Read by QxMD icon Read

anemia aplastic in children

Y B Choi, E S Yi, J W Lee, K W Sung, H H Koo, K H Yoo
We compared the outcomes of immunosuppressive treatment (IST) with those of alternative donor hematopoietic stem cell transplantation (HSCT) in children and adolescents with severe aplastic anemia (SAA). The medical records of 42 patients with SAA who received frontline IST (N=19) or frontline HSCT with an alternative donor (N=23) between 1998 and 2012 were analyzed retrospectively. Six patients responded in the frontline IST group, whereas 11 underwent salvage HSCT after IST failure. Twenty-one of 23 patients who underwent frontline HSCT survived without treatment failure...
September 26, 2016: Bone Marrow Transplantation
Rumesh Chandar, Venkatesh Chandrasekaran, Barath Jagadisan, Delhi Kumar, Niranjan Biswal
A 7-year-old male child with very severe aplastic anemia developed refractory disease, which was attributed to febrile hemolytic transfusion reactions and was treated with immunosuppressants, which lead to a transient improvement. However, the child worsened subsequently and succumbed to an underlying hemophagocytic lymphohistiocytosis that was recognized late due to an overlap of clinical and biochemical parameters in both the conditions. Hemophagocytic lymphohistiocytosis should be an early suspicion in children with refractory very severe aplastic anemia and the detection of underlying gene mutations can predict disease severity...
August 19, 2016: Journal of Pediatric Hematology/oncology
Amélie Fradet-Turcotte, Justine Sitz, Damien Grapton, Alexandre Orthwein
Maintaining genomic integrity is essential to preserve normal cellular physiology and to prevent the emergence of several human pathologies including cancer. The breast cancer susceptibility gene 2 (BRCA2, also known as the Fanconi anemia (FA) complementation group D1 (FANCD1)) is a potent tumor suppressor that has been extensively studied in DNA double-stranded break (DSB) repair by homologous recombination (HR). However, BRCA2 participates in numerous other processes central to maintaining genome stability, including DNA replication, telomere homeostasis and cell cycle progression...
October 2016: Endocrine-related Cancer
Line K Rasmussen, Elisabeth V Stenbøg, Gitte B Kerndrup, Henrik Hasle
An association between hepatitis and aplastic anemia (AA) is known as hepatitis-associated AA, and is characterized by an acute attack of hepatitis followed by the development of AA. We report 2 clinical cases of acute seronegative hepatitis in which pancytopenia with mild dysplasia developed after 3 months; however, neither of our cases fulfilled the histological criteria of AA, but rather myelodysplastic syndrome. This novel association bears considerable resemblance to hepatitis-associated AA, and raises the question of whether hepatitis-associated dysmyelopoiesis should be included in the spectrum of hypocellular myelodysplastic syndrome...
July 27, 2016: Journal of Pediatric Hematology/oncology
Christopher J Gamper, Clifford M Takemoto, Allen R Chen, Heather J Symons, David M Loeb, James F Casella, Amy E Dezern, Karen E King, Andrea M McGonigle, Richard J Jones, Robert A Brodsky
OBJECTIVE: Use of high-dose cyclophosphamide without hematopoietic stem cell transplant to treat severe aplastic anemia (SAA) has been controversial due to concern for increased infectious toxicity as compared with antithymocyte globulin and cyclosporine A. As children often tolerate dose-intensive therapy better than adults, we sought to perform a detailed retrospective analysis of both treatment response and toxicity in 28 patients younger than 22 years of age treated with 29 courses of high-dose cyclophosphamide as the sole form of immunosuppression...
July 27, 2016: Journal of Pediatric Hematology/oncology
L Wang, H X Wang, L Zhu, X L Zheng, Z D Wang, H M Yan, L Ding, D M Han
OBJECTIVE: To observe the efficacy of matched unrelated donor hematopoietic stem cell transplant (HSCT) with transfusion of multipotent mesenchymal cells (MSC) in pediatric severe aplastic anemia (SAA). METHODS: 19 children with SAA received matched unrelated donor HSCT with MSC, and the hematopoietic recovery and transplant-associated complications of these children were monitored. RESULTS: All patients achieved rapid hematopoietic reconstruction after HSCT, and the median durations to neutrophil and platelet recovery were 12 (9-21) days and 14 (8-24) days respectively, but delayed rejection occurred in one case four months after HSCT...
June 14, 2016: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
Sioban B Keel, Angela Scott, Marilyn Sanchez-Bonilla, Phoenix A Ho, Suleyman Gulsuner, Colin C Pritchard, Janis L Abkowitz, Mary-Claire King, Tom Walsh, Akiko Shimamura
Clinical and histopathological distinction between the inherited versus acquired bone marrow failure or myelodysplastic syndromes is challenging. The identification of inherited bone marrow failure/ myelodysplastic syndromes is critical to inform appropriate clinical management. To investigate whether a subset of pediatric and young adults undergoing transplant for aplastic anemia or myelodysplastic syndrome have germline mutations in bone marrow failure/myelodysplastic syndrome genes, we performed a targeted genetic screen of samples obtained between 1990-2012 from children and young adults with aplastic anemia or myelodysplastic syndrome transplanted at the Fred Hutchinson Cancer Research Center...
July 14, 2016: Haematologica
Birol Baytan, Adalet Meral Güneş, Solmaz Çelebi, Ünsal Günay
OBJECTIVE: Fungal infection is a significant problem, causing of infective deaths of leukemic patients. The situation in developing countries is not well documented. The purpose of this study was characterizing IFD by analyzing data retrospectively to determine the incidence, predisposing factors, diagnostic methods, efficacy of treatment, and the outcome in pediatric patients with hematological disorders. METHODS: There were 160 children with leukemia (22 AML, 129 ALL) and 9 with aplastic anemia (AA)...
December 5, 2009: Turkish Journal of Haematology: Official Journal of Turkish Society of Haematology
Barış Malbora, Zekai Avcı, Fulden Dönmez, Bülent Alioğlu, Esra Baskın, Füsun Alehan, Namık Özbek
OBJECTIVE: Posterior reversible leukoencephalopathy syndrome (PRES) is characterized by headache, altered mental status, cortical blindness, and seizures associated with neuroradiological findings. It involves predominantly white matter of the parieto-occipital lobes. Several medications and disorders play a role in the etiology of PRES. In this study, we aimed to show how the prognosis of PRES in hematological diseases of childhood might be according to the etiological factors. METHODS: Here, we report PRES in six patients, aged 4 to 14 years, with diagnoses of leukemia and aplastic anemia...
September 5, 2010: Turkish Journal of Haematology: Official Journal of Turkish Society of Haematology
Beom Joon Kim, Kyung Pil Moon, Ji-Hong Yoon, Eun-Jung Lee, Jae Young Lee, Seong Koo Kim, Jae Wook Lee, Nack Gyun Chung, Bin Cho, Hack Ki Kim
PURPOSE: Severe aplastic anemia (SAA), a fatal disease, requires multiple transfusion, immunosuppressive therapy, and finally, hematopoietic stem cell transplantation (HSCT) as the definitive treatment. We hypothesized that iron overloading associated with multiple transfusions and HSCTrelated complications may adversely affect cardiac function. Left ventricular (LV) function was assessed in children after HSCT for SAA. METHODS: Forty-six consecutive patients with a median age of 9...
April 2016: Korean Journal of Pediatrics
Takeshi Mori, Ryojiro Tanaka, Kosuke Nishida, Nobuyuki Yamamoto, Akira Hayakawa, Noriyuki Nishimura, Kandai Nozu, Kazumoto Iijima
Transient hyperphosphatasemia (TH) is defined as marked elevation of serum alkaline phosphatase (ALP), predominantly its bone or liver isoform. It is a rare condition and is usually detected on laboratory examination in patients without any clinical symptoms. In typical patients with TH, ALP spontaneously normalizes, but no apparent cause of TH has been identified. Some drugs are suspected triggers of TH, but no clear evidence of their association with TH has been shown to date. We herein report three cases of TH in pediatric patients...
May 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
Kyogo Suzuki, Hideki Muramatsu, Yusuke Okuno, Atsushi Narita, Asahito Hama, Yoshiyuki Takahashi, Makoto Yoshida, Yasuo Horikoshi, Ken-Ichiro Watanabe, Kazuko Kudo, Seiji Kojima
Idiopathic aplastic anemia (AA) is a rare hematological complication of Down syndrome (DS). The safety and efficacy of immunosuppressive therapy (IST) in individuals with DS remain unknown. We used a standard regimen of IST, comprising antithymocyte globulin and cyclosporine A, to treat three children with DS and idiopathic acquired AA. Two patients achieved a hematological (complete or partial) response and became transfusion independent at the final follow-up. The third patient failed to respond to IST and underwent bone marrow transplantation from a human leukocyte antigen (HLA)-mismatched unrelated donor...
July 2016: International Journal of Hematology
Giuseppe Maggiore, Gérard Socie, Marco Sciveres, Anne-Marie Roque-Afonso, Silvia Nastasio, Catherine Johanet, Fréderic Gottrand, Sébastien Fournier-Favre, Emmanuel Jacquemin, Olivier Bernard
BACKGROUND: A few children with acute or chronic liver disease display histological features compatible with autoimmune hepatitis, but lack specific serological markers. AIM: To describe features, management and outcome of childhood seronegative autoimmune hepatitis. METHODS: From 1988 to 2010, 38 children were included under the following criteria: negative virological studies, no serum autoantibodies, exclusion of other causes of liver diseases, and liver histology compatible with autoimmune hepatitis...
July 2016: Digestive and Liver Disease
X T Xie, W He, W Shi, X X Zhou, X H Qiao
OBJECTIVE: This study was designed to compare the effects of the anti-human T lymphocyte globulin (Fresenius, ATG-F)and rabbit anti-human thymocyte immunoglobulin (Genzyme, R-ATG)in the treatment of childhood aplastic anemia (AA) and their effects. METHOD: A total of 59 children with aplastic anemia were analyzed in the present study, including 34 cases of severe aplastic anemia (SAA), 12 cases of very severe aplastic anemia (VSAA) and 13 cases of transfusion-dependent non-severe aplastic anemia (NSAA)...
April 2016: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
Michaela Nováková, Markéta Žaliová, Martina Suková, Marcin Wlodarski, Aleš Janda, Eva Froňková, Vít Campr, Kateřina Lejhancová, Ondřej Zapletal, Dagmar Pospíšilová, Zdeňka Černá, Tomáš Kuhn, Peter Švec, Vendula Pelková, Zuzana Zemanová, Gitte Kerndrup, Marry van den Heuvel-Eibrink, Vincent van der Velden, Charlotte Niemeyer, Tomáš Kalina, Jan Trka, Jan Starý, Ondřej Hrušák, Ester Mejstříková
GATA-2 deficiency was recently described as common cause of overlapping syndromes of immunodeficiency, lymphedema, familiar myelodysplastic syndrome or acute myeloid leukemia. The aim of our study was to analyze bone marrow and peripheral blood samples of children with myelodysplastic syndrome or aplastic anemia to define prevalence of the GATA2 mutation and to assess whether mutations in GATA-2 transcription factor exhibit specific immunophenotypic features. The prevalence of a GATA2 mutation in a consecutively diagnosed cohort of children was 14% in advanced forms of myelodysplastic syndrome (refractory anemia with excess blasts, refractory anemia with excess blasts in transformation, and myelodysplasia-related acute myeloid leukemia), 17% in refractory cytopenia of childhood, and 0% in aplastic anemia...
June 2016: Haematologica
Te-Fu Weng, Kang-Hsi Wu, Han-Ping Wu, Ching-Tien Peng, Yu-Hua Chao
BACKGROUND: Invasive aspergillosis (IA) has recently increased and has a high mortality rate in immunocompromised patients. IA before hematopoietic stem cell transplantation (HSCT) is not uncommon, but how to cope with it is very tough. The serum aspergillus galactomannan antigen (GM) is a helpful marker for diagnosis of IA, and a serial follow-up of GM levels is important to evaluate the response of treatment. However, data on the changes of GM during HSCT are very limited. CASE PRESENTATION: Patient 1 was a 2-year-old female with severe aplastic anemia...
2016: Italian Journal of Pediatrics
Jane S Hankins, Rhiannon R Penkert, Paul Lavoie, Li Tang, Yilun Sun, Julia L Hurwitz
Parvovirus B19 infection causes transient aplastic crisis in sickle cell disease (SCD) due to a temporary interruption in the red blood cell production. Toxicity from hydroxyurea includes anemia and reticulocytopenia, both of which also occur during a transient aplastic crisis event. Hydroxyurea inhibits proliferation of hematopoietic cells and may be immunosuppressive. We postulated that hydroxyurea could exacerbate parvovirus B19-induced aplastic crisis and inhibit the development of specific immune responses in children with SCD...
April 2016: Experimental Biology and Medicine
Daisuke Hasegawa
Myelodysplastic syndrome (MDS) without increased blasts, i.e., low-grade MDS, is the most common subtype of pediatric MDS and has characteristics different from adult form of the disease. Although histological findings of bone marrow (BM) biopsies suggest that low-grade MDS is a morphologically distinctive entity, a subset of pediatric low-grade MDS may clinically overlap with aplastic anemia (AA), such as high likelihood of hypocellular marrow and normal karyotype. In addition, children with low-grade MDS are as likely to respond to immunosuppressive therapy as those with AA, which indicates that a part of these disorders might share a common pathogenesis, that is, T cell-mediated inhibition of hematopoiesis...
April 2016: International Journal of Hematology
Hoon Kook, Nack-Gyun Chung, Hyoung Jin Kang, Ho Joon Im
The treatment of choice for aplastic anemia (AA) in children has been HLA-matched family donor (MFD) hematopoietic stem cell transplantation (HSCT). For those lacking MFD, immunosuppressive therapy (IST) consisting of horse antithymocyte globulin (ATG) and cyclosporine has been successful. The choices of second and third line treatments are more complex and debatable, especially in the situation of unavailability of horse ATG. IST with rabbit ATG seems to be less effective. Recently, improved survival of non-MFD HSCTs has been documented...
April 2016: International Journal of Hematology
Tang-Her Jaing, Chieh-Tsai Wu, Shih-Hsiang Chen, Yu-Chuan Wen, Tsung-Yen Chang, Wen-Yu Chuang
We report 4 pediatric cases of biopsy-proven posttransplant lymphoproliferative disorder (PTLD) in the context of allogeneic hematopoietic stem cell transplantation (HSCT). All cases showed diffuse staining with latent membrane protein-1 in immunohistochemistry. The median age at transplant of 4 patients with PTLD was 10.1 years (range, 2.2 to 13.2 y). The median interval between HSCT and the diagnosis of PTLD was 5.5 months (range, 4 to 8 mo). All patients were treated with rituximab at dosage of 375 mg/m at weekly intervals...
April 2016: Journal of Pediatric Hematology/oncology
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"