18,958 results match your criteria Aplastic Anemia


Cyclin-Dependent Kinase 4/6 Inhibitor (Palbociclib) Induced Aplastic Anemia in a Patient with Metastatic Breast Cancer.

Case Rep Hematol 2018 13;2018:9249506. Epub 2018 Dec 13.

Section of Hematology and Medical Oncology, Department of Medicine, Boston Medical Center, Boston, Massachusetts, USA.

Breast cancer is the most common cancer diagnosed in women worldwide. Over the years, breast cancer treatment has undergone revolutionary changes especially for women with hormone receptor positive metastatic disease. As a result, women are living longer with their disease, particularly in developed countries. Read More

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http://dx.doi.org/10.1155/2018/9249506DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6311830PMC
December 2018

[Thinking on Treatment of Severe Aplastic Anemia by Detoxication, Blood Activating, and Yin Nourishing Method].

Zhongguo Zhong Xi Yi Jie He Za Zhi 2016 Sep;36(9):1132-1134

Chinese medicine therapy has advantages in treating aplastic anemia (AA) in depart- ments of Chinese medicine (CM) blood diseases. But there is no unified syndrome typing standard of CM for severe AA. Heat-toxin induced yin deficiency syndrome is one of severe AA syndrome types com- monly seen in authors' long-term clinical practice. Read More

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September 2016
1 Read

Consider Allogeneic Bone Marrow Transplantation for Older, Fit Patients with Aplastic Anemia.

Authors:
George E Georges

Biol Blood Marrow Transplant 2019 Jan 11. Epub 2019 Jan 11.

Associate Member, Fred Hutchison Cancer Research Center, Seattle, Washington; Associate Professor, University of Washington, Seattle, Washington. Electronic address:

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http://dx.doi.org/10.1016/j.bbmt.2019.01.015DOI Listing
January 2019

Comparable Outcome with a Faster Engraftment of Optimized Haploidentical Hematopoietic Stem Cell Transplantation Compared to Transplantations from Other Donor Types in Pediatric Acquired Aplastic Anemia.

Biol Blood Marrow Transplant 2019 Jan 10. Epub 2019 Jan 10.

Department of Pediatrics, University of Ulsan College of Medicine, Asan Medical Center Children's Hospital, Seoul, Korea.

Haploidentical family donors have been used as an alternative source in hematopoietic cell transplantation for patients with severe aplastic anemia. We evaluated and compared the outcomes of transplantation in pediatric-acquired severe aplastic anemia based on donor type. Sixty-seven patients who underwent transplantation between 1998 and 2017 were included. Read More

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http://dx.doi.org/10.1016/j.bbmt.2019.01.010DOI Listing
January 2019

The Progress of Traditional Chinese Medicine in the Treatment of Aplastic Anemia.

J Transl Int Med 2018 Dec 31;6(4):159-164. Epub 2018 Dec 31.

Department of Hematology, First Hospital Affiliated to Zhejiang Chinese Medical University, Hangzhou 310006, Zhejiang Province, China.

Aplastic anemia (AA) is a common hematologic disease that is characterized by hematopoietic failure of the bone marrow and pancytopenia of the peripheral blood, which can be divided into severe and non-severe aplastic ane-mia, or acute and chronic aplastic anemia according to the severity of the disease and the progress of the clinical course. During the past years, the advantages of Traditional Chinese Medicine (TCM) on the treatment of AA have been well clarified and its theory system has been improved as well. This review is mainly on representing the pathogenesis, therapeutic principle and method, research progression, and advantages of TCM on AA. Read More

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http://dx.doi.org/10.2478/jtim-2018-0031DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6326026PMC
December 2018

Fatigue, symptom burden, and health-related quality of life in patients with myelodysplastic syndrome, aplastic anemia, and paroxysmal nocturnal hemoglobinuria.

Cancer Med 2019 Jan 11. Epub 2019 Jan 11.

Department of Leukemia, The University of Texas MD Anderson Cancer Center, Houston, Texas.

Background: Fatigue is distressing and affects quality of life (QoL) among patients with myelodysplastic syndrome (MDS), aplastic anemia (AA), and paroxysmal nocturnal hemoglobinuria (PNH). Limited data exist on the impact of fatigue, QoL, and related symptoms in these patients.

Objective: Prospectively assess fatigue (functional assessment of cancer therapy-anemia [FACT-An]); QoL (FACT-An subscales); pain (brief pain inventory); and depression, anxiety, and stress (depression anxiety stress scale-21) and strategies used to manage these symptoms in patients with MDS, AA, and PNH. Read More

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http://doi.wiley.com/10.1002/cam4.1953
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http://dx.doi.org/10.1002/cam4.1953DOI Listing
January 2019
2 Reads

Immunosuppressive therapy with rabbit antithymocyte globulin therapy for acquired aplastic anemia: a multi-institutional retrospective study in Japanese adult patients.

Int J Hematol 2019 Jan 9. Epub 2019 Jan 9.

Division of Hematology and Oncology, Department of Medicine, Kyoto Prefectural University of Medicine, 465 Kajii-cho, Kamigyo-ku, Kyoto, 602-8566, Japan.

We retrospectively analyzed efficacy and safety of therapy with rabbit antithymocyte globulin (rATG) in combination with cyclosporine A (CsA) in 30 Japanese adult patients with acquired aplastic anemia (AA) in the Kyoto Clinical Hematology Study Group. The median observation period was 31 months and the median age of the patients was 54 years. The objective response rates (ORRs) to rATG plus CsA increased over time until 18 months after the start of treatment; the rate of achievement of better than partial response at 18 months was 66. Read More

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http://link.springer.com/10.1007/s12185-018-02583-w
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http://dx.doi.org/10.1007/s12185-018-02583-wDOI Listing
January 2019
2 Reads

Mean platelet component and mean platelet volume as useful screening markers for myelodysplastic syndrome.

Health Sci Rep 2018 May 2;1(5):e50. Epub 2018 May 2.

Department of Central Clinical Laboratory Osaka Medical College Hospital Takatsuki Osaka Japan.

Background: Hematologic disorders, including myelodysplastic syndrome (MDS), are difficult to identify in routine hematologic examinations using automated hematology analyzers. However, the practical uses of mean platelet component and mean platelet volume (MPV) measured by these analyzers as screening markers for MDS, remain unclear.

Methods: Mean platelet component and MPV values were measured in the peripheral blood of patients with MDS, aplastic anemia, idiopathic thrombocytopenic purpura, myeloproliferative neoplasms, and in healthy controls using an automated hematologic analyzer. Read More

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http://dx.doi.org/10.1002/hsr2.50DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6266360PMC

Pancytopenia of Unknown Cause in Adult Patients Admitted to a Tertiary Hospital in Ethiopia: Case series.

Ethiop J Health Sci 2018 Jul;28(4):375-382

Department of Internal Medicine, Jimma University, Jimma.

Background: Over the past few years, we have witnessed a dramatic increase in the number of patients presenting with severe pancytopenia to Jimma University Hospital. We now present sociodemographic and clinical characteristics of adult patients admitted with pancytopenia of unknown cause to Jimma University Hospital during the period of March 2015 to June 2016. Complete blood count and other diagnostic tests were done for all patients to uncover underlying causes. Read More

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http://dx.doi.org/10.4314/ejhs.v28i4.3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6308738PMC
July 2018
1 Read

Clinical features of dyskeratosis congenita in mainland China: case reports and literature review.

Int J Hematol 2019 Jan 3. Epub 2019 Jan 3.

Department of Pediatrics, Tongji Hospital, Tongji University School of Medicine, Xincun Road 389, Shanghai, 200065, People's Republic of China.

Dyskeratosis congenita (DC) is a rare-inherited bone marrow failure syndrome associated with multi-system disorder. To summarize the clinical features, epidemiology, and treatment of DC in mainland China, we retrospectively reviewed the medical records of two patients diagnosed with DC at our hospital and published reports on other DC patients in mainland China. The clinical features of 82 DC patients were summarized. Read More

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http://dx.doi.org/10.1007/s12185-018-02582-xDOI Listing
January 2019
1.679 Impact Factor

High frequency of acquired aplastic anaemia in Tanzania.

Am J Hematol 2018 Dec 27. Epub 2018 Dec 27.

Department of Haematology and Blood Transfusion, Muhimbili National Hospital and Muhimbili University of Health and Allied Sciences (MUHAS), Dar-es-Salaam, Tanzania.

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http://dx.doi.org/10.1002/ajh.25388DOI Listing
December 2018

Kidney transplantation for treatment of end-stage kidney disease after haematopoietic stem cell transplantation: case series and literature review.

Clin Exp Nephrol 2018 Dec 24. Epub 2018 Dec 24.

Department of Urology, Tokyo Women's Medical University, 8-1 Kawata-machi, Shinjuku-ku, Tokyo, 162-8666, Japan.

Background: The safety of kidney transplantation (KT) for end-stage kidney disease (ESKD) after haematopoietic stem cell transplantation (HSCT) for haematological disease has not been investigated thoroughly.

Methods: In this retrospective multicentre study, we investigated the clinical courses of six ESKD patients that received KT after HSCT for various haematological diseases. Data for six such patients were obtained from three institutions in our consortium. Read More

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http://link.springer.com/10.1007/s10157-018-1672-1
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http://dx.doi.org/10.1007/s10157-018-1672-1DOI Listing
December 2018
3 Reads
1.708 Impact Factor

Late Mortality after Allogeneic Bone Marrow Transplantation in Childhood for Bone Marrow Failure Syndromes and Severe Aplastic Anemia.

Biol Blood Marrow Transplant 2018 Dec 20. Epub 2018 Dec 20.

Institute for Cancer Outcomes and Survivorship, University of Alabama at Birmingham, Birmingham, Alabama; Division of Hematology and Oncology, Department of Pediatrics, University of Alabama at Birmingham, Birmingham, Alabama. Electronic address:

Children with bone marrow failure syndromes and severe aplastic anemia (SAA) are treated with allogeneic blood or marrow transplantation (BMT). However, there is a paucity of studies examining late mortality risk after allogeneic BMT performed in childhood for bone marrow failure syndromes and SAA and evaluating how this risk differs between these diseases. We investigated cause-specific late mortality in 2-year survivors of allogeneic BMT for bone marrow failure syndromes and SAA performed before age 22 years between 1974 and 2010 at 2 US transplantation centers. Read More

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http://dx.doi.org/10.1016/j.bbmt.2018.12.063DOI Listing
December 2018
1 Read

Acquired and germline predisposition to bone marrow failure: Diagnostic features and clinical implications.

Semin Hematol 2019 Jan 23;56(1):69-82. Epub 2018 Jun 23.

Hematology Section, Department of Laboratory Medicine, Clinical Center, National Institutes of Health, Bethesda, 20892 MD, USA. Electronic address:

Bone marrow failure and related syndromes are rare disorders characterized by ineffective bone marrow hematopoiesis and peripheral cytopenias. Although many are associated with characteristic clinical features, recent advances have shown a more complicated picture with a spectrum of broad and overlapping phenotypes and imperfect genotype-phenotype correlations. Distinguishing acquired from inherited forms of marrow failure can be challenging, but is of crucial importance given differences in the risk of disease progression to myelodysplastic syndrome, acute myeloid leukemia, and other malignancies, as well as the potential to genetically screen relatives and select the appropriate donor if hematopoietic stem cell transplantation becomes necessary. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00371963183006
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http://dx.doi.org/10.1053/j.seminhematol.2018.05.016DOI Listing
January 2019
5 Reads

Expression of C1q in the serum of patients with non‑severe aplastic anemia, and its association with disease severity.

Mol Med Rep 2018 Dec 12. Epub 2018 Dec 12.

Department of Hematology, Tianjin Medical University General Hospital, Tianjin 300052, P.R. China.

A type of aplastic anemia (AA), non-severe aplastic anemia (NSAA) is defined as AA that does not meet the diagnostic criteria of severe aplastic anemia (SAA). Complement component 1q (C1q) has an important role in the pathogenesis of various autoimmune diseases; however, the role of C1q in the immune pathogenesis of NSAA is not clear. The current study aimed to determine whether C1q has an important role in the pathogenesis of NSAA. Read More

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http://dx.doi.org/10.3892/mmr.2018.9754DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6323203PMC
December 2018
2 Reads
1.484 Impact Factor

Pathogenesis of Acquired Aplastic Anemia and the Role of the Bone Marrow Microenvironment.

Front Oncol 2018 5;8:587. Epub 2018 Dec 5.

Division of Hematology, Department of Medicine, University Hospital Basel, Basel, Switzerland.

Aplastic anemia (AA) is characterized by bone marrow (BM) hypocellularity, resulting in peripheral cytopenias. An antigen-driven and likely auto-immune dysregulated T-cell homeostasis results in hematopoietic stem cell injury, which ultimately leads to the pathogenesis of the acquired form of this disease. Auto-immune and inflammatory processes further influence the disease course as well as response rate to therapy, mainly consisting of intensive immunosuppressive therapy and allogeneic hematopoietic cell transplantation. Read More

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https://www.frontiersin.org/article/10.3389/fonc.2018.00587/
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http://dx.doi.org/10.3389/fonc.2018.00587DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6290278PMC
December 2018
6 Reads

Benzene-induced mouse hematotoxicity is regulated by a protein phosphatase 2A complexes that stimulates transcription of cytochrome P4502E1.

J Biol Chem 2018 Dec 19. Epub 2018 Dec 19.

Department of Toxicology, Sun Yat-sen University, China.

Chronic benzene exposure is associated with hematotoxicity and the development of aplastic anemia and leukemia. However, the signaling pathways underlying benzene-induced hematotoxicity remain to be defined. Here, we investigated the role of protein phosphatase 2A (PP2A) in the regulation of benzene-induced hematotoxicity in a murine model. Read More

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http://www.jbc.org/lookup/doi/10.1074/jbc.RA118.006319
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http://dx.doi.org/10.1074/jbc.RA118.006319DOI Listing
December 2018
8 Reads

Pericardial effusion as a complication of chronic graft versus host disease.

BMJ Case Rep 2018 Dec 14;11(1). Epub 2018 Dec 14.

Internal Medicine, Banner University Medical Center, Tucson, Arizona, USA.

A 24-year-old man with previous matched unrelated donor allogenic bone marrow transplant for aplastic anaemia and chronic graft versus host disease on steroid taper presented with progressively worsening anasarca. CT revealed large pericardial effusion, while echocardiogram was concerning for early tamponade physiology. He underwent emergent pericardiocentesis with pericardial drain placement. Read More

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http://dx.doi.org/10.1136/bcr-2018-227507DOI Listing
December 2018
1 Read

Morbidity pattern and interferon gamma level in sickle cell anemia patients with autosplenectomy.

Niger J Clin Pract 2018 Dec;21(12):1615-1621

Department of Immunology, Faculty of Basic Medical Science, College of Medicine, University of Ibadan, Ibadan, Nigeria.

Introduction: In sickle cell anemia patients (SCA), the spleen suffers multiple occlusion of its microvasculature causing ischemia and subsequently autosplenectomy. Among the functions of the spleen is the production of gamma interferon (IFN-γ) which has several immunological roles. This function could be impaired in these patients. Read More

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http://dx.doi.org/10.4103/njcp.njcp_217_18DOI Listing
December 2018
1 Read

Long-term safety and efficacy of deferasirox in patients with myelodysplastic syndrome, aplastic anemia and other rare anemia in Taiwan.

Hematology 2019 Dec;24(1):247-254

k Department of Internal Medicine , Chung Shan Medical University Hospital , Taichung , Taiwan.

Objective: Patients with myelodysplastic syndromes (MDS), aplastic anemia (AA) or other rare anemia require chronic blood transfusions which can lead to iron overload and subsequent excess iron-mediated complications. Intensive iron chelation with deferasirox could remove excess iron and can alleviate these events; however, the long-term safety and efficacy in Chinese population are not clearly characterized. This study examined the long-term efficacy and safety of deferasirox in a real-world setting in Taiwan. Read More

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http://dx.doi.org/10.1080/16078454.2018.1557860DOI Listing
December 2019
4 Reads

Correction to: Structural modeling of a novel TERC variant in a patient with aplastic anemia and short telomeres.

Ann Hematol 2018 Dec 14. Epub 2018 Dec 14.

Laboratory of Medical Genetics, Bambino Gesù Children's Hospital, Rome, Italy.

The original version of this article contained a mistake in the affiliation of E. Bellacchio. Correct affiliation is presented here. Read More

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http://dx.doi.org/10.1007/s00277-018-3581-5DOI Listing
December 2018

Bone Marrow Fat and Hematopoiesis.

Front Endocrinol (Lausanne) 2018 28;9:694. Epub 2018 Nov 28.

Department of Hematology, West China Hospital, Sichuan University, Chengdu, China.

Bone marrow fat cells comprise the largest population of cells in the bone marrow cavity, a characteristic that has attracted the attention of scholars from different disciplines. The perception that bone marrow adipocytes are "inert space fillers" has been broken, and currently, bone marrow fat is unanimously considered to be the third largest fat depot, after subcutaneous fat and visceral fat. Bone marrow fat (BMF) acts as a metabolically active organ and plays an active role in energy storage, endocrine function, bone metabolism, and the bone metastasis of tumors. Read More

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http://dx.doi.org/10.3389/fendo.2018.00694DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6280186PMC
November 2018

The Hematopoietic Cell Transplant Comorbidity Index predicts survival after allogeneic transplant for non-malignant diseases.

Blood 2018 Dec 13. Epub 2018 Dec 13.

Fred Hutchinson Cancer Research Center, Seattle, WA, United States;

Despite overall improvements, mortality after allogeneic hematopoietic cell transplantation (HCT) for non-malignant diseases remains a significant problem. We evaluated whether pre-HCT conditions defined by the HCT Comorbidity Index (HCT-CI), predicts probability of post-transplant survival in these patients. Using the Center for International Blood and Marrow Transplant Research (CIBMTR) database, we identified 4,083 patients with non-malignant diseases transplanted between 2007-2014. Read More

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http://dx.doi.org/10.1182/blood-2018-09-876284DOI Listing
December 2018

Pathogenic TERT promoter variants in telomere diseases.

Genet Med 2018 Dec 7. Epub 2018 Dec 7.

Hematology Branch, National Heart, Lung, and Blood Institute (NHLBI), NIH, Bethesda, MD, USA.

Purpose: The acquisition of pathogenic variants in the TERT promoter (TERTp) region is a mechanism of tumorigenesis. In nonmalignant diseases, TERTp variants have been reported only in patients with idiopathic pulmonary fibrosis (IPF) due to germline variants in telomere biology genes.

Methods: We screened patients with a broad spectrum of telomeropathies (n = 136), their relatives (n = 52), and controls (n = 195) for TERTp variants using a customized massively parallel amplicon-based sequencing assay. Read More

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http://dx.doi.org/10.1038/s41436-018-0385-xDOI Listing
December 2018
1 Read

Multiplexed CRISPR/Cas9-mediated knockout of 19 Fanconi anemia pathway genes in zebrafish revealed their roles in growth, sexual development and fertility.

PLoS Genet 2018 Dec 12;14(12):e1007821. Epub 2018 Dec 12.

Cancer Genomics Unit, Cancer Genetics and Comparative Genomics Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland, United States of America.

Fanconi Anemia (FA) is a genomic instability syndrome resulting in aplastic anemia, developmental abnormalities, and predisposition to hematological and other solid organ malignancies. Mutations in genes that encode proteins of the FA pathway fail to orchestrate the repair of DNA damage caused by DNA interstrand crosslinks. Zebrafish harbor homologs for nearly all known FA genes. Read More

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http://dx.plos.org/10.1371/journal.pgen.1007821
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http://dx.doi.org/10.1371/journal.pgen.1007821DOI Listing
December 2018
3 Reads

Autologous cord blood transplantation in children with acquired severe aplastic anemia.

Pediatr Transplant 2018 Dec 9:e13325. Epub 2018 Dec 9.

BeiJing Children's Hospital, Capital Medical University, Beijing, China.

Objective: To investigate the efficacy of ACBT for acquired SAA in children.

Methods: Four patients including three girls and one boy were diagnosed with acquired SAA. Their ages were 5, 3, 1, and 3 years, respectively. Read More

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http://dx.doi.org/10.1111/petr.13325DOI Listing
December 2018
1 Read

The world's first clinical trial for an aplastic anemia patient with thrombocytopenia administering platelets generated from autologous iPS cells.

Int J Hematol 2018 Dec 7. Epub 2018 Dec 7.

Department of Bioethics and Humanities, University of Washington School of Medicine, Seattle, WA, 98195-7120, USA.

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http://dx.doi.org/10.1007/s12185-018-02565-yDOI Listing
December 2018
1 Read

Infections in patients with aplastic Anemia in Chiang Mai University.

BMC Hematol 2018 4;18:35. Epub 2018 Dec 4.

Division of Hematology, Department of Internal Medicine, Faculty of Medicine, Chiang Mai University, Chiang Mai, 50200 Thailand.

Background: Infection is a major complication in aplastic anemia (AA) patients. Primary objectives of this study were to determine the prevalence of infections and to determine types of pathogens associated with infections in patients with AA. Secondary objectives were to evaluate overall survival after infections as well as risk factors of infections in patients with AA. Read More

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http://dx.doi.org/10.1186/s12878-018-0129-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6280474PMC
December 2018
1 Read

Haematological immune-related adverse events induced by anti-PD-1 or anti-PD-L1 immunotherapy: a descriptive observational study.

Lancet Haematol 2019 Jan 4;6(1):e48-e57. Epub 2018 Dec 4.

Service de Médecine Interne and Immunologie Clinique, Hôpital Bicêtre, Assistance Publique-Hôpitaux de Paris, Le Kremlin-Bicêtre, France; Université Paris Sud, Centre de Recherche en Immunologie des Infections Virales et des Maladies Auto-Immunes, INSERM, Le Kremlin-Bicêtre, France; Division d'Immunovirologie, Commissariat à l'Energie Atomique et aux Energies Alternatives, Fontenay-aux-Roses, France.

Background: Anti-programmed cell death 1 (PD-1) and anti-programmed cell death ligand 1 (PD-L1) antibodies are novel immunotherapies for cancer that can induce immune-related adverse events (irAEs). These adverse events can involve all organs, including the haemopoietic system. Thus far, haematological irAEs (haem-irAEs) have not been extensively characterised. Read More

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http://dx.doi.org/10.1016/S2352-3026(18)30175-3DOI Listing
January 2019
2 Reads

Autoimmune hemolytic anemia, autoimmune neutropenia and aplastic anemia in the elderly.

Eur J Intern Med 2018 12;58:77-83

UOC Oncoematologia, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico di Milano; Università degli Studi di Milano, Italy.

The physiology of the immune system involves morphologic and functional changes occurring along ageing, with a decrease in immune response and an increase in autoimmune phenomena, even in the absence of overt disese. Autoimmune cytopenias, namely autoimmune hemolytic anemia (AIHA), chronic idiopathic neutropenia (CIN) and aplastic anemia (AA), show different epidemiologic predilection, but are increasingly diagnosed in the elderly, where complications and comorbidities are more frequent. A systematic review of recent literature, shows that comorbidities as well as underlying deficiencies, medications, neoplasms, and, pathophysiologic chronic organ failures, frequently challenge the differential diagnosis in this setting and should always be evaluated and excluded. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S09536205183023
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http://dx.doi.org/10.1016/j.ejim.2018.05.034DOI Listing
December 2018
4 Reads

Alternative donor transplants for severe aplastic anemia.

Hematology Am Soc Hematol Educ Program 2018 11;2018(1):467-473

Department of Hematology, Fondazione Policlinico Universitario Gemelli IRCCS, Universita' Cattolica del Sacro Cuore, Rome, Italy.

Allogeneic hematopoietic stem-cell transplantation remains the only curative treatment for patients with acquired severe aplastic anemia (SAA). When a matched sibling is not available, one can search for a matched unrelated donor or a cord blood unit (CB) in the international registries or, more recently, for an HLA haploidentical (HAPLO) family member. International guidelines call for a course of antithymocyte globulin (ATG) and cyclosporine before a patient with SAA receives a transplant from a donor other than an HLA identical sibling, but whether this is necessary for patients age <20 years is less clear. Read More

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http://dx.doi.org/10.1182/asheducation-2018.1.467DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6245994PMC
November 2018
1 Read

A brief, but comprehensive, guide to clonal evolution in aplastic anemia.

Authors:
Daria V Babushok

Hematology Am Soc Hematol Educ Program 2018 11;2018(1):457-466

Division of Hematology-Oncology, Department of Medicine, Hospital of the University of Pennsylvania, Philadelphia, PA; and.

Acquired aplastic anemia (AA) is an immune-mediated bone marrow aplasia that is strongly associated with clonal hematopoiesis upon marrow recovery. More than 70% of AA patients develop somatic mutations in their hematopoietic cells. In contrast to other conditions linked to clonal hematopoiesis, such as myelodysplastic syndrome (MDS) or clonal hematopoiesis of indeterminate potential in the elderly, the top alterations in AA are closely related to its immune pathogenesis. Read More

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http://dx.doi.org/10.1182/asheducation-2018.1.457DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6245980PMC
November 2018
10 Reads

Activity of eltrombopag in severe aplastic anemia.

Hematology Am Soc Hematol Educ Program 2018 11;2018(1):450-456

Division of Hematology, Hospital A Beneficência Portuguesa, Sao Paulo, Brazil.

Since the approval of horse antithymocyte globulin (ATG) decades ago, there was a long hiatus in therapies with activity in severe aplastic anemia (SAA). This scenario changed in 2014 when eltrombopag, a thrombopoietin receptor agonist, was approved for SAA after an insufficient response to initial immunosuppressive therapy (IST). The basis for this approval was the observation of single-agent activity of eltrombopag in this patient population, where 40% to 50% recovered blood counts at times involving >1 lineage. Read More

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http://dx.doi.org/10.1182/asheducation-2018.1.450DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6245975PMC
November 2018
1 Read

[Advance of Mechanisms and Clinical Applications about Rapamycin for Treating Immune Mediated Hemocytopenia--Review].

Authors:
Hao Gu Run-Hui Wu

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2018 Dec;26(6):1836-1840

Department of Hematology and Oncology,Beijing Children's Hospital,Capital Medical University,Beijing 100045,China

Immune-mediated hemocytopenia is a common cytopenic diseases without bone marrow hematopoietic abnormalities, the patient's quality of life is significantly reduced when first-line treatments are ineffective. Rapamycin, possesses a clear mechanism of targeting mTOR protein, can upregulate regulatory T cells and induces apoptosis of specific cells, by regulating the lymphocyte subsets, so as to treat various types of immune-mediated hemocytopenia with a certain therapeutic effect. In this reviews, the action mechanism and clinical application of rapamycin in immune thrombocytopenia(ITP), autoimmune hemolytic anemia(AIHA), acquired aplastic anemia and autoimmune lymphoproliferative syndrome(ALPS) etc. Read More

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http://dx.doi.org/10.7534/j.issn.1009-2137.2018.06.043DOI Listing
December 2018
2 Reads

[Relationship between HLA Gene Polymorphism and Aplastic Anemia in Northern Chinese Han Patients].

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2018 Dec;26(6):1731-1737

Department of Hematology, First Affiliated Hospital, Chinese PLA General Hospital, Beijing 100048,

Objective: To explore the relationship between HLA-A, -B, -C, -DRB1, -DQB1 gene polymorphism and aplastic anemia (AA)of 65 cases in Northern China.

Methods: The high resolution genotyping of HLA-A, -B, -C, -DRB1, -DQB1 alleles in 65 AA patients and 772 healthy controls was performed with polymerase chain reaction-sequence specific oligonucleotide (PCR-SSO), the relationship between HLA-A, -B, -C, -DRB1, -DQB1 gene polymorphism and aplastic anemia was analyzed by Pearson Chi-square,Continuity Correction, Two-sided Fisher's Exact Test and Odds Ratio.

Results: The HLA-B*1302(10% vs 4. Read More

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http://dx.doi.org/10.7534/j.issn.1009-2137.2018.06.025DOI Listing
December 2018
6 Reads

Hereditary spherocytosis: Retrospective evaluation of 65 children.

Turk J Pediatr 2018 ;60(3):264-269

Department of Pediatric Hematology, University of Health Sciences, Ankara Child Health and Diseases Hematology Oncology Training and Research Hospital, Ankara, Turkey.

Güngör A, Yaralı N, Fettah A, Ok-Bozkaya İ, Özbek N, Kara A. Hereditary spherocytosis: Retrospective evaluation of 65 children. Turk J Pediatr 2018; 60: 264-269. Read More

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http://dx.doi.org/10.24953/turkjped.2018.03.005DOI Listing
January 2018
8 Reads

Genetic Profile and Microsatellite Instability in a Case of Secondary Esophageal Squamous Cell Carcinoma 12 Years After Allogeneic Hematopoietic Stem Cell Transplantation for Aplastic Anemia.

J Pediatr Hematol Oncol 2018 Nov 28. Epub 2018 Nov 28.

Departments of Pediatrics.

We report on a 16-year-old Japanese boy in whom an esophageal squamous cell carcinoma (ESCC) developed 12 years after allogeneic hematopoietic stem cell transplantation was performed for aplastic anemia. A high frequency of microsatellite instability was detected in samples of ESCC. Moreover, the detection of pathogenic variants, including single nucleotide substitution of TP53 (c. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001355DOI Listing
November 2018
1 Read

[Progress in prediction and recovery of immunosuppressive therapy in patients with severe aplastic anemia].

Authors:
S X Ding R Fu

Zhonghua Xue Ye Xue Za Zhi 2018 Nov;39(11):960-964

Tianjin Medical University General Hospital, Tianjin 300052, China.

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http://dx.doi.org/10.3760/cma.j.issn.0253-2727.2018.11.019DOI Listing
November 2018
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[A preliminary study on SLAMF6 expression in patients with severe aplastic anemia].

Zhonghua Xue Ye Xue Za Zhi 2018 Nov;39(11):927-931

Department of Hematology, General Hospital, Tianjin Medical University, Tianjin 300052, China.

To explore the expression of SLAMF6 on CD8(+) T cells in patients with severe aplastic anemia (SAA) and its correlation with disease immune status. By flow cytometry (FCM), SLAMF6 expression level in peripheral blood CD8(+) T cells was detected in 21 patients with SAA and 15 normal controls respectively from February 2017 to April 2018. The correlation between SLAMF6 expression level and hematopoietic functions, including HGB, PLT, the neutrophil granulocyte and reticulocyte absolute value in peripheral blood, hyperplasia degree (percentage of granulocytes, erythrocytes, lymphocytes and megakaryocytes in bone marrow) and perforin, granzyme B, IFN-γ expression level in CD8(+) T cells were evaluated. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0253-2727.2018.11.011DOI Listing
November 2018
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[Efficacy and safety of haploidentical hematopoietic stem cell transplantation for 17 patients with paroxysmal nocturnal hemamoglobinuria].

Zhonghua Xue Ye Xue Za Zhi 2018 Nov;39(11):904-907

Jiangsu Institute of Hematology, Department of Hematology, the First Affiliated Hospital of Soochow University, Key Lab of Thrombosis and Hemostasis of Ministry of Health, Suzhou 215006, China.

To explore the efficacy and safety of haploidentical hematopoietic stem cell transplantation (Haplo-HSCT) for paroxysmal nocturnal hemoglobinuria (PNH). A total of 17 patients with PNH who received Haplo-HSCT from January 2013 to September 2017 were analyzed retrospectively. Of them, 4 patients had de novo PNH, 13 patients had aplastic anemia-PNH syndrome (AA-PNH). Read More

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http://dx.doi.org/10.3760/cma.j.issn.0253-2727.2018.11.006DOI Listing
November 2018
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Bilateral Spontaneous Hyphemas in a Patient with Aplastic Anemia.

Case Rep Ophthalmol 2018 Sep-Dec;9(3):444-448. Epub 2018 Oct 18.

Jones Eye Institute, Department of Ophthalmology, University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA.

Bilateral spontaneous hyphemas are a rare ophthalmic event. Aplastic anemia is a hematologic condition with well-documented manifestations in the posterior segment but not the anterior segment. We present a patient with aplastic anemia without obvious risk factors for hyphema who developed bilateral spontaneous hyphemas. Read More

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https://www.karger.com/Article/FullText/493784
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http://dx.doi.org/10.1159/000493784DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6244096PMC
October 2018
2 Reads

Temozolomide-induced Aplastic Anemia Treated with Eltrombopag and Granulocyte Colony Stimulating Factor: A Report of a Rare Complication.

Cureus 2018 Sep 18;10(9):e3329. Epub 2018 Sep 18.

Internal Medicine, University of North Dakota School of Medicine and Health Sciences, Bismarck, USA.

Temozolomide is an alkylating agent used in the treatment for glioblastoma multiforme (GBM), the most frequent primary malignant brain tumor in adults. Temozolomide was approved in March 2005 for treatment of GBM, with the Stupp protocol (radiotherapy and concomitant use of temozolomide). Despite initial studies demonstrating mild and well-tolerated side effects, several recent reports describe severe hematologic adverse effects associated with temozolomide use. Read More

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https://www.cureus.com/articles/15003-temozolomide-induced-a
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http://dx.doi.org/10.7759/cureus.3329DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6248811PMC
September 2018
11 Reads

NK cells suppress CD8 T cell immunity via NKG2D in severe aplastic anemia.

Cell Immunol 2018 Oct 12. Epub 2018 Oct 12.

Department of Hematology, Tianjin Medical University General Hospital, 154 Anshan Street, Heping District, Tianjin 300052, PR China. Electronic address:

The roles of natural killer (NK) cells in shaping the immune system had raised wide interests. Here we intended to explore the regulatory functions of NK cells on CD8 T cells in severe aplastic anemia (SAA) using human participants and lymphocyte infusion-induced bone marrow failure (BMF) mouse model. In SAA patients, NK cells had over-expressions of NKG2D and NKp46, under-expression of NKG2A and enhanced cytotoxicity. Read More

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http://dx.doi.org/10.1016/j.cellimm.2018.10.004DOI Listing
October 2018
15 Reads

Quetiapine Induced Autoimmune Hemolytic Anemia in a Child Patient: A Case Report.

Clin Psychopharmacol Neurosci 2018 Nov;16(4):501-504

Departments of Child Hematology and Oncology, Faculty of Medicine, Kahramanmaras Sutcu Imam University, Kahramanmaras, Turkey.

Autoimmune hemolytic anemia is a disease characterized with destruction of erythrocytes as a result of antibody produce against patient's own erythrocytes and anemia. Autoimmune hemolytic anemia can be roughly stratified into two groups according to serological features and secondary causes including drugs induced hemolytic anemia. Drugs induced autoimmune hemolytic anemia is very rare in pediatric patients. Read More

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http://dx.doi.org/10.9758/cpn.2018.16.4.501DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6245293PMC
November 2018
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Aplastic anaemia in pregnancy - a single centre, North American series.

Br J Haematol 2018 Nov 20. Epub 2018 Nov 20.

Departments of Medicine, Pathology and Laboratory Medicine, University of Toronto, Toronto, Ontario, Canada.

Aplastic anaemia (AA) is infrequently observed in pregnancy. We describe 19 pregnancies in 9 women at a tertiary care centre over a period of 30 years. Spontaneous resolution of AA did not occur postpartum in the five pregnancies where AA was first diagnosed in pregnancy. Read More

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http://dx.doi.org/10.1111/bjh.15654DOI Listing
November 2018
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Comparison of High Sensitivity and Conventional Flow Cytometry for Diagnosing Overt Paroxysmal Nocturnal Hemoglobinuria and Detecting Minor Paroxysmal Nocturnal Hemoglobinuria Clones.

Ann Lab Med 2019 Mar;39(2):150-157

Department of Laboratory Medicine, University of Ulsan College of Medicine, Ulsan University Hospital, Ulsan, Korea.

Background: High sensitivity flow cytometry (HS-FCM) was recently developed for diagnosing paroxysmal nocturnal hemoglobinuria (PNH). We compared its performance with conventional flow cytometry (C-FCM) for diagnosing overt PNH and detecting minor (0.1-1%) PNH clones in aplastic anemia (AA)/low-grade myelodysplastic syndrome (MDS) patients. Read More

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http://dx.doi.org/10.3343/alm.2019.39.2.150DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6240522PMC
March 2019
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Epidemiological, clinical and genetic characterization of aplastic anemia patients in Pakistan.

Ann Hematol 2018 Nov 13. Epub 2018 Nov 13.

Department of Biochemistry, Quaid-i-Azam University, Islamabad, 44000, Pakistan.

Aplastic anemia (AA) is the most serious non-malignant blood disorder in Pakistan, ranked second in prevalence, after thalassemia. We investigated various epidemiological, clinical, and genetic factors of AA in a Pakistani cohort of 214 patients reporting at our hospital between June 2014 and December 2015. A control group of 214 healthy subjects was included for comparison of epidemiological and clinical features. Read More

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http://link.springer.com/10.1007/s00277-018-3542-z
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http://dx.doi.org/10.1007/s00277-018-3542-zDOI Listing
November 2018
8 Reads
2.634 Impact Factor

Aplastic Anemia in China.

J Transl Int Med 2018 Sep 9;6(3):134-137. Epub 2018 Oct 9.

Department of Hematology, General Hospital of Tianjin Medical University, Tianjin 300052, China.

Aplastic anemia (AA) is a hematologic disease characterized by pancytopenia. Up to now, severe aplastic anemia (SAA) has been recognized by international and domestic scholars as an autoimmune disease with bone marrow (BM) failure mediated by the hyperfunctional T lymphocytes. The incidence of AA is more in China compared with other countries. Read More

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http://dx.doi.org/10.2478/jtim-2018-0028DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6231301PMC
September 2018
2 Reads