WordNet

not representative of a group, class, or type; "a group that is atypical of the target audience"; "a class of atypical mosses"; "atypical behavior is not the accepted type of response that we expect from children" (同)untypical
deviating from normal expectations; somewhat odd, strange, or abnormal; "these days large families are atypical"; "atypical clinical findings"; "atypical pneumonia"; "highly irregular behavior" (同)irregular
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
of or relating to the spinal cord
of or relating to bone marrow
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

PrepTutorEJDIC

型にはまらない,異常な
(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
白血病

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/02/26 18:04:06」(JST)

wiki en

Atypical chronic myeloid leukemia (aCML)^[1] is a type of leukemia. It is a heterogeneous disorder belonging to the group of myelodysplastic/myeloproliferative (MDS/MPN) syndromes.

In aCML many clinical features (splenomegaly, myeloid predominance in the bone marrow with some dysplastic features but without a differentiation block) and laboratory abnormalities (myeloid proliferation, low leukocyte alkaline phosphatase values) suggest the diagnosis of chronic myelogenous leukemia (CML). However the lack of the pathognomonic Philadelphia chromosome^[2] and of the resulting BCR-ABL1 fusion point to a different pathogenetic process. Since no specific recurrent genomic or karyotypic abnormalities have been identified in aCML, the molecular pathogenesis of this disease has remained elusive and the outcome dismal (median survival 37 months)^[3]with no improvement over the last 20 years. This sharply contrasts with the outcome for CML, for which the prognosis was dramatically improved by the development of imatinib as a specific inhibitor of the BCR-ABL protein and in particular for CML.^[4]^[5]^[6]^[7]

In 2012 SETBP1 was identified as a novel oncogene in aCML; specific somatic mutations of this gene were discovered in people with aCML and related diseases. These mutations, which are identical to the ones present in SGS as germline mutations, impair the degradation of SETBP1 and therefore cause increased cellular levels of the protein.^[8]

References

^ Vardiman, J.W. et al. The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changes. Blood 114, 937-51 (2009).
^ Nowell, P.C. & Hungerford, D.A. National Academy of Sciences. A minute Chromosome in Human Chronic Granulocytic Leukemia. Science 132, 1497-1497 (1960)
^ Kurzrock, R. et al. BCR rearrangement-negative chronic myelogenous leukemia revisited. J Clin Oncol. 19, 2915-26 (2001).
^ Druker, B.J. et al. Efficacy and safety of a specific inhibitor of the BCR-ABL tyrosine kinase in chronic myeloid leukemia. N Engl J Med 344, 1031-7 (2001).
^ Rebora, P. et al. Are chronic myeloid leukemia patients more at risk for second malignancies? A population-based study. Am J Epidemiol 172, 1028-33 (2010).
^ Gambacorti-Passerini, C. et al. Multicenter independent assessment of outcomes in chronic myeloid leukemia patients treated with imatinib. J Natl Cancer Inst. 103., 553-61 (2011).
^ Goldman, J.M. Chronic myeloid leukemia: a historical perspective. Semin Hematol 47, 302-11 (2010).
^ Piazza R, Valletta S, Winkelmann N, Redaelli S, Spinelli R, Pirola A, Antolini L, Mologni L, Donadoni C, Papaemmanuil E, Schnittger S, Kim DW, Boultwood J, Rossi F, Gaipa G, De Martini GP, di Celle PF, Jang HG, Fantin V, Bignell GR, Magistroni V, Haferlach T, Pogliani EM, Campbell PJ, Chase AJ, Tapper WJ, Cross NC, Gambacorti-Passerini C. Recurrent SETBP1 mutations in atypical chronic myeloid leukemia. Nat Genet. 2013 Jan;45(1):18-24. doi: 10.1038/ng.2495. Epub 2012 Dec 9.

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1. 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
2. 慢性骨髄性白血病の治療の概要 overview of the treatment of chronic myeloid leukemia
3. 慢性骨髄性白血病の分子遺伝学 molecular genetics of chronic myeloid leukemia
4. 慢性期の慢性骨髄性白血病の初期治療 initial treatment of chronic myeloid leukemia in chronic phase
5. 血小板増加症患者に対するアプローチ approach to the patient with thrombocytosis

English Journal

ST1926, an orally active synthetic retinoid, induces apoptosis in chronic myeloid leukemia cells and prolongs survival in a murine model.

Nasr RR1, Hmadi RA2, El-Eit RM1, Iskandarani AN1, Jabbour MN3, Zaatari GS3, Mahon FX4, Pisano CC5, Darwiche ND2.
International journal of cancer. Journal international du cancer.Int J Cancer.2015 Aug 1;137(3):698-709. doi: 10.1002/ijc.29407. Epub 2015 Jan 21.
The tyrosine kinase inhibitor, imatinib, is the first line of treatment for chronic myeloid leukemia (CML) patients. Unfortunately, patients develop resistance and relapse due to bcr-abl point mutations and the persistence of leukemia initiating cells (LIC). Retinoids regulate vital biological proce
PMID 25557649

Evaluation of a father and son with atypical chronic myeloid leukemia with SETBP1 mutations and a review of the literature.

Wang L1, Du F2, Zhang HM1, Wang HX1.
Brazilian journal of medical and biological research = Revista brasileira de pesquisas médicas e biológicas / Sociedade Brasileira de Biofísica ... [et al.].Braz J Med Biol Res.2015 Jul;48(7):583-7. doi: 10.1590/1414-431X20154557. Epub 2015 May 26.
We report the case of a father and son diagnosed with atypical chronic myeloid leukemia (aCML). Both patients harbored SETBP1 mutations, which are present in 24.3% of aCML patients. Moreover, both shared the variant encoding p.Pro737His, but the aCML severity was greater in the son because of the pr
PMID 26017341

[Molecular characterization of atypical chronic myeloid leukemia and chronic neutrophilic leukemia].

Senín A1, Arenillas L2, Martínez-Avilés L3, Fernández-Rodríguez C3, Bellosillo B3, Florensa L2, Besses C1, Álvarez-Larrán A4.
Medicina clínica.Med Clin (Barc).2015 Jun 8;144(11):487-90. doi: 10.1016/j.medcli.2014.03.020. Epub 2014 May 20.
BACKGROUND AND OBJECTIVE: Atypical chronic myeloid leukemia (aCML) and chronic neutrophilic leukemia (CNL) display similar clinical and hematological characteristics. The objective of the present study was to determine the mutational status of SETBP1 and CSF3R in these diseases.PATIENTS AND METHOD:
PMID 24854193

Japanese Journal

慢性好中球性白血病と非定型慢性骨髄性白血病(BCR-ABL陰性)におけるCSF3R変異

桐戸敬太
血液内科 68(3), 387-391, 2014-03
NAID 40020030484

Successful transplantation of ethnically mismatched cord blood in a boy with atypical chronic myeloid leukemia

JAING Tang-Her,HUNG Iou-Jih,CHEN Shih-Hsiang,LEE Wen-I,WEN Yu-Chuan,FANG En-Chen
International journal of hematology 97(1), 144-146, 2013-01-01
NAID 10031144647

巨赤芽球性貧血を合併したため定型的病像を欠いた慢性骨髄性白血病

小磯博美,塚本憲史,島野俊一,唐沢正光,村上博和,野島美久
臨床血液 52(11), 1772-1776, 2011
症例は61歳男性。統合失調症で通院中であった。2007年11月貧血のため，当院紹介。WBC 8,660/μl, Hb 6.0 g/dl, MCV 114 fl, MCH 38.6 pg, PLT 10.0×104/μl, LDH 1,236 IU/lと大球性貧血，血小板減少があり，過分葉好中球，骨髄芽球，巨大桿状核球，有核赤血球を認めた。骨髄は過形成であった。ビタミンB12と葉酸は低値で，巨赤芽球 …
NAID 130004501498

「非定型慢性骨髄性白血病」

　　[★]

英: atypical chronic myeloid leukemia
関: 慢性骨髄性白血病

[show details]

非定型慢性骨髄性白血病 : 59 件
非定形慢性骨髄性白血病 : 1 件

「非定型性慢性骨髄性白血病」

　　[★]

英: atypical chronic myeloid leukemia
同: aCML

「atypical」

　　[★]

adj.

非定型の, 非定型的な, 非定型性の, 異型の

関: atypia、atypically、atypism、meta、nonclassic、off-type、variant

「myeloid」

　　[★]

adj.

骨髄の、骨髄性の、骨髄球性の、ミエロイド

関: BM、bone marrow、myelo、myelocytic、myelogenous

「chronic」

　　[★]

adj.

慢性の、慢性的な、慢性型の

関: chronically、chronicity

「myelo」

　　[★]

comb form

骨髄

ex.

myelopathy, myeloid

[1] Vardiman, J.W. et al. The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changes. Blood 114, 937-51 (2009).

[2] Nowell, P.C. & Hungerford, D.A. National Academy of Sciences. A minute Chromosome in Human Chronic Granulocytic Leukemia. Science 132, 1497-1497 (1960)

[3] Kurzrock, R. et al. BCR rearrangement-negative chronic myelogenous leukemia revisited. J Clin Oncol. 19, 2915-26 (2001).

[4] Druker, B.J. et al. Efficacy and safety of a specific inhibitor of the BCR-ABL tyrosine kinase in chronic myeloid leukemia. N Engl J Med 344, 1031-7 (2001).

[5] Rebora, P. et al. Are chronic myeloid leukemia patients more at risk for second malignancies? A population-based study. Am J Epidemiol 172, 1028-33 (2010).

[6] Gambacorti-Passerini, C. et al. Multicenter independent assessment of outcomes in chronic myeloid leukemia patients treated with imatinib. J Natl Cancer Inst. 103., 553-61 (2011).

[7] Goldman, J.M. Chronic myeloid leukemia: a historical perspective. Semin Hematol 47, 302-11 (2010).

[8] Piazza R, Valletta S, Winkelmann N, Redaelli S, Spinelli R, Pirola A, Antolini L, Mologni L, Donadoni C, Papaemmanuil E, Schnittger S, Kim DW, Boultwood J, Rossi F, Gaipa G, De Martini GP, di Celle PF, Jang HG, Fantin V, Bignell GR, Magistroni V, Haferlach T, Pogliani EM, Campbell PJ, Chase AJ, Tapper WJ, Cross NC, Gambacorti-Passerini C. Recurrent SETBP1 mutations in atypical chronic myeloid leukemia. Nat Genet. 2013 Jan;45(1):18-24. doi: 10.1038/ng.2495. Epub 2012 Dec 9.

リンク元	「非定型慢性骨髄性白血病」「非定型性慢性骨髄性白血病」
関連記事	「atypical」「myeloid」「chronic」「myelo」

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案内

atypical chronic myeloid leukemia