急性巨核球性白血病
WordNet
- of critical importance and consequence; "an acute (or critical) lack of research funds"
- having or demonstrating ability to recognize or draw fine distinctions; "an acute observer of politics and politicians"; "incisive comments"; "icy knifelike reasoning"; "as sharp and incisive as the stroke of a fang"; "penetrating insight"; "frequent penetrative observations" (同)discriminating, incisive, keen, knifelike, penetrating, penetrative, piercing, sharp
- extremely sharp or intense; "acute pain"; "felt acute annoyance"; "intense itching and burning" (同)intense
- having or experiencing a rapid onset and short but severe course; "acute appendicitis"; "the acute phase of the illness"; "acute patients"
- of an angle; less than 90 degrees
- malignant neoplasm of blood-forming tissues; characterized by abnormal proliferation of leukocytes; one of the four major types of cancer (同)leukaemia, leucaemia, cancer of the blood
- of or pertaining to large bone marrow cells
PrepTutorEJDIC
- (先の)『鋭い』,とがった / (痛み・感情などが)『激しい』,強い / (知力・感覚などが)『鋭い』,鋭敏な / (事態が)重大な / (病気が)急性の / (音が)高い,鋭い / 鋭角の
- 白血病
UpToDate Contents
全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.
English Journal
- Mutation characterization in the GATA-1 gene in patients with Down's Syndrome diagnosed with transient abnormal myelopoiesis or acute megakaryoblastic leukemia.
- Mansini AP, Rubio PL, Rossi JG, Gallego MS, Medina A, Zubizarreta PA, Felice MS, Alonso CN.AbstractPatients with Down's Syndrome have a higher risk of developing acute megakaryoblastic leukemia (AML). Ten per cent of newborn infants with this syndrome have transient abnormal myelopoiesis (TAM), indistinguishable from AML, which generally remits spontaneously. A high incidence of GATA-1 gene mutations was described in both groups of patients. Fourteen bone marrow DNA samples (10 ATM/4 AML) were analyzed by PCR and sequencing; these samples were obtained from 13 patients with Down's Syndrome to describe the rate and mutation characteristics of the GATA-1 gene in the studied population and its consequences at a protein level. Mutations were detected in 10 out of 10 TAM and in 3 out of 4 AML, which at a protein level would result in an early termination codon (n= 5), alterations in the splicing site (n= 6) or sequence change (n= 3). The high rate of GATA-1 gene mutations was confirmed in newborn infants with Down's Syndrome and MAT or AML.
- Archivos argentinos de pediatría.Arch Argent Pediatr.2013 Dec;111(6):532-6. doi: 10.1590/S0325-00752013000600013.
- Patients with Down's Syndrome have a higher risk of developing acute megakaryoblastic leukemia (AML). Ten per cent of newborn infants with this syndrome have transient abnormal myelopoiesis (TAM), indistinguishable from AML, which generally remits spontaneously. A high incidence of GATA-1 gene mutat
- PMID 24196768
- Differential diagnosis of myelofibrosis based on WHO 2008 criteria: Acute panmyelosis with myelofibrosis, acute megakaryoblastic leukemia with myelofibrosis, primary myelofibrosis and myelodysplastic syndrome with myelofibrosis.
- Bae E, Park CJ, Cho YU, Seo EJ, Chi HS, Jang S, Lee KH, Lee JH, Lee JH, Suh JJ, Im HJ.Author information Department of Laboratory Medicine, VHS Medical Center, Seoul, South Korea; Department of Laboratory Medicine, College of Medicine and Asan Medical Center, University of Ulsan, Seoul, South Korea.AbstractINTRODUCTION: The aim of this study was to characterize clinicopathological features of acute panmyelosis with myelofibrosis (APMF), acute megakaryoblastic leukemia with myelofibrosis (AMKL-MF), primary myelofibrosis (PMF) and myelodysplastic syndrome with myelofibrosis (MDS-MF) in order to provide the keys to the differential diagnosis of bone marrow (BM) fibrosis.
- International journal of laboratory hematology.Int J Lab Hematol.2013 Dec;35(6):629-36. doi: 10.1111/ijlh.12101. Epub 2013 May 22.
- INTRODUCTION: The aim of this study was to characterize clinicopathological features of acute panmyelosis with myelofibrosis (APMF), acute megakaryoblastic leukemia with myelofibrosis (AMKL-MF), primary myelofibrosis (PMF) and myelodysplastic syndrome with myelofibrosis (MDS-MF) in order to provide
- PMID 23693053
- NUP98/JARID1A is a novel recurrent abnormality in pediatric acute megakaryoblastic leukemia with a distinct HOX gene expression pattern.
- de Rooij JD, Hollink IH, Arentsen-Peters ST, van Galen JF, Berna Beverloo H, Baruchel A, Trka J, Reinhardt D, Sonneveld E, Zimmermann M, Alonzo TA, Pieters R, Meshinchi S, van den Heuvel-Eibrink MM, Zwaan CM.Author information Department of Pediatric Hematology/Oncology, Erasmus MC-Sophia Children's Hospital, Rotterdam, The Netherlands.AbstractCytogenetic abnormalities and early response to treatment are the main prognostic factors in acute myeloid leukemia (AML). Recently, NUP98/NSD1 (t(5; 11)(q35; p15)), a cytogenetically cryptic fusion, was described as recurrent event in AML, characterized by dismal prognosis and HOXA/B gene overexpression. Using split-signal fluorescence in situ hybridization, other NUP98-rearranged pediatric AML cases were identified, including several acute megakaryoblastic leukemia (AMKL) cases with a cytogenetically cryptic fusion of NUP98 to JARID1A (t(11;15)(p15;q35)). In this study we screened 105 pediatric AMKL cases to analyze the frequency of NUP98/JARID1A and other recurrent genetic abnormalities. NUP98/JARID1A was identified in 11/105 patients (10.5%). Other abnormalities consisted of RBM15/MKL1 (n=16), CBFA2T3/GLIS2 (n=13) and MLL-rearrangements (n=13). Comparing NUP98/JARID1A-positive patients with other pediatric AMKL patients, no significant differences in sex, age and white blood cell count were found. NUP98/JARID1A was not an independent prognostic factor for 5-year overall (probability of overall survival (pOS)) or event-free survival (probability of event-free survival (pEFS)), although the 5-year pOS for the entire AMKL cohort was poor (42±6%). Cases with RBM15/MLK1 fared significantly better in terms of pOS and pEFS, although this was not independent from other risk factors in multivariate analysis. NUP98/JARID1A cases were characterized by HOXA/B gene overexpression, which is a potential druggable pathway. In conclusion, NUP98/JARID1A is a novel recurrent genetic abnormality in pediatric AMKL.
- Leukemia.Leukemia.2013 Dec;27(12):2280-8. doi: 10.1038/leu.2013.87. Epub 2013 Mar 27.
- Cytogenetic abnormalities and early response to treatment are the main prognostic factors in acute myeloid leukemia (AML). Recently, NUP98/NSD1 (t(5; 11)(q35; p15)), a cytogenetically cryptic fusion, was described as recurrent event in AML, characterized by dismal prognosis and HOXA/B gene overexpre
- PMID 23531517
Japanese Journal
- Detection of RBM15-MKL1 Fusion Was Useful for Diagnosis and Monitoring of Minimal Residual Disease in Infant Acute Megakaryoblastic Leukemia
- 次世代シーケンサーを用いたDown症候群のTAMと急性巨核芽球性白血病の全エクソン解析 (第55回日本小児血液・がん学会学術集会特集号) -- (シンポジウム 次世代シーケンサーによる小児血液,腫瘍疾患における研究の進展)
- 日本小児血液・がん学会雑誌 = Japanese journal of Pediatric Hematology/Oncology 51(5), 389-396, 2014
- NAID 40020343530
Related Links
- Acute megakaryocytic leukemia (AMegL) is a rare subtype of acute myeloid leukemia (AML) evolving from primitive megakaryoblasts. Because of its rarity and the lack of precise diagnostic criteria in the past, few series ...
- Acute myeloblastic leukemia type 7; Acute megakaryocytic leukemia; Acute myeloid leukemia M7; Acute myeloblastic leukemia type 7; Acute megakaryocytic leukemia ... ClinicalTrials.gov lists trials that are studying or have ...
Related Pictures
★リンクテーブル★
[★]
- 英
- acute megakaryocytic leukemia AMgL
- 同
- M7
- 関
- 急性巨核芽球性白血病、巨核球性白血病、急性骨髄性白血病
疫学
- ダウン症候群に合併する白血病のうち最も多い → これは治療に反応し予後良好
検査
- 光顕的MPO陰性。電顕的MPO陽性。
- アウエル小体-
- フローサイトメトリー:CD41陽性, CD42陽性
- 血算:血球減少(しかし30%の患者では血小板数は10万を超えている) (WCH.2113)
- 血小板機能:凝集能低下
- 血液生化学:LDH上昇
- X線写真:骨融解像、骨硬化像が認められる
合併症
予後
参考
- http://clinicalflow.com/Cases/Case_List/Acute_Myelogenous_Leukemia_(AML)_not_otherwise_categorized_-_General_Information/Acute_Megakaryoblastic_Leukemia_(AMKL)_(M7)
[★]
- 英
- acute megakaryoblastic leukemia、acute megakaryocytic leukemia
- 同
- M7]]
- 関
- 急性巨核球性白血病、巨核球性白血病
[★]
急性巨核芽球性白血病
- 関
- acute megakaryocytic leukemia、megakaryocytic leukemia
[★]
- (疾患)急性の、急性型の、急性的な。(形状が)鋭い、鋭角の。(感覚、才知などが)鋭い。明敏な、鋭い眼識のある。
- 関
- acutely、quick、sharp
[★]
- 関
- megakaryocyte
[★]
巨核球白血病、骨髄巨核球白血病