Nationwide epidemiological survey of familial myelodysplastic syndromes/acute myeloid leukemia in Japan: a multicenter retrospective study.

Leukemia & Lymphoma
Kensuke TakaokaM Kurokawa

Abstract

Although several pedigrees of familial myelodysplastic syndromes/acute myeloid leukemia (fMDS/AML) have been reported, the epidemiology and clinical features has been poorly understood. To explore the epidemiology of this entity, we performed a retrospective nationwide epidemiological survey in Japan using questionnaire sheets. The questionnaire was sent to 561 institutions or hospitals certified by Japanese Society of Hematology, unearthing the existence of 41 pedigrees of fMDS/AML. Among them, we obtained the clinical information of 31 patients in 20 pedigrees. The median age of the initial diagnosis was 51 years (range 9-88 years) and the WHO classification 2008 ranged from refractory anemia (RA) to AML. Focusing on the familial MDS patients, refractory anemia with excess blasts (RAEB)-2 was the largest group (27.3%). The median overall survival (OS) of fMDS and fAML in this study were 71.6 and 12.4 months, and the five-year OS were 61.3 and 50%, respectively.

References

Dec 3, 2004·The New England Journal of Medicine·Matthew L SmithJude Fitzgibbon
Jan 5, 2008·British Journal of Haematology·Carolyn OwenJude Fitzgibbon
Feb 1, 2008·Bulletin of the World Health Organization·Guy Hutton, Jamie Bartram
Feb 1, 2008·Bulletin of the World Health Organization·Stephen R C HowieRichard A Adegbola
Mar 7, 2008·Haematologica·Eva MontanéUNKNOWN Catalan Group for Study of Agranulocytosis and Aplastic Anemia
Apr 23, 2009·Blood·Richard M Stone
May 25, 2011·Haematologica·Elena Liew, Carolyn Owen
Dec 14, 2011·Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology·Lynn R GoldinMagnus Björkholm
Mar 16, 2012·The New England Journal of Medicine·Matthew J WalterTimothy A Graubert
Apr 27, 2012·British Journal of Haematology·Harriet HolmeInderjeet Dokal
May 1, 2012·American Journal of Human Genetics·Michael KirwanInderjeet Dokal
Jun 22, 2013·British Journal of Haematology·Anjali ShahPaul C Lambert
Aug 9, 2013·Therapeutic Advances in Hematology·Eric M NickelsLucy A Godley
Jan 29, 2014·Annals of the New York Academy of Sciences·Allison H WestJane E Churpek
Sep 23, 2014·Blood·Rafael Bejar, David P Steensma
Jan 13, 2015·Nature Genetics·Michael Y ZhangAkiko Shimamura
Apr 30, 2015·Cancer Cell·Chantana PolprasertJaroslaw P Maciejewski
May 13, 2017·International Journal of Hematology·Hiroshi KawabataUNKNOWN Japanese National Research Group on Idiopathic Bone Marrow Failure Syndromes

❮ Previous
Next ❯

Related Concepts

Related Feeds

Acute Myeloid Leukemia

Acute myeloid leukemia (AML) is a clinically and genetically heterogeneous disease with approximately 20,000 cases per year in the United States. AML also accounts for 15-20% of all childhood acute leukemias, while it is responsible for more than half of the leukemic deaths in these patients. Here is the latest research on this disease.

CNS Lymphoma

In CNS lymphoma, cancerous cells from lymph tissues or other parts of the body form tumors in the brain and/or spinal cord. Here is the latest research on this rare non-Hodgkin lymphoma.

AML: Role of LSD1 by CRISPR (Keystone)

Find the latest rersearrch on the ability of CRISPR-Cas9 mutagenesis to profile the interactions between lysine-specific histone demethylase 1 (LSD1) and chemical inhibitors in the context of acute myeloid leukemia (AML) here.