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MYH9-related disorder

MedGen UID:
344306
Concept ID:
C1854520
Disease or Syndrome
Synonym: Sebastian Syndrome

Definition

An autosomal dominant disorder characterized by the triad of thrombocytopenia, giant platelets, and characteristic inclusions in peripheral blood leukocytes, without other organ dysfunction; it is associated with mutation of the MYH9 gene. [from NCI]

Professional guidelines

PubMed

Savige J, Ariani F, Mari F, Bruttini M, Renieri A, Gross O, Deltas C, Flinter F, Ding J, Gale DP, Nagel M, Yau M, Shagam L, Torra R, Ars E, Hoefele J, Garosi G, Storey H
Pediatr Nephrol 2019 Jul;34(7):1175-1189. Epub 2018 Jul 9 doi: 10.1007/s00467-018-3985-4. PMID: 29987460
Rabbolini DJ, Chun Y, Latimer M, Kunishima S, Fixter K, Valecha B, Tan P, Chew LP, Kile BT, Burt R, Radhakrishnan K, Bird R, Ockelford P, Gabrielli S, Chen Q, Stevenson WS, Ward CM, Morel-Kopp MC
Platelets 2018 Dec;29(8):793-800. Epub 2017 Nov 1 doi: 10.1080/09537104.2017.1356920. PMID: 29090586
Okano S, Takase M, Iseki K, Toriumi N, Kaneda M, Kunishima S
J Pediatr Hematol Oncol 2015 Aug;37(6):e352-5. doi: 10.1097/MPH.0000000000000379. PMID: 26056797

Recent clinical studies

Etiology

Costa RF, Bucharles SGE, Kagueiama DE, Kus CP, Andrade AA, Dirschnabel AJ, de Araujo MR, de Lima AAS
Spec Care Dentist 2022 Nov;42(6):651-656. Epub 2022 May 10 doi: 10.1111/scd.12727. PMID: 35537118
Yabe TE, King K, Russell S, Satgunaseelan L, Gupta R, Chen J, Ashford B
Diagn Pathol 2022 Feb 6;17(1):23. doi: 10.1186/s13000-022-01210-x. PMID: 35125114Free PMC Article
Tsang HC, Bussel JB, Mathew S, Liu YC, Imahiyerobo AA, Orazi A, Geyer JT
Mod Pathol 2017 Apr;30(4):486-498. Epub 2017 Jan 6 doi: 10.1038/modpathol.2016.218. PMID: 28059092
Kuzmanović M, Kunishima S, Putnik J, Stajić N, Paripović A, Bogdanović R
Vojnosanit Pregl 2014 Apr;71(4):395-8. doi: 10.2298/vsp121127001k. PMID: 24783421
Pujol-Moix N, Kelley MJ, Hernández A, Muñiz-Diaz E, Español I
Haematologica 2004 Mar;89(3):330-7. PMID: 15020273

Diagnosis

Sakamoto A, Uchiyama T, Kaname T, Iguchi A, Ohara O, Ishimura M, Onum M, Kunishima S, Ishiguro A
Br J Haematol 2024 Jun;204(6):2400-2404. Epub 2024 Apr 22 doi: 10.1111/bjh.19484. PMID: 38650331
Arif AR, Zhao M, Chen W, Xue M, Luo S, Wang Y
Platelets 2022 Nov 17;33(8):1307-1311. Epub 2022 Jul 5 doi: 10.1080/09537104.2022.2096211. PMID: 35791514
Bastida JM, Gonzalez-Porras JR, Rivera J, Lozano ML
Int J Mol Sci 2021 Apr 21;22(9) doi: 10.3390/ijms22094330. PMID: 33919295Free PMC Article
Bury L, Megy K, Stephens JC, Grassi L, Greene D, Gleadall N, Althaus K, Allsup D, Bariana TK, Bonduel M, Butta NV, Collins P, Curry N, Deevi SVV, Downes K, Duarte D, Elliott K, Falcinelli E, Furie B, Keeling D, Lambert MP, Linger R, Mangles S, Mapeta R, Millar CM, Penkett C, Perry DJ, Stirrups KE, Turro E, Westbury SK, Wu J, BioResource N, Gomez K, Freson K, Ouwehand WH, Gresele P, Simeoni I
Hum Mutat 2020 Jan;41(1):277-290. Epub 2019 Oct 15 doi: 10.1002/humu.23927. PMID: 31562665Free PMC Article
Kamath V, Gnanasekaran KK, Mammen J
Hematol Oncol Stem Cell Ther 2016 Dec;9(4):137-140. Epub 2016 Sep 2 doi: 10.1016/j.hemonc.2016.08.002. PMID: 27614228

Therapy

Li X, Wang W, Ni X, Cheng D, Chen J
J Nephrol 2023 Jul;36(6):1707-1709. Epub 2023 May 31 doi: 10.1007/s40620-023-01651-7. PMID: 37258992
Hu Y, Yang C, Amorim T, Maqbool M, Lin J, Li C, Fang C, Xue L, Kwart A, Fang H, Yin M, Janocha AJ, Tsuchimoto D, Nakabeppu Y, Jiang X, Mejia-Garcia A, Anwer F, Khouri J, Qi X, Zheng QY, Yu JS, Yan S, LaFramboise T, Anderson KC, Herlitz LC, Munshi NC, Lin J, Zhao J
Cancer Res 2021 Feb 1;81(3):713-723. Epub 2020 Dec 7 doi: 10.1158/0008-5472.CAN-20-1010. PMID: 33288657Free PMC Article

Prognosis

Bury L, Megy K, Stephens JC, Grassi L, Greene D, Gleadall N, Althaus K, Allsup D, Bariana TK, Bonduel M, Butta NV, Collins P, Curry N, Deevi SVV, Downes K, Duarte D, Elliott K, Falcinelli E, Furie B, Keeling D, Lambert MP, Linger R, Mangles S, Mapeta R, Millar CM, Penkett C, Perry DJ, Stirrups KE, Turro E, Westbury SK, Wu J, BioResource N, Gomez K, Freson K, Ouwehand WH, Gresele P, Simeoni I
Hum Mutat 2020 Jan;41(1):277-290. Epub 2019 Oct 15 doi: 10.1002/humu.23927. PMID: 31562665Free PMC Article
Tsang HC, Bussel JB, Mathew S, Liu YC, Imahiyerobo AA, Orazi A, Geyer JT
Mod Pathol 2017 Apr;30(4):486-498. Epub 2017 Jan 6 doi: 10.1038/modpathol.2016.218. PMID: 28059092
Pujol-Moix N, Kelley MJ, Hernández A, Muñiz-Diaz E, Español I
Haematologica 2004 Mar;89(3):330-7. PMID: 15020273

Clinical prediction guides

Wang L, Huang J, Li X
Clin Lab 2024 Mar 1;70(3) doi: 10.7754/Clin.Lab.2023.230918. PMID: 38469783
Arif AR, Zhao M, Chen W, Xue M, Luo S, Wang Y
Platelets 2022 Nov 17;33(8):1307-1311. Epub 2022 Jul 5 doi: 10.1080/09537104.2022.2096211. PMID: 35791514
Huang YC, Shih YH, Lin CY, Chiu PF, Kuo SF, Lin JS, Shen MC
Int J Hematol 2020 Dec;112(6):878-882. Epub 2020 Jul 25 doi: 10.1007/s12185-020-02947-1. PMID: 32712863
Bury L, Megy K, Stephens JC, Grassi L, Greene D, Gleadall N, Althaus K, Allsup D, Bariana TK, Bonduel M, Butta NV, Collins P, Curry N, Deevi SVV, Downes K, Duarte D, Elliott K, Falcinelli E, Furie B, Keeling D, Lambert MP, Linger R, Mangles S, Mapeta R, Millar CM, Penkett C, Perry DJ, Stirrups KE, Turro E, Westbury SK, Wu J, BioResource N, Gomez K, Freson K, Ouwehand WH, Gresele P, Simeoni I
Hum Mutat 2020 Jan;41(1):277-290. Epub 2019 Oct 15 doi: 10.1002/humu.23927. PMID: 31562665Free PMC Article
Pujol-Moix N, Kelley MJ, Hernández A, Muñiz-Diaz E, Español I
Haematologica 2004 Mar;89(3):330-7. PMID: 15020273

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