ミエロカセキシス
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出典(authority):フリー百科事典『ウィキペディア(Wikipedia)』「2015/05/01 15:34:23」(JST)
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| Myelokathexis | 
| Classification and external resources | 
| ICD-9 | 
288.0[1] | 
| DiseasesDB | 
32164 | 
Myelokathexis is a congenital disorder of the white blood cells that causes severe, chronic leukopenia (a reduction of circulating white blood cells) and neutropenia (a reduction of neutrophil granulocytes).[2] The disorder is believed to be inherited in an autosomal dominant manner.[2][3] Myelokathexis refers to retention (kathexis) of neutrophils in the bone marrow (myelo).
Treatment
Myelokathexis is amongst the diseases treated with bone marrow transplantation and cord blood stem cells.[citation needed]
References
- ^ "ICD-9-CM Disease Index: M". Retrieved 2008-02-17. 
 
- ^ a b Aprikyan AA, Liles WC, Park JR, Jonas M, Chi EY, Dale DC (Jan 2000). "Myelokathexis, a congenital disorder of severe neutropenia characterized by accelerated apoptosis and defective expression of bcl-x in neutrophil precursors" (FREE FULL TEXT). Blood 95 (1): 320–327. PMID 10607719. 
 
- ^ Hord, JD; Whitlock, JA; Gay, JC; Lukens, JN (Sep–Oct 1997). "Clinical features of myelokathexis and treatment with hematopoietic cytokines: a case report of two patients and review of the literature". Journal of Pediatric Hematology/Oncology : official journal of the American Society of Pediatric Hematology/Oncology 19 (5): 443–448. PMID 9329467.  edit
 
 
See also
- WHIM syndrome Warts, hypogammaglobulinemia, infections, and myelokathexis OMIM: 193670
 
 
UpToDate Contents
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English Journal
- Molecular Pathways: Targeting the CXCR4-CXCL12 Axis-Untapped Potential in the Tumor Microenvironment.
 
- Scala S1.
 
- Clinical cancer research : an official journal of the American Association for Cancer Research.Clin Cancer Res.2015 Oct 1;21(19):4278-85. doi: 10.1158/1078-0432.CCR-14-0914. Epub  2015 Jul 21.
 
- Evidence suggests that the CXC-chemokine receptor-4 pathway plays a role in cancer cell homing and metastasis, and thus represents a potential target for cancer therapy. The homeostatic microenvironment chemokine CXCL12 binds the CXCR4 and CXCR7 receptors, activating divergent signals on multiple pa
 
- PMID 26199389
 
- How I treat Waldenström macroglobulinemia.
 
- Treon SP1.
 
- Blood.Blood.2015 Aug 6;126(6):721-32. doi: 10.1182/blood-2015-01-553974. Epub  2015 May 22.
 
- Waldenström macroglobulinemia (WM) is a B-cell neoplasm manifested by the accumulation of clonal immunoglobulin (Ig)M-secreting lymphoplasmacytic cells. MYD88 and CXCR4 warts, hypogammaglobulinemia, infections, myelokathexis syndrome-like somatic mutations are present in >90% and 30% to 35% of W
 
- PMID 26002963
 
- MYD88 L265P and CXCR4 mutations in lymphoplasmacytic lymphoma identify cases with high disease activity.
 
- Schmidt J1, Federmann B1, Schindler N1, Steinhilber J1, Bonzheim I1, Fend F1, Quintanilla-Martinez L1.
 
- British journal of haematology.Br J Haematol.2015 Jun;169(6):795-803. doi: 10.1111/bjh.13361. Epub  2015 Mar 29.
 
- Recurrent mutations in MYD88 have been identified in >90% of lymphoplasmacytic lymphoma (LPL). Recently, WHIM (warts, hypogammaglobulinaemia, infections, myelokathexis) syndrome-like mutations in CXCR4 have been described in 28% of LPL cases, and seem to impact clinical presentation and response 
 
- PMID 25819228
 
Japanese Journal
- 好中球減少で発見されたWHIM症候群 (Myelokathexis) の幼児例
 
- Altered leukocyte response to CXCL12 in patients with warts hypogammaglobulinemia, infections, myelokathexis (WHIM) syndrome
 
Related Links
- ^ICD-9-CM Disease Index: M". http://www.dbmi.columbia.edu/hripcsak/icd9/2indexm.html. Retrieved 2008-02-17. ^ a b Aprikyan AA, Liles WC, Park JR, Jonas M, Chi EY, Dale DC (Jan 2000). "Myelokathexis, a congenital disorder of ...
 
- Looking for online definition of myelokathexis in the Medical Dictionary? myelokathexis explanation free. What is myelokathexis? Meaning of myelokathexis medical term. What does myelokathexis mean? http://medical Bing ? ...
 
Related Pictures







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- neutropenia
 
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- 好中球減少、顆粒球減少症 granulocytopenia
 
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- 好中球
 
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- WHIM syndrome
 
- 同
 
- いぼ・低γグロブリン血・感染・骨髄性細胞貯留症候群 Warts-Hypogammaglobulinemia-Infection-Myelokathexis syndrome
 
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- WHIM症候群