Literature DB >> 9202609

Occurrence of myeloproliferative disorder in patients with Noonan syndrome.

B Bader-Meunier1, G Tchernia, F Miélot, J L Fontaine, C Thomas, S Lyonnet, J M Lavergne, J P Dommergues.   

Abstract

We report four cases of Noonan syndrome associated with chronic myelomonocytic leukemia in childhood. These children shared some hematologic features: thrombocytopenia, splenomegaly in the first months of life, occurrence of chronic myelomonocytic leukemia without abnormalities of the initial bone marrow karyotype, and, in three cases, improvement of the hematologic disease. A common pathophysiologic process in such patients is suggested.

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Year:  1997        PMID: 9202609     DOI: 10.1016/s0022-3476(97)70273-7

Source DB:  PubMed          Journal:  J Pediatr        ISSN: 0022-3476            Impact factor:   4.406


  41 in total

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Authors:  M Tartaglia; G Zampino; B D Gelb
Journal:  Mol Syndromol       Date:  2010-01-15

Review 2.  JMML genomics and decisions.

Authors:  Charlotte M Niemeyer
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Authors:  Christian P Kratz; Suthee Rapisuwon; Helen Reed; Henrik Hasle; Philip S Rosenberg
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6.  Molecular basis of juvenile myelomonocytic leukemia.

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Journal:  Haematologica       Date:  2010-02       Impact factor: 9.941

7.  Mutations in RIT1 cause Noonan syndrome with possible juvenile myelomonocytic leukemia but are not involved in acute lymphoblastic leukemia.

Authors:  Hélène Cavé; Aurélie Caye; Nehla Ghedira; Yline Capri; Nathalie Pouvreau; Natacha Fillot; Aurélien Trimouille; Cédric Vignal; Odile Fenneteau; Yves Alembik; Jean-Luc Alessandri; Patricia Blanchet; Odile Boute; Patrice Bouvagnet; Albert David; Anne Dieux Coeslier; Bérénice Doray; Olivier Dulac; Valérie Drouin-Garraud; Marion Gérard; Delphine Héron; Bertrand Isidor; Didier Lacombe; Stanislas Lyonnet; Laurence Perrin; Marlène Rio; Joëlle Roume; Sylvie Sauvion; Annick Toutain; Catherine Vincent-Delorme; Marjorie Willems; Clarisse Baumann; Alain Verloes
Journal:  Eur J Hum Genet       Date:  2016-01-13       Impact factor: 4.246

8.  Shp2 and Pten have antagonistic roles in myeloproliferation but cooperate to promote erythropoiesis in mammals.

Authors:  Helen He Zhu; Xiaolin Luo; Kaiqing Zhang; Jian Cui; Huifang Zhao; Zhongzhong Ji; Zhicheng Zhou; Jufang Yao; Lifan Zeng; Kaihong Ji; Wei-Qiang Gao; Zhong-Yin Zhang; Gen-Sheng Feng
Journal:  Proc Natl Acad Sci U S A       Date:  2015-10-12       Impact factor: 11.205

9.  PTPN11 is the first identified proto-oncogene that encodes a tyrosine phosphatase.

Authors:  Rebecca J Chan; Gen-Sheng Feng
Journal:  Blood       Date:  2006-10-19       Impact factor: 22.113

10.  Severe congenital thrombocytopaenia--first clinical manifestation of Noonan syndrome.

Authors:  Paula Nunes; Sara Aguilar; Sara Noéme Prado; Maria João Palaré; Anabela Ferrão; Anabela Morais
Journal:  BMJ Case Rep       Date:  2012-03-20
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