, Kang Zhou, Wenrui Yang, Yang Yang, Liping Jing, Fengkui Zhang
Acquired aplastic anemia is an immune-mediated bone marrow failure syndrome [1] that is mainly characterized by hypocellular marrow and a reduction of whole blood cells. The abnormal activation and hyperfunction of T lymphocytes is believed to cause hematopoietic stem and progenitor cell destruction, which was inferred mainly from effective immunosuppressive therapies for the disease [1,2]. Successful management of severe aplastic anemia (SAA) relies on adequate hematopoietic stem cells and effective interruption of abnormal immunity. Immunosuppressive therapy (IST) with antithymocyte globulin (ATG) and cyclosporine A (CsA) is the first-line choice for patients without a matched sibling donor, elderly patients, and patients who are ineligible for hematopoietic stem cell transplantation (HSCT) [2–5] and has a response rate between 50–70% [3,6]. However, 60% patients are still in need of further treatment after frontline standard IST [7].
Developing role of eltrombopag in the treatment of aplastic anemia
Published in Expert Opinion on Orphan Drugs, 2018
For many decades, the strategy to improve outcomes in aplastic anemia has been to increase the potency or introduce different immunomodulatory agents that were synergistic and/or complementary to h-ATG/CsA regarding mechanism with minimal overlapping toxicity. For the most part, these efforts were unsuccessful as h-ATG/CsA remained the standard regimen given the better risk:benefit profile [4,5]. Some regimens appeared to have activity like h-ATG/CsA but were prohibitively toxic, other regimens did not add to what was obtained with h-ATG/CsA alone, while other regimens were unexpectedly inferior [4,41]. Thus, it appeared that increasing lymphocyte depletion and/or modulating the immune response would not yield better outcomes in SAA and argued for a different strategic approach in ameliorating hematopoiesis.