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transient abnormal myelopoiesis

Yoshihiko Shitara, Naoto Takahashi, Yoshinori Aoki, Motohiro Kato, Riki Nishimura, Shinya Tsuchida, Akira Oka
Infants with Down Syndrome (DS) are at risk of developing a transient abnormal myelopoiesis (TAM). TAM is characterised by increased circulating blast cells but usually self-limiting. DS patients with TAM sometimes show fetal hydrops and effusion in body cavities, but the mechanism remains unclear. We report here a case of infant with DS who had pericardial effusion, TAM, and eosinophilia. In her pericardial effusion, white blood cell count was 6.0 × 10(3)/µL, 41% of which were eosinophils. After administration of prednisolone, pericardial effusion gradually decreased, and TAM and eosinophilia improved...
2017: Tohoku Journal of Experimental Medicine
Guglielmo Salvatori, Silvia Foligno, Pietro Sirleto, Silvia Genovese, Serena Russo, Valentina Coletti, Andrea Dotta, Matteo Luciani
Congenital leukemia is rare disease with an incidence of one to five cases per million births. Transient abnormal myelopoiesis (TAM), also called transient myeloproliferative disorder, is a pre-leukemia disorder that may occur in Down syndrome (DS) or non-DS infants. TAM may enter spontaneous remission; however, continual monitoring is required, as this disorder has been observed to develop into acute megakaryoblastic leukemia in 16-30% of cases. In the literature, 16 cases of TAM in non-DS infants have been reported...
January 2017: Oncology Letters
Liqun Yin, Mark A Lovell, Michael L Wilson, Qi Wei, Xiayuan Liang
OBJECTIVES: Down syndrome (DS)-associated transient abnormal myelopoiesis (TAM) or acute megakaryoblastic leukemia (AMKL) in monozygotic twins is exceedingly rare and has not been well characterized. METHODS: We describe a unique case of monozygotic twins with simultaneous TAM from a triplet pregnancy at 34 weeks' gestation. Previously reported cases of TAM and DS-AMKL in monozygotic twins have been reviewed to compare with our cases. The current concept of a sequential multistep process in leukemogenesis and disease evolution of TAM into DS-AMKL through the collaboration among trisomy 21, GATA1, and other gene mutations is also reviewed...
December 2016: American Journal of Clinical Pathology
Shunsuke Minakata, Naoki Sakata, Norihisa Wada, Yuhei Konishi, Satoshi Marutani, Takuji Enya, Hidenori Nakagawa, Hiroshi Wada, Tsukasa Takemura
Transient abnormal myelopoesis is mostly self-resolving and has a good prognosis, but some patients subsequently die of liver fibrosis. We report the case of an infant with Down syndrome who developed life-threatening liver fibrosis at the same time as the blasts were about to disappear. This patient also had a marked increase in eosinophils, which were possibly harboring a GATA1 mutation and were expressing a high level of platelet-derived growth factor-B mRNA; these may have been involved in the development of liver fibrosis...
November 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
Eline J M Bertrums, Arjan Buijs, Martine van Grotel, Natasja Dors, Jasmijn D E de Rooij, Valerie de Haas, Sanne Hopman, Marjolijn C J Jongmans, C M Zwaan, Marry M van den Heuvel-Eibrink
Transient myeloproliferative disorder (TMD) is a leukemia type that occurs typically in newborns. In Down syndrome, TMD is referred to as transient abnormal myelopoiesis (TAM).(32) Recently, transientness has also been reported in acute myeloid leukemia patients with germline trisomy 21 mosaicism, and even in cases with somatic trisomy 21, with or without GATA1 mutations. TMD cases without trisomy 21 are rare, and recurrent genetic aberrations that aid in clinical decision-making are scarcely described. We describe here a TMD patient without trisomy 21 or GATA1 mutation in whom single-nucleotide polymorphism analysis of leukemic blasts revealed a novel combined submicroscopic deletion (5q31...
September 26, 2016: Pediatric Blood & Cancer
Hajime Maeda, Hayato Go, Takashi Imamura, Maki Sato, Nobuo Momoi, Mitsuaki Hosoya
Infants with Down syndrome (DS) are at risk of developing a transient myeloproliferative disorder during the neonatal period, known as transient abnormal myelopoiesis (TAM). It is characterized by clonal myeloproliferation and is typically self-limiting. However, TAM can be a life-threatening disorder, when complicated by liver fibrosis. Here, we evaluated cytokine profiles in two male DS infants having TAM with or without liver dysfunction. The first patient, Patient 1, had hyperleukocytosis with cholestatic liver dysfunction, coagulopathy, and increased counts of blasts and was treated with exchange transfusion (ExT) due to the serious general condition...
2016: Tohoku Journal of Experimental Medicine
Neha Bhatnagar, Laure Nizery, Oliver Tunstall, Paresh Vyas, Irene Roberts
Children with constitutional trisomy 21 (Down syndrome (DS)) have a unique predisposition to develop myeloid leukaemia of Down syndrome (ML-DS). This disorder is preceded by a transient neonatal preleukaemic syndrome, transient abnormal myelopoiesis (TAM). TAM and ML-DS are caused by co-operation between trisomy 21, which itself perturbs fetal haematopoiesis and acquired mutations in the key haematopoietic transcription factor gene GATA1. These mutations are found in almost one third of DS neonates and are frequently clinically and haematologcially 'silent'...
October 2016: Current Hematologic Malignancy Reports
B Bain
Subjects with Down's syndrome have an increased risk, particularly during childhood, of developing acute leukaemia. There is an increased incidence both of acute lymphoblastic leukaemia and of acute myeloid leukaemia (AML), particularly acute megakaryoblastic leukaemia, often presenting with the clinical features of acute myelofibrosis. In addition neomates with Down's syndrome show a high incidence of polycythaemia and may also develope a syndrome simulating acute leukaemia, commonly designated transient abnormal myelopoiesis (TAM)...
1991: Leukemia & Lymphoma
E Lobato-Mendizabal, A Marín-Lopez, G López-de-la-Cruz, M Presno-Bernal, G J Ruiz-Argüelles
The case of a newborn with Down's syndrome and congenital leukaemia is reported. The malignant white blood cells displayed the CD41 antigen (glycoprotein Ilb/IIIa) identified by monoclonal antibodies HP1-ld and FMC24 and the CD9/p24 antigen identified by monoclonal antibody FMC27. The number of cells in S-phase was 14%, as assessed by the incorporation of 5-bromo 2-deoxyuridine. No other chromosomal abnormalities were identified in addition to 47 XY + 21. The patient died 15 days after the diagnosis, due to Pneumocystis Carinii pneumonia...
1990: Leukemia & Lymphoma
Su Han Lum, Soo Sin Choong, Shekhar Krishnan, Zulqarnain Mohamed, Hany Ariffin
INTRODUCTION: Children with Down syndrome (DS) are at increased risk of developing distinctive clonal myeloid disorders, including transient abnormal myelopoiesis (TAM) and myeloid leukaemia of DS (ML-DS). TAM connotes a spontaneously resolving congenital myeloproliferative state observed in 10%-20% of DS newborns. Following varying intervals of apparent remission, a proportion of children with TAM progress to develop ML-DS in early childhood. Therefore, TAM and ML-DS represent a biological continuum...
June 2016: Singapore Medical Journal
Barbara J Bain
No abstract text is available yet for this article.
August 2016: American Journal of Hematology
Satoshi Saida
Children with Down syndrome (DS) have a markedly increased risk of leukemia. They are at particular risk of acute megakaryoblastic leukemia, known as myeloid leukemia associated with DS (ML-DS), the development of which is closely linked to a preceding temporary form of neonatal leukemia called transient abnormal myelopoiesis (TAM). Findings from recent clinical and laboratory studies suggest that constitutional trisomy 21 and GATA1 mutation(s) cause TAM, and that additional genetic alteration(s) including those in epigenetic regulators and signaling molecules are involved in the progression from TAM to ML-DS...
April 2016: International Journal of Hematology
Satoshi Saida
Leukemia arises through an evolutionary process of somatic mutation and selection. Transient abnormal myelopoiesis (TAM) is a clonal pre-leukemic disorder that progresses to myeloid leukemia of Down syndrome (ML-DS) through the accumulation of genetic alterations. To investigate the mechanism underlying leukemogenesis, a xenograft model of TAM was established using NOG mice. Serial engraftment after cell transplantation from a TAM patient who developed ML-DS a year later showed the self-renewal capacity of these cells...
December 2015: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Ikuko Fujihara, Ryu Yanagisawa, Yu Fukushima, Kazutoshi Komori, Yoshifumi Ogiso, Kazuo Sakashita
No abstract text is available yet for this article.
February 2016: British Journal of Haematology
Sa A Wang, Robert P Hasserjian
OBJECTIVES: At the 2013 Society for Hematopathology/European Association for Hematopathology Workshop, 36 cases were submitted to the session that covered acute erythroid leukemia (AEL), acute megakaryoblastic leukemia (AMKL), and reactive mimics. METHODS: Cases were reviewed by the session chairs and workshop panel to reach a consensus diagnosis. RESULTS: For acute erythroleukemia, erythroid/myeloid type, discussion acknowledged overlapping features between AEL and myelodysplastic syndromes...
July 2015: American Journal of Clinical Pathology
J A Tamblyn, A Norton, L Spurgeon, V Donovan, A Bedford Russell, J Bonnici, K Perkins, P Vyas, I Roberts, M D Kilby
OBJECTIVE: To systematically review current evidence regarding prenatal diagnosis and management of transient abnormal myelopoiesis (TAM) in fetuses with trisomy 21. A novel case of GATA1-positive TAM, in which following serial in utero blood transfusion clinical improvement and postnatal remission were observed, is included. SEARCH STRATEGY AND DATA COLLECTION: A systematic search of electronic databases (inception to October 2014) and reference lists, hand-searching of journals and expert contact...
January 2016: Archives of Disease in Childhood. Fetal and Neonatal Edition
Karen M Chisholm, Claudia V Rivetta, Amy Heerema-McKenney
Transient abnormal myelopoiesis (TAM) and myeloid leukemia associated with Down syndrome (ML of DS) have morphologically indistinguishable blasts. TAM usually presents and regresses within the first three months of life. In a subset of patients, myelopoiesis remains abnormal, and the persistence of elevated blasts after 6 months is considered ML of DS. Current tools including cytogenetics and flow cytometry fail to distinguish blasts of TAM that will regress from blasts of ML of DS. One gene expression profiling study suggested PRAME expression was significantly increased in ML of DS compared to TAM...
2015: Annals of Clinical and Laboratory Science
Teppei Ohkawa, Satoshi Miyamoto, Manabu Sugie, Daisuke Tomizawa, Kohsuke Imai, Masayuki Nagasawa, Tomohiro Morio, Shuki Mizutani, Masatoshi Takagi
We encountered a case of neonatal acute megakaryoblastic leukemia not associated with Down syndrome (DS). Molecular cytogenetic analysis of leukemic blast cells indicated that increased blast cell status was caused by transient abnormal myelopoiesis with trisomy 21 and GATA1 mutation. Based on these molecular cytogenetic data, intensive chemotherapy was avoided, and the patient was successfully cured with low-dose cytarabine. Morphologically, leukemic blast cells of acute megakaryoblastic leukemia in a non-DS neonate are indistinguishable from a blast cell of transient abnormal myelopoiesis...
2015: Pediatrics International: Official Journal of the Japan Pediatric Society
Sanjita Ravishankar, Laurie Hoffman, Terakeith Lertsburapa, Jennifer Welch, Diana Treaba, Monique E De Paepe
Transient abnormal myelopoiesis (TAM), a clonal proliferation of predominantly megakaryocytic precursor cells, affects 4%-10% of newborns with Down syndrome. Approximately 20%-30% of TAM survivors are at risk of development of acute myeloid leukemia (myeloid leukemia associated with Down syndrome, ML-DS). We report unusual placental findings in a female infant with trisomy 21 born at 38 weeks of gestation. In line with previous descriptions of placental pathology in infants with TAM, abundant blast-like cells were present in the lumen of chorionic and stem villous vessels...
May 2015: Pediatric and Developmental Pathology
Audrey Bidet, Stéphanie Dulucq, Nathalie Aladjidi
No abstract text is available yet for this article.
January 2015: British Journal of Haematology
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