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Pure red cell aplasia

Panagiota Zikidou, Anastassia Grapsa, Zoe Bezirgiannidou, Athanassios Chatzimichael, Elpis Mantadakis
Background: Human parvovirus B19 (HPV-B19) is the etiologic agent of erythema infectiosum, of transient aplastic crises in individuals with underlying chronic hemolytic disorders, and of chronic pure red cell aplasia in immunocompromised individuals. Case report: We describe a 14-year-old girl with long-standing Evans syndrome, who presented with severe anemia, reticulocytopenia and thrombocytopenia. A bone marrow aspirate revealed severe erythroid hypoplasia along with the presence of giant pronormoblasts, while serological studies and real-time PCR of whole blood were positive for acute parvovirus B19 infection...
2018: Mediterranean Journal of Hematology and Infectious Diseases
Marzieh Tavakol, Seyed Alireza Mahdaviani, Mir Reza Ghaemi, Mohammad Vaezi, Atosa Dorudinia, Hamidreza Jamaati, Ali Akbar Velayati
Good's syndrome, the adult onset hypogammaglobulinemia associated with thymoma has been explained about six decades ago. It generally presents with recurrent infections and several paraneoplastic syndromes including myasthenia gravis, pure red cell aplasia, connective tissue disorders, superior vena cava, Horner's syndrome, lichen planus and inflammatory bowel disease. Lack of B cell, dysfunction of T cell, CD4+ T cell lymphopenia, reversed CD4/CD8+ T cell ratio, autoantibodies against Th17 related cytokines have been respected as the pathogenesis of the immune dysregulation this syndrome...
February 2018: Iranian Journal of Allergy, Asthma, and Immunology
Aron Simkins, Abhishek Maiti, Nicholas J Short, Nitin Jain, Uday Popat, Keyur P Patel, Thein H Oo
Association of pure red-cell aplasia with thymoma is well documented. However, acquired amegakaryocytic thrombocytopenia (AAMT) has been rarely associated with thymoma with only five reported cases in literature. We report a patient with thymoma complicated by pure red cell aplasia (PRCA) and AAMT who progressed to develop aplastic anemia (AA). The patient was refractory to 10-months of immunosuppressive therapy with cyclosporine, prednisone, and antithymocyte globulin. She was eventually treated with allogeneic stem cell transplantation (allo-SCT)...
January 31, 2018: Hematology/oncology and Stem Cell Therapy
Yusuke Oji, Masayoshi Inoue, Yoshito Takeda, Naoki Hosen, Yasushi Shintani, Manabu Kawakami, Takuya Harada, Yui Murakami, Miki Iwai, Mari Fukuda, Sumiyuki Nishida, Jun Nakata, Yoshiki Nakae, Satoshi Takashima, Toshiaki Shirakata, Hiroko Nakajima, Kana Hasegawa, Hiroshi Kida, Takashi Kijima, Soyoko Morimoto, Fumihiro Fujiki, Akihiro Tsuboi, Eiichi Morii, Satoshi Morita, Junichi Sakamoto, Atsushi Kumanogoh, Yoshihiro Oka, Meinoshin Okumura, Haruo Sugiyama
Thymic epithelial tumors are rare malignancies, and no optimal therapeutic regimen has been defined for patients with advanced disease. Patients with advanced thymic epithelial tumors, which were resistant or intolerable to prior therapies, were eligible for this study. Patients received 9 mer-WT1-derived peptide emulsified with Montanide ISA51 adjuvant via intradermal administration once a week as a monotherapy. After the 3 month-protocol treatment, the treatment was continued mostly at intervals of 2 to 4 weeks until disease progression or intolerable adverse events occurred...
January 11, 2018: International Journal of Cancer. Journal International du Cancer
Tina Rubic-Schneider, Masataka Kuwana, Brigitte Christen, Manuela Aßenmacher, Otmar Hainzl, Frank Zimmermann, Robert Fischer, Vera Koppenburg, Salah-Dine Chibout, Timothy M Wright, Andreas Seidl, Michael Kammüller
Immunogenicity of biotherapeutics and the elicitation of anti-drug antibodies are a key concern for their efficacy, pharmacokinetics, and safety. A particularly severe consequence of immunogenicity of a biotherapeutic is the rare development of antibody-mediated pure red cell aplasia (PRCA) in anemic patients treated with aggregated forms of recombinant human erythropoietin (rhEPO). Here, we investigated in vitro T-cell responses to experimentally heat-induced rhEPO aggregates, and to tungsten-induced rhEPO aggregates in clinical lots associated with rhEPO-neutralizing antibodies and PRCA...
February 14, 2017: Blood Advances
Min Shi, Rong He, Andrew L Feldman, David S Viswanatha, Dragan Jevremovic, Dong Chen, William G Morice
Although T-cell large granular lymphocytic leukemia (T-LGLL) is a clinically indolent disorder, patients with moderate-to-severe cytopenia require therapeutic intervention. The recent discovery of STAT3 mutations has shed light on the genetic basis of T-LGLL pathogenesis. However, the association of STAT3 mutational status with patients' clinical, histopathologic, and other laboratory features has not been thoroughly evaluated in T-LGLL. In this study, STAT3 mutations were identified in 18 of 36 patients with T-LGLL (50%), including Y640F (12/18, 66...
December 26, 2017: Human Pathology
Alexandra Serris, Zahir Amoura, Florence Canouï-Poitrine, Benjamin Terrier, Eric Hachulla, Nathalie Costedoat-Chalumeau, Thomas Papo, Olivier Lambotte, David Saadoun, Miguel Hié, Philippe Blanche, Bertrand Lioger, Jacques-Eric Gottenberg, Bertrand Godeau, Marc Michel
The aim of the study was to assess the efficacy and safety of rituximab (RTX) for treating systemic lupus erythematosus (SLE)-associated immune cytopenias. This multicenter retrospective cohort study of adults from French referral centers and networks for adult immune cytopenias and SLE involved patients ≥18 years old with a definite diagnosis of SLE treated with RTX specifically for SLE-associated immune cytopenia from 2005 to 2015. Response assessment was based on standard definitions. In total, 71 patients, 61 women (85...
December 16, 2017: American Journal of Hematology
Xiujuan Gui, Xinhai Zhu, Liangjun Guo, Guoqiang Tan, Yan Liu, Yi Tan, Qiufang Chen, Yuwei Song, Shaoqiang Lin
RATIONALE: Thymomas are associated with numerous autoimmune disorders, such as myasthenia gravis (MG), pure red cell aplasia (PRCA), and systemic lupus erythematosus (SLE). However, graft-versus-host disease (GVHD)-like erythroderma is a relatively uncommon paraneoplastic disorder associated with thymomas and signifies a poor prognosis. PATIENT CONCERNS: A 35-year-old woman with medical history significant for stage IVa type AB thymoma presented with patchy erythema over face, trunk, and extremities that failed to respond to topical steroids...
December 2017: Medicine (Baltimore)
Wei Zou, Zekun Wang, Min Xiong, Aaron Yun Chen, Peng Xu, Safder S Ganaie, Yomna Badawi, Steve Kleiboeker, Hiroshi Nishimune, Shui Qing Ye, Jianming Qiu
Human parvovirus B19 (B19V) infection of human erythroid progenitor cells (EPCs) induces a DNA damage response and cell cycle arrest at late S phase, which facilitates viral DNA replication. However, it is not clear exactly which cellular factors are employed by this single-stranded DNA virus. Here, we used microarrays to systematically analyze the dynamic transcriptome of EPCs infected with B19V. We found that DNA metabolism, DNA replication, DNA repair, DNA damage response, cell cycle, and cell cycle arrest pathways were significantly regulated after B19V infection...
December 13, 2017: Journal of Virology
J F Chen, J M Kang, Y F Zhang, M F Wang, C X Dong, L Y Wang, L P Guo, L H Yang
No abstract text is available yet for this article.
November 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
Suresh Kumar Balasubramanian, Meena Sadaps, Swapna Thota, Mai Aly, Bartlomiej P Przychodzen, Cassandra M Hirsch, Valeria Visconte, Tomas Radivoyevitch, Jaroslaw P Maciejewski
Pure red cell aplasia is an orphan disease, and as such lacks rationally established standard therapies. Most cases are idiopathic; a subset is antibody-mediated. There is overlap between idiopathic cases and those with T-cell large granular lymphocytic leukemia, hypogammaglobulinemia, and low-grade lymphomas. In each of the aforementioned, the pathogenetic mechanisms may involve autoreactive cytotoxic responses. We selected 62 uniformly diagnosed pure red cell aplasia patients and analyzed their pathophysiologic features and responsiveness to rationally applied first-line and salvage therapies in order to propose diagnostic and therapeutic algorithms that may be helpful in guiding the management of prospective patients, 52% of whom were idiopathic, while the others involved large granular lymphocytic leukemia, thymoma, and B-cell dyscrasia...
February 2018: Haematologica
Waqar Ahmed, Rizwan Ul Haq Dogar, Sumit Acharya
Parvovirus B19 infection should be contemplated as one of the differential diagnoses of persistent anemia in transplanted patients. There must be high index of suspicion of Parvovirus B19, when post-transplant patients present with refractory and severe anemia with reticulocytopenia and all the other common causes of anemia such as blood loss, adverse effects of immunosuppressant agents and graft dysfunction has been ruled out. In suspected patients, diagnosis is confirmed by serological tests (IgM and IgG), PCR from blood and/or bone marrow and by bone marrow biopsy finding of pure red cell aplasia...
December 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
Gérard London, Johannes Mann, David Goldsmith, Christian Combe, Frank Dellanna, Philippe Zaoui, Nadja Hoebel, Andriy Krendyukov, Karen MacDonald, Ivo Abraham
AIMS: To assess real-world effectiveness and safety of intravenous (IV) HX575, a biosimilar epoetin-α, in hemodialysis (HD) patients. MATERIALS AND METHODS: This prospective, observational, pharmacoepidemiological study of adult HD patients treated with IV HX575 for renal anemia for up to 24 months was conducted in 114 centers in 10 European countries. Of 2,086 enrolled subjects (safety sample), 2,023 had ≥ 1 follow-up visit (effectiveness sample). RESULTS: Most (59...
January 2018: Clinical Nephrology
Rosario Varela Gómez, Goretti Vázquez Vázquez, Victor Noriega Concepción, Andrea Galego García, Concepción Andón Saavedra
The literature reports an incidence of Pure Red Cell Aplasia (PRCA) ranging from 6-30% of all cases of ABO-incompatible HSCT. Although most patients resolve spontaneously after withdrawal immunosuppression, some of them require more aggressive treatment to manage this condition.
October 18, 2017: Hematology/oncology and Stem Cell Therapy
Hasan Hashem, Susan J Kelly, Nancy J Ganson, Michael S Hershfield
PURPOSE OF REVIEW: A new autoinflammatory disease, deficiency of adenosine deaminase 2 (DADA2), caused by mutations in the CECR1 gene, was first reported in 2014. This review aims to update progress in defining, treating, and understanding this multi-faceted disorder. RECENT FINDINGS: DADA2 was first described in patients with systemic inflammation, mild immune deficiency, and vasculopathy manifested as recurrent stroke or polyarteritis nodosa (PAN). More than 125 patients have now been reported, and the phenotype has expanded to include children and adults presenting primarily with pure red cell aplasia (PRCA), or with antibody deficiency...
October 5, 2017: Current Rheumatology Reports
Yasuyuki Saito, Yuri Sawada, Yasuhiko Koga, Noriaki Sunaga, Yusuke Tsukagoshi, Yoshimasa Hachisu, Takashi Osaki, Reiko Sakurai, Kyoichi Kaira, Akihiro Ono, Ken Sato, Hiromi Koiso, Tetsunari Oyama, Takeshi Hisada, Masanobu Yamada
A 41-year-old woman treated with isoniazid (INH) for latent tuberculosis infection and an oral corticosteroid for sarcoidosis developed severe anemia two months after initiating INH. A bone marrow examination showed erythroblastopenia, and a diagnosis of INH-induced pure red cell aplasia (PRCA) was made. Her reticulocyte count and hemoglobin levels improved two weeks after discontinuation of INH. A literature review of INH-induced PRCA shows that it occurs very rarely in the context of autoimmune disorders...
October 15, 2017: Internal Medicine
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
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
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
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
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