慢性骨髄増殖性疾患
WordNet
- bring disorder to (同)disarray
- a physical condition in which there is a disturbance of normal functioning; "the doctor prescribed some medicine for the disorder"; "everyone gets stomach upsets from time to time" (同)upset
- a disturbance of the peace or of public order
- being long-lasting and recurrent or characterized by long suffering; "chronic indigestion"; "a chronic shortage of funds"; "a chronic invalid"
- of long duration; "chronic money problems" (同)continuing
- habitual; "a chronic smoker" (同)inveterate
PrepTutorEJDIC
- 〈U〉『無秩序』,混乱,乱雑(confusion) / 《しばしば複数形で》(社会的・政治的な)粉争,騒動 / 〈C〉(肉体的・精神的な)不調,異常,障害 / …‘の'秩序を乱す / 〈心身〉‘に'異常を起こさせる
- (病気が)長期にわたる,慢性の / 《名詞の前にのみ用いて》常習の,癖になった
UpToDate Contents
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- 1. 骨髄増殖性腫瘍の概要 overview of the myeloproliferative neoplasms
- 2. 原発性骨髄線維症の臨床症状および診断 clinical manifestations and diagnosis of primary myelofibrosis
- 3. 本態性血小板血症の診断および臨床症状 diagnosis and clinical manifestations of essential thrombocythemia
- 4. 真性多血症の臨床症状および診断 clinical manifestations and diagnosis of polycythemia vera
- 5. 慢性骨髄性白血病の臨床症状および診断 clinical manifestations and diagnosis of chronic myeloid leukemia
English Journal
- Sensitivity of hematological malignancies to graft-versus-host effects: an EBMT megafile analysis.
- Stern M1, de Wreede LC2, Brand R2, van Biezen A3, Dreger P4, Mohty M5, de Witte TM6, Kröger N7, Ruutu T8.Author information 1Department of Biomedicine, University Hospital Basel and University of Basel, Basel, Switzerland.2Department of Medical Statistics and Bioinformatics, Leiden University Medical Center, Netherlands.3EBMT Data Office, University Medical Centre, Leiden, Leiden, Netherlands.4Medizinische Klinik V, Universität Heidelberg, Heidelberg, Germany.5Department of Hematology, CHU Nantes, Nantes, France.6Radboud University Nijmegen Medical Center, Nijmegen, Netherlands.7Department of Stem Cell Transplantation, University Medical Center, Hamburg Eppendorf, Hamburg, Germany.8Department of Medicine, Helsinki University Central Hospital, Helsinki, Finland.AbstractAfter allogeneic stem cell transplantation, graft-versus-host disease (GvHD) occurs through recognition of histocompatibility mismatches by donor T lymphocytes. The same mechanism operates in eliminating malignant cells (the graft-versus-tumor or GvT effect). We hypothesized that comparing the correlation between GvHD and relapse might provide a surrogate marker for the susceptibility of diseases to allo-immune effects. We studied 48,111 first allogeneic transplants performed between 1998 and 2007. In chronic myeloid leukemia (CML), the relapse risk declined clearly and proportionally to severity of acute and chronic GvHD. Acute lymphoblastic leukemia and BCR-ABL negative myeloproliferative neoplasias were comparably sensitive to GvHD as CML, whereas myelodysplastic syndromes and lymphoproliferative disorders showed intermediate sensitivity. GvHD was only associated with modest reductions in relapse risk in acute myeloid leukemia (AML) and plasma cell disorders (PCD). Except for PCD, hazard rates for relapse decreased to almost zero at 48 months of follow-up in all diseases. These data confirm observations of potent GvT effects associated with GvHD. The strength of the GvHD/GvT correlation differs significantly between hematological malignancies. The parallel drop of relapse rates in different diseases despite differences in GvHD/GvT ratios suggests that GvT effects might operate in the absence of GvHD, particularly in AML.Leukemia accepted article preview online, 30 April 2014; doi:10.1038/leu.2014.145.
- Leukemia.Leukemia.2014 Apr 30. doi: 10.1038/leu.2014.145. [Epub ahead of print]
- After allogeneic stem cell transplantation, graft-versus-host disease (GvHD) occurs through recognition of histocompatibility mismatches by donor T lymphocytes. The same mechanism operates in eliminating malignant cells (the graft-versus-tumor or GvT effect). We hypothesized that comparing the corre
- PMID 24781016
- Molecular Pathways: Molecular Basis for Sensitivity and Resistance to JAK Kinase Inhibitors.
- Meyer SC1, Levine RL.Author information 1Authors' Affiliations: Human Oncology and Pathogenesis Program and Leukemia Service, Department of Medicine, Memorial Sloan-Kettering Cancer Center, New York, New York.AbstractJanus-activated kinases (JAK) are the mediators of a variety of cytokine signals via their cognate receptors that result in activation of intracellular signaling pathways. Alterations in JAK1, JAK2, JAK3, and TYK2 signaling contribute to different disease states, and dysregulated JAK-STAT signaling is associated with hematologic malignancies, autoimmune disorders, and immune-deficient conditions. Genetic alterations of JAK2 occur in the majority of patients with myeloproliferative neoplasms and occur in a subset of patients with acute leukemias. JAK-mediated signaling critically relies on STAT transcription factors, and on activation of the MAPK and PI3K/Akt signaling axes. Hyperactive JAK at the apex of these potent oncogenic signaling pathways therefore represents an important target for small-molecule kinase inhibitors in different disease states. The JAK1/2 inhibitor ruxolitinib and the JAK3 inhibitor tofacitinib were recently approved for the treatment of myelofibrosis and rheumatoid arthritis, respectively, and additional ATP-competitive JAK inhibitors are in clinical development. Although these agents show clinical activity, the ability of these JAK inhibitors to induce clinical/molecular remissions in hematologic malignancies seems limited and resistance upon chronic drug exposure is seen. Alternative modes of targeting JAK2 such as allosteric kinase inhibition or HSP90 inhibition are under evaluation, as is the use of histone deacetylase inhibitors. Combination therapy approaches integrating inhibition of STAT, PI3K/Akt, and MAPK pathways with JAK kinase inhibitors might be critical to overcome malignancies characterized by dysregulated JAK signaling. Clin Cancer Res; 20(8); 2051-9. ©2014 AACR.
- Clinical cancer research : an official journal of the American Association for Cancer Research.Clin Cancer Res.2014 Apr 15;20(8):2051-9. doi: 10.1158/1078-0432.CCR-13-0279. Epub 2014 Feb 28.
- Janus-activated kinases (JAK) are the mediators of a variety of cytokine signals via their cognate receptors that result in activation of intracellular signaling pathways. Alterations in JAK1, JAK2, JAK3, and TYK2 signaling contribute to different disease states, and dysregulated JAK-STAT signaling
- PMID 24583800
- A highly specific q-RT-PCR assay to address the relevance of the JAK2WT and JAK2V617F expression levels and control genes in Ph-negative myeloproliferative neoplasms.
- Fantasia F1, Di Capua EN, Cenfra N, Pessina G, Mecarocci S, Rago A, Cotroneo E, Busanello A, Equitani F, Lo-Coco F, Nervi C, Cimino G.Author information 1Department of Medico-Surgical Sciences and Biotechnologies, University of Rome "Sapienza" Polo Pontino, , Latina, Italy.AbstractIn Ph- myeloproliferative neoplasms, the quantification of the JAK2V617F transcripts may provide some advantages over the DNA allele burden determination. We developed a q-RT-PCR to assess the JAK2WT and JAK2V617F mRNA expression in 105 cases (23 donors, 13 secondary polycythemia, 22 polycythemia vera (PV), 38 essential thrombocythemia (ET), and 9 primary myelofibrosis (PMF)). Compared with the standard allele-specific oligonucleotide (ASO)-PCR technique, our assay showed a 100 % concordance rate detecting the JAK2V617F mutation in 22/22 PV (100 %), 29/38 (76.3 %) ET, and 5/9 (55.5 %) PMF cases, respectively. The sensitivity of the assay was 0.01 %. Comparing DNA and RNA samples, we found that the JAK2V617F mutational ratios were significantly higher at the RNA level both in PV (p = 0.005) and ET (p = 0.001) samples. In PV patients, JAK2WT expression levels positively correlated with the platelets (PLTs) (p = 0.003) whereas a trend to negative correlation was observed with the Hb levels (p = 0.051). JAK2V617F-positive cases showed the lowest JAK2WT and ABL1 mRNA expression levels. In all the samples, the expression pattern of beta-glucoronidase (GUSB) was more homogeneous than that of ABL1 or β2 microglobulin (B2M). Using GUSB as normalizator gene, a significant increase of the JAK2V617F mRNA levels was seen in two ET patients at time of progression to PV. In conclusion, the proposed q-RT-PCR is a sensitive and accurate method to quantify the JAK2 mutational status that can also show clinical correlations suggesting the impact of the residual amount of the JAK2WT allele on the Ph- MPN disease phenotype. Our observations also preclude the use of ABL1 as a housekeeping gene for these neoplasms.
- Annals of hematology.Ann Hematol.2014 Apr;93(4):609-16. doi: 10.1007/s00277-013-1920-0. Epub 2013 Oct 31.
- In Ph- myeloproliferative neoplasms, the quantification of the JAK2V617F transcripts may provide some advantages over the DNA allele burden determination. We developed a q-RT-PCR to assess the JAK2WT and JAK2V617F mRNA expression in 105 cases (23 donors, 13 secondary polycythemia, 22 polycythemia ve
- PMID 24173087
Japanese Journal
- 慢性骨髄増殖性疾患におけるJAK2 V617F変異解析の検討
- 影山 祐子,横田 浩充,佐藤 優実子,小野 佳一,矢冨 裕
- 医学検査 : 日本臨床衛生検査技師会誌 = The Japanese journal of medical technology 58(3), 248-251, 2009-03-25
- NAID 10026333043
- 國本 泰臣,長谷川 賢作,田口 大蔵,北野 博也
- Otology Japan 19(1), 64-68, 2009-03-25
- 腫瘤形成性白血病は顆粒球肉腫（Granulocytic sarcoma）や緑色腫（Chloroma）とも言われ、骨髄細胞由来の腫瘤形成性腫瘍と定義される。今回われわれは、化学療法によって完全寛解と判断されていたにもかかわらず、白血病細胞による側頭骨への腫瘤形成をきたした症例を経験したので報告する。症例は62 歳女性、右耳鳴と右自声強聴を主訴に当科受診した。当初はコレステリン肉芽腫として治療するも改 …
- NAID 10025822373
Related Links
- Myeloproliferative disorders are a group of diseases in which the bone marrow produces too many red blood cells, white blood cells, or platelets. ... Normally, the bone marrow makes stem cells (immature blood cells) that develop into ...
- Abstract The Philadelphia chromosome-negative chronic myeloproliferative disorders (CMPD), polycythemia vera (PV), essential thrombocythemia (ET) and chronic idiopathic myelofibrosis (IMF), have overlapping clinical ...
★リンクテーブル★
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- 英
- chronic idiopathic myelofibrosis, CIMF
- 関
- 骨髄線維症。骨髄増殖性疾患 MPD、慢性骨髄増殖性疾患 chronic myeloproliferative disorders CMPD
概念
クローン性の造血幹細胞の疾患で、骨髄の線維化、髄外造血、脾腫をきたし、病因は不明である。慢性特発性骨髄線維症は最も一般ではないCMPDの一つであり、特定のクローンのマーカーが無い場合、診断は困難である。これは骨髄の線維化や脾腫は多血症や慢性骨髄性白血病でもみられるためである。また骨髄の線維化は様々な疾患でみられるためである(骨髄線維症#病因)。 (HIM.674)
疫学
- 60歳以降。 ⇔ 他のCMPDにおけるや二次性の骨髄線維症では全年齢で起こりう
検査
末梢血
- IMD
- 髄外造血を反映。未分化な血球の末梢への出現。狭小化した細網系を血球が通過することによる血球の変形。
- 赤血球：正球性貧血。奇形赤血球(涙滴赤血球)、赤芽球
- 白血球：症例の半数で増加(1万-2万/μl)、減少例もあり。骨髄芽球、前骨髄球、骨髄球、後骨髄球などを認める。
- 血小板：正常～減少。形態異常(巨大血小板、小型の巨核球)
- 好中球アルカリホスファターゼスコア：上昇
骨髄穿刺
骨髄生検
- 線維増生著明
- 有核細胞減少、造血細胞減少
- 巨核球系細胞増加 (IMD)
鑑別診断(IMD.977)
[★]
- 英
- chronic myeloproliferative disorders CMPD
- 関
- 骨髄増殖性疾患
慢性骨髄増殖性疾患
HIM.672
[★]
- 英
- chronic myeloproliferative disorders
- 関
- 原発性骨髄線維症
- 関
[★]
- 障害：個人的苦痛や機能の障害があるので「疾病」とは言えるものの、その背景にある臓器障害がもう一つはっきりしない場合に用いられる。(PSY.9)
- an untidy state; a lack of order or organization (⇔order)
- violent behaviour of large groups of people
- an illness that cause a part of the body to stop functioning correctly
- disease <> illness <> disorder
- 乱す、乱雑にする。(人)の(心身の)調子を狂わせる。
[★]
- 関
- chronically、chronicity
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骨髄増殖性疾患 MDS