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Pure red cell aplasia

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https://www.readbyqxmd.com/read/28894066/-thymoma-with-pure-red-cell-aplasia-report-of-a-case
#1
Masanobu Hayakawa, Kazuyuki Oda
A thymoma with pure red cell aplasia (PRCA) is relatively rare. A 71-year-old woman complainted of dizziness and her blood cell count showed a severe anemia. She was diagnosed as PRCA by bone marrow aspiration biopsy, which showed marked decrease in number of erythroblasts. In addition, the chest computed tomography revealed a solid tumor in the anterior mediastinum. She underwent extended thymothymectomy through median sternotomy. The resected specimen was 10.5×9.7 cm in diameter. The pathological diagnosis was type AB thymoma of the World Health Organization classification, and Masaoka stage I...
September 2017: Kyobu Geka. the Japanese Journal of Thoracic Surgery
https://www.readbyqxmd.com/read/28893803/pure-red-cell-aplasia-and-myasthenia-gravis-a-patient-having-both-autoimmune-conditions-in-the-absence-of-thymoma
#2
Annelise Aquilina, David James Camilleri, Josanne Aquilina
This is a patient who was presented initially with symptoms of malaise, tiredness and exertional dyspnoea and found to have a severe normocytic normochromic anaemia with low reticulocyte counts. Bone marrow confirmed the diagnosis of pure red cell aplasia (PRCA) and at the time serology for recent parvovirus infection was positive. He was successfully treated with transfusions and intravenous Ig. Six years later, he had a mild relapse of his PRCA and subsequently developed severe dysphagia and dysarthria which were fatigable...
September 11, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28883274/successful-long-term-management-with-low-dose-prednisolone-in-an-adult-patient-with-diamond-blackfan-anemia
#3
Toshie Ogasawara, Kiyotaka Kawauchi, Naoki Mori, Hiroshi Sakura, Fumiyo Katoh, Hitoshi Kanno, Etsuro Ito
Diamond-Blackfan anemia (DBA) is a rare congenital disease caused by mutations in ribosomal protein genes and is characterized by pure red cell aplasia. While the prognosis is relatively favorable, quality of life (QOL) among DBA patients is negatively impacted by the adverse effects of long-term prednisolone (PSL) therapy and blood transfusions. We describe a 43-year-old man who was diagnosed with DBA (Hb of 2.18 g/dl) at the age of two months. He was initially treated with PSL and blood transfusions, followed by cyclosporine and low-dose (6 mg/day) PSL, which resulted in a sustained hemoglobin level of 9 g/dl without severe adverse events or loss of QOL...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28875009/abo-incompatibility-and-hematopoietic-stem-cell-transplantation-outcomes
#4
Mohammad Vaezi, Davoud Oulad Dameshghi, Maryam Souri, Seyed Amin Setarehdan, Kamran Alimoghaddam, Ardeshir Ghavamzadeh
Introduction: The increased risk of hemolytic reactions and erythrocyte recovery delay in ABO incompatible hematopoietic stem cell transplantation (HSCT) are well established. Effects of ABO incompatibility on other transplantation outcomes are evaluated in this study. Subjects and Methods: We prospectively followed 501 patients undergoing allogeneic stem cell transplantation regarding their ABO compatibility groups for a median time of 34.7 months. Patients were studied in minor, major and bidirectional mismatched and matched groups...
April 1, 2017: International Journal of Hematology-oncology and Stem Cell Research
https://www.readbyqxmd.com/read/28872489/a-case-of-pure-red-cell-aplasia-during-nivolumab-therapy-for-cardiac-metastatic-melanoma
#5
Akihiko Yuki, Tatsuya Takenouchi, Sumiko Takatsuka, Takuro Ishiguro
Nivolumab is an antibody against programmed cell death 1 and functions as an immune checkpoint inhibitor for various malignancies, including unresectable melanomas. Nivolumab causes several immune-related adverse events, which typically include skin rash, pneumonitis, thyroid dysfunction, hepatitis, and colitis; in rare cases, anemia may be present. There are several reports of autoimmune hemolytic anemia that has developed in response to nivolumab; however, there are few reports of pure red cell aplasia (PRCA)...
September 1, 2017: Melanoma Research
https://www.readbyqxmd.com/read/28750791/use-of-nandrolone-decanoate-in-treatment-of-pure-red-cell-aplasia-secondary-to-diclofenac-administration-a-case-report
#6
Paula Nassar de Marchi, André Nanny Le Sueur Vieira, José Francisco Antunes Ribeiro, Silvano Salgueiro Geraldes, Paulo Roberto Rodrigues Ramos, Alessandra Melchert, Priscylla Tatiana Chalfun Guimarães-Okamoto
Pure red cell aplasia (PRCA) is a disorder that leads to a nonregenerative anemia that results from erythroid precursors failing to reach maturity in the bone marrow, whereas the numbers of mature myeloid and megakaryocytic cells remain normal. PRCA can be induced by autoimmune processes, infections, drugs, toxins, and radiation, and is diagnosed by a bone marrow cytology examination after excluding the most common causes of nonregenerative anemia. Immunosuppressive therapies are used to treat PRCA, and usually involve the use of glucocorticoids, cyclosporin, or azathioprine...
March 2017: Topics in Companion Animal Medicine
https://www.readbyqxmd.com/read/28739760/cyclosporin-a-reversed-chemoresistance-of-a-patient-with-pure-red-cell-aplasia-secondary-to-thymoma
#7
Peng Li, Fengcai Du, Zhaohua Gong, Baohong Hu, Cheng Chi, Hongjin Chu, Jian Chen
This case study reports on a patient who relapsed with thymoma (mixed type) nine years after tumor resection. After four courses of rescue chemotherapy (docetaxel and cisplatinum), the patient was further diagnosed with pure red cell aplasia. It was noticed that cyclosporin A (CsA), which was administered to treat aplasia, could reverse chemoresistance. Its mechanism is not completely clear, but the hypothesis of CsA inhibiting P-glycoprotein mediated drug efflux is the most acceptable.
August 2017: Anticancer Research
https://www.readbyqxmd.com/read/28664500/trend-and-treatment-patterns-of-aplastic-anemia-in-korea-pure-red-cell-aplasia-and-myelodysplastic-syndrome-in-korea-a-nation-wide-analysis
#8
Yunsuk Choi, Jae-Cheol Jo, Hee-Jeong Jeon, Dong Wook Kim, Myung Hee Chang, Hawk Kim
Aplastic anemia (AA) and pure red cell aplasia (PRCA) appear to be more prevalent in Asian countries including Korea. However, there are no exact data regarding its prevalence and frequency of allogeneic hematopoietic cell transplantation (HCT) in Korea. Here, we present demographic data relating to AA/PRCA/MDS in Korea. Data were prepared by retrieval from a computerized database maintained by the National Health Insurance Service and Korea National Statistical Office. HCT data were collected from all HCT centers in Korea...
June 29, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28644950/hematopoietic-cell-transplantation-in-fanconi-anemia-and-dyskeratosis-congenita-a-minireview
#9
REVIEW
Mouhab Ayas
Bone marrow failure syndrome is an epithet of bone marrow failure (all or single-cell lineage) that is attributable to an underlying genetic aberration usually with a constellation of somatic abnormalities. Multiple inheritance patterns have been described in these disorders; many are transmitted in an autosomal recessive pattern, which may consequently lead to a higher prevalence of such illnesses in homogeneous societies such as Saudi Arabia, where consanguineous marriages are not uncommon. At King Faisal Specialist Hospital and Research Center, the most common entity referred for allogeneic hematopoietic cell transplantation (HCT) is Fanconi anemia, followed by pure red aplasia, and, less commonly, dyskeratosis congenita, congenital neutropenia, and others...
June 15, 2017: Hematology/oncology and Stem Cell Therapy
https://www.readbyqxmd.com/read/28637614/classical-inherited-bone-marrow-failure-syndromes-with-high-risk-for-myelodysplastic-syndrome-and-acute-myelogenous-leukemia
#10
REVIEW
Sharon A Savage, Carlo Dufour
The inherited marrow failure syndromes (IBMFS) are a heterogeneous group of diseases characterized by failure in the production of one or more blood lineage. The clinical manifestations of the IBMFS vary according to the type and number of blood cell lines involved, including different combinations of anemia, leukopenia, and thrombocytopenia. In some IBMFS, systemic non-hematologic manifestations, including congenital malformations, mucocutaneous abnormalities, developmental delay, and other medical complications, may be present...
April 2017: Seminars in Hematology
https://www.readbyqxmd.com/read/28598573/successful-treatment-of-tacrolimus-related-pure-red-cell-aplasia-and-autoimmune-hemolytic-anemia-with-rituximab-in-a-pediatric-cardiac-transplant-patient
#11
Chenue Abongwa, Ghada Abusin, Ayman El-Sheikh
Acquired pure red cell aplasia (PRCA) and autoimmune hemolytic anemia (AIHA) are rare complications of immunosuppression in pediatric solid organ transplant patients. We report a 14-month-old female child who developed Coombs positive hemolytic anemia and reticulocytopenia while on tacrolimus after cardiac transplantation. She was successfully treated with rituximab after failing treatment with corticosteroids and intravenous immunoglobulins. Clinicians should consider PRCA differential diagnosis in a patient presenting with reticulocytopenia and hemolysis...
June 9, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28582594/histologic-and-cytologic-bone-marrow-findings-in-dogs-with-suspected-precursor-targeted-immune-mediated-anemia-and-associated-phagocytosis-of-erythroid-precursors
#12
Cynthia de A Lucidi, Christian L E de Rezende, L Ari Jutkowitz, Michael A Scott
BACKGROUND: Precursor-targeted immune-mediated anemia (PIMA) has been suspected in dogs with nonregenerative anemia and bone marrow findings varying from erythroid hyperplasia to pure red cell aplasia. Phagocytosis of erythroid precursors/rubriphagocytosis (RP) reported in some affected dogs suggests a destructive component to the pathogenesis of PIMA. OBJECTIVES: The purpose of the study was to characterize laboratory and clinical findings in dogs with suspected PIMA and RP, with emphasis on cytologic and histologic bone marrow findings...
June 5, 2017: Veterinary Clinical Pathology
https://www.readbyqxmd.com/read/28543448/successful-treatment-of-pure-red-cell-aplasia-because-of-abo-major-mismatched-stem-cell-transplant
#13
Katie Sackett, Claudia S Cohn, Kayla Fahey-Ahrndt, Angela R Smith, Andrew D Johnson
BACKGROUND: Pure red cell aplasia (PRCA) is a well-documented potential side effect of ABO major mismatched allogeneic hematopoietic stem cell transplants. This side effect may be self-limiting, but is sometimes treated using modalities such as steroids, antithymocyte globulin, donor lymphocyte infusions, rituximab, or plasma exchanges. Another well-documented cause of pure red cell aplasia is a chronic parvovirus B19 infection, which may be seen in immunocompromised hosts. The treatment of this cause of PRCA includes removal of immunosuppression, intravenous immunoglobulin (IVIg), or rituximab; however, this condition may also be self-limiting...
May 24, 2017: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/28525589/bone-marrow-involvement-in-systemic-lupus-erythematosus
#14
Emilie Chalayer, Nathalie Costedoat-Chalumeau, Odile Beyne-Rauzy, Jacques Ninet, Stephane Durupt, Jacques Tebib, Bouchra Asli, Olivier Lambotte, Martine Ffrench, Christian Vasselon, Pascal Cathébras
BACKGROUND: Besides peripheral cytopenias, bone marrow abnormalities, such as fibrosis, pure red cell aplasia, and aplastic anemia have been reported in patients with systemic lupus erythematosus (SLE), suggesting that bone marrow may be a target organ in SLE. Our objective was to describe this bone marrow involvement. METHODS: This registry is a nationwide retrospective study. Centers provided data concerning medical history, SLE manifestations, type of hematologic disorder, treatments and outcome...
May 19, 2017: QJM: Monthly Journal of the Association of Physicians
https://www.readbyqxmd.com/read/28488966/haemolysis-pure-red-cell-aplasia-and-red-cell-antibody-formation-associated-with-major-and-bidirectional-abo-incompatible-haematopoietic-stem-cell-transplantation
#15
Gordana Tomac, Ines Bojanić, Sanja Mazić, Ivana Vidović, Mirela Raos, Branka Golubić Ćepulić, Ranka Serventi Seiwerth, Jadranka Kelečić, Boris Labar
BACKGROUND: Acute and delayed haemolysis, alloimmunisation and pure red cell aplasia (PRCA) are potential complications after ABO incompatible haematopoietic stem cell transplantation (HSCT). The aims of this study were to investigate acute and delayed red blood cell (RBC) antibody-associated complications, including haemolysis, PRCA and alloimmunisation in major and bidirectional ABO incompatible HSCT. MATERIALS AND METHODS: We retrospectively examined the transplant courses of 36 recipients of bone marrow or peripheral blood stem cells from ABO incompatible donors and evaluated the current practice of performing plasmapheresis in patients with higher isoagglutinin titres...
April 19, 2017: Blood Transfusion, Trasfusione del Sangue
https://www.readbyqxmd.com/read/28473943/cyclosporine-is-a-potential-curative-treatment-option-for-advanced-thymoma
#16
Yusuke Isshiki, Hiroaki Tanaka, Yoshio Suzuki, Yukihiro Yoshida
BACKGROUND: Thymectomy can effectively cure most thymoma patients; however, patients with advanced thymoma typically require chemotherapy, which is associated with limited efficacy in this context. Here we provide the first report of a patient with recurrent thymoma who achieved complete remission (CR) using cyclosporine therapy. CASE PRESENTATION: A 63-year-old woman who had undergone resection surgery for recurrent type B1 thymoma developed pure red cell aplasia (PRCA), and CT findings revealed thymoma recurrence...
2017: Experimental Hematology & Oncology
https://www.readbyqxmd.com/read/28450687/thymoma-with-immunodeficiency-good-syndrome-associated-with-myasthenia-gravis
#17
Shunsuke Takai, Asako Tagawa, Tomoko Ogawa, Hiroyuki Kato, Noriko Saito, Shinya Okada
Good syndrome is a rare condition in which thymoma is associated with hypogammaglobulinemia; it is characterized by repeated respiratory or systemic infections caused by bacteria, viruses, and fungi, as well as with various autoimmune disorders such as pure red cell aplasia. A 65-year-old woman was admitted to our hospital with ptosis and abdominal muscle weakness. Based on the presence of anti-acetylcholine receptor (Ach-R) antibodies, she was diagnosed with myasthenia gravis (MG). At that time, invasive thymoma of Masaoka stage IVa was also detected...
April 27, 2017: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/28425751/development-of-red-blood-cell-autoantibodies-following-treatment-with-checkpoint-inhibitors-a-new-class-of-anti-neoplastic-immunotherapeutic-agents-associated-with-immune-dysregulation
#18
Laura L Cooling, John Sherbeck, Jonathon C Mowers, Sheri L Hugan
Ipilimumab, nivolumab, and pembrolizumab represent a new class of immunotherapeutic drugs for treating patients with advanced cancer. Known as checkpoint inhibitors, these drugs act to upregulate the cellular and humoral immune response to tumor antigens by inhibiting T-cell autoregulation. As a consequence, they can be associated with immune-related adverse events (irAEs) due to loss of self-tolerance, including rare cases of immune-related cytopenias. We performed a retrospective clinical chart review, including serologic, hematology, and chemistry laboratory results, of two patients who developed red blood cell (RBC) autoantibodies during treatment with a checkpoint inhibitor...
January 2017: Immunohematology
https://www.readbyqxmd.com/read/28387173/pure-red-cell-aplasia-due-to-parvovirus-b19-erythropoietin-resistant-anemia-in-a-pediatric-kidney-recipient
#19
LETTER
Baris Malbora, Serdar Saritas, Eda Ataseven, Burcu Belen, Caner Alparslan, Onder Yavascan, Dilek Yilmaz Ciftdogan, Berna Atabay, Meral Turker
No abstract text is available yet for this article.
June 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28382272/pure-red-cell-aplasia-associated-with-good-syndrome
#20
Masayuki Okui, Takashi Yamamichi, Ayaka Asakawa, Masahiko Harada, Hirotoshi Horio
Pure red cell aplasia (PRCA) and hypogammaglobulinemia are paraneoplastic syndromes that are rarer than myasthenia gravis in patients with thymoma. Good syndrome coexisting with PRCA is an extremely rare pathology. We report the case of a 50-year-old man with thymoma and PRCA associated with Good syndrome who achieved complete PRCA remission after thymectomy and postoperative immunosuppressive therapy, and provide a review of the pertinent literature.
April 2017: Korean Journal of Thoracic and Cardiovascular Surgery
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