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Aplastic anaemia

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Geetanjali S Rathore, Kathryn S Leung, Eyal Muscal
BACKGROUND: Neurological complications, especially encephalopathy and seizures, are commonly seen in bone marrow transplant patients. Infections, chemotoxicity, graft versus host disease, or secondary central nervous system malignancies are the most common underlying etiologies. There is increased awareness that autoimmune encephalitis may cause neurological dysfunction in immunocompetent children. The potential role of such a mechanism in children undergoing bone marrow transplantation is unknown...
September 2015: Pediatric Neurology
H Schrezenmeier, S Körper, B Höchsmann
Aplastic anemia (AAI) is a rare life-threatening disorder which is characterized by bi- or tricytopenia and hypoplastic or aplastic bone marrow. AA can present as an acquired or congenital disorder. In recent years it was noted that a subgroup of patients with seemingly acquired AA with onset in adulthood carry mutations which cause or at least predispose to bone marrow failure, e.g. mutations in the genes of the telomerase complex. Options for first-line treatment are allogeneic stem cell transplantation or immunosuppression...
September 2015: Der Internist
Min Wang, Xingxin Li, Jun Shi, Yingqi Shao, Meili Ge, Jinbo Huang, Zhendong Huang, Jing Zhang, Neng Nie, Yizhou Zheng
Treatment options for patients with severe aplastic anemia (SAA) in developing countries are limited. A cohort of 261 patients with SAA received a novel immunosuppressive strategy of cyclosporine alternately combined with levamisole plus danazol (CSA&LMS-based regimen), which included 70 VSAA and 191 moderate SAA [initial absolute neutrophil count (ANC) >200/μL] cases. The CSA&LMS-based regimen was administrated orally with an initial dose of CSA 3 mg/kg in adults and 5 mg/kg in children every other day, LMS 150 mg in adults and 2...
August 2015: International Journal of Hematology
Angelica Barone, Annunziata Lucarelli, Daniela Onofrillo, Federico Verzegnassi, Sonia Bonanomi, Simone Cesaro, Francesca Fioredda, Anna Paola Iori, Saverio Ladogana, Anna Locasciulli, Daniela Longoni, Marina Lanciotti, Alessandra Macaluso, Rosalba Mandaglio, Nicoletta Marra, Baldo Martire, Matteo Maruzzi, Giuseppe Menna, Lucia Dora Notarangelo, Giovanni Palazzi, Marta Pillon, Ugo Ramenghi, Giovanna Russo, Johanna Svahn, Fabio Timeus, Fabio Tucci, Chiara Cugno, Marco Zecca, Piero Farruggia, Carlo Dufour, Paola Saracco
Acquired aplastic anemia (AA) is a rare heterogeneous disease characterized by pancytopenia and hypoplastic bone marrow. The incidence is 2-3/million inhabitants/year, in Europe, but higher in East Asia. Survival in severe aplastic anemia (SAA) has markedly improved in the past 2 decades because of advances in hematopoietic stem cell transplantation, immunosuppressive and biologic drugs, and supportive care. In SAA hematopoietic stem cell transplant (HSCT) from a matched sibling donor (MSD) is the treatment of choice...
June 2015: Blood Cells, Molecules & Diseases
Carlo Dufour, Marta Pillon, Gerard Sociè, Alicia Rovò, Elisa Carraro, Andrea Bacigalupo, Rosi Oneto, Jakob Passweg, Antonio Risitano, Andrè Tichelli, Regis Peffault de Latour, Hubert Schrezenmeier, Britta Hocshmann, Christina Peters, Austin Kulasekararaj, Anja Van Biezen, Sujith Samarasinghe, Ayad Ahmed Hussein, Mouhab Ayas, Mahmoud Aljurf, Judith Marsh
This study analysed the outcome of 563 Aplastic Anaemia (AA) children aged 0-12 years reported to the Severe Aplastic Anaemia Working Party database of the European Society for Blood and Marrow Transplantation, according to treatment received. Overall survival (OS) after upfront human leucocyte antigen-matched family donor (MFD) haematopoietic stem cell transplantation (HSCT) or immunosuppressive treatment (IST) was 91% vs. 87% (P 0·18). Event-free survival (EFS) after upfront MFD HSCT or IST was 87% vs. 33% (P 0·001)...
May 2015: British Journal of Haematology
Chen Liang, Jialin Wei, Erlie Jiang, Qiaoling Ma, Aiming Pang, Sizhou Feng, Mingzhe Han
In this work we describe a 3-year-old boy with hepatitis-associated aplastic anemia (HAAA) treated successfully with autologous cord blood transplantation combined with immunosuppressive therapy. There is little previous experience in the utility of autologous cord blood transplantation in the treatment of HAAA. Nowadays, for patients born after 1980, an HLA matched sibling donor is not usually available because of the family planning policy in our country. So more and more parents choose to preserve the umbilical cord blood for their children...
April 2015: Transfusion and Apheresis Science
Jonathan C Howell, Sarita A Joshi, Lindsey Hornung, Jane Khoury, Richard E Harris, Susan R Rose
BACKGROUND: Diamond-Blackfan anemia (DBA), an inherited marrow failure syndrome, has severe hypoplastic anemia in infancy and association with aplastic anemia, MDS/leukemia, and other malignancies. Short stature is present in most patients. Isolated cases have demonstrated improved growth on growth hormone (GH) therapy. PROCEDURES: GH treatment data were obtained from 19 children with DBA (6 at our site and 13 from Genentech). Control data from 44 non-GH treated children were provided by Diamond Blackfan Anemia Registry...
March 2015: Pediatric Blood & Cancer
A Rovó, A Tichelli, C Dufour
Since the introduction of the concept of aplastic anemia (AA) by Paul Ehrlich in 1888 and despite the current better understanding of the underlying mechanisms involved in this disease, a clear delimitation among BM failure syndromes is still a matter of debate. The diagnosis of AA can be difficult basically due to the overlapping morphological characteristics with other BM failure disorders. This paper reviews critical data relevant to the diagnosis of acquired AA and recommends work out steps and main considerations to determine severity and characterization of the disease...
February 2013: Bone Marrow Transplantation
Jeffrey K Davies, Eva C Guinan
The current outlook for a child with severe idiopathic aplastic anaemia (AA) is very much better than in previous decades. In part, this may reflect better differentiation of idiopathic and inherited marrow failure. For children with idiopathic AA and a human leucocyte antigen (HLA)-matched sibling donor (MSD), allogeneic haematopoietic stem-cell transplantation (AHSCT) is the primary therapy of choice, offering long-term disease-free survival of 90%, although graft-versus-host disease remains a cause of long-term morbidity...
February 2007: British Journal of Haematology
Edo Hasanbegović
We report two cases of very rare congenital hypoplastic anemia (Diamond-Blackfan anemia) in children. Both patients were boys admitted to our Clinic as severe anemia of unknown etiology, and had been previously treated in their Cantonal hospitals. Diagnose of congenital hypoplastic anemia was set up according: anamnesis, clinical picture with dominant features of anemia, cytomorphologic finding of peripheral blood picture, according blood marrow aspirate finding and according erithropoetin level level in blood...
2005: Medicinski Arhiv
J S Wasser, R Yolken, D R Miller, L Diamond
A 31-yr-old female with congenital hypoplastic anemia (Diamond-Blackfan syndrome) whose long course terminated in acute myelogenous leukemia is described. In contrast to Fanconi anemia, malignant transformation rarely occurs in congenital hypoplastic anemia. This patient's diagnosis of congenital hypoplastic anemia is supported by her clinical course, absence of renal abnormalities, a negative family history for hematologic disorders, normal chromosome studies, failure of her skin fibroblasts to transform in culture with SV-40 virus, macrocytic erythrocyte indices, erythrocyte enzyme studies, and bone marrow findings...
May 1978: Blood
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