نتایج جستجو برای: congenital leukemia

تعداد نتایج: 399984  

Journal: :Current opinion in hematology 1997
O I Krijanovski C A Sieff

Diamond-Blackfan anemia (DBA) is a rare, congenital, hypoplastic anemia that usually presents in early infancy. Congenital anomalies, particularly of the head and upper limbs, are present in about a quarter of reported patients. The disease is characterized by a moderate-to-severe macrocytic anemia, occasional neutropenia or thrombocytosis, a normocellular bone marrow with erythroid hypoplasia,...

2015
Muhammad A Mir Samith T Kochuparambil Roshini S Abraham Vilmarie Rodriguez Matthew Howard Amy P Hsu Amie E Jackson Steven M Holland Mrinal M Patnaik

Guanine-adenine-thymine-adenine 2 (GATA2) mutated disorders include the recently described MonoMAC syndrome (Monocytopenia and Mycobacterium avium complex infections), DCML (dendritic cell, monocyte, and lymphocyte deficiency), familial MDS/AML (myelodysplastic syndrome/acute myeloid leukemia) (myeloid neoplasms), congenital neutropenia, congenital lymphedema (Emberger's syndrome), sensorineura...

Journal: :Blood 2013
Dahae Won So Youn Shin Chan-Jeoung Park Seongsoo Jang Hyun-Sook Chi Kyoo-Hyung Lee Jin-Ok Lee Eul-Ju Seo

Acute promyelocytic leukemia is characterized by the rearrangement of the retinoic acid receptor α (RARA) gene and its fusion with other genes. We report a novel case of variant acute promyelocytic leukemia with the karyotype der (2)t(2;17)(q32;q21). Array comparative genomic hybridization revealed distinct chromosome breakpoints within the RARA and oligonucleotide/oligosaccharide-binding fold ...

Journal: :Blood 2001
C A Tschan C Pilz C Zeidler K Welte M Germeshausen

Point mutations in the granulocyte colony-stimulating factor receptor (G-CSFR) gene have been linked to the development of secondary leukemia in patients with congenital neutropenia (CN). This report presents data on a now 18-year-old patient with CN who has received G-CSF treatment since 1989 and who developed acute myeloid leukemia (AML) in 1998. To evaluate whether there is an association be...

Journal: :Journal of the College of Physicians and Surgeons--Pakistan : JCPSP 2012
Naureen Mushtaq Rabia Wali Zehra Fadoo Ali Faisal Saleem

Fanconi anaemia (FA) is an autosomal recessive inherited disorder with progressive bone marrow failure, associated congenital malformation and solid and haematological malignancies. Acute myeloid leukemia is the commonest haematological malignancy followed by myelodysplastic syndrome in children with FA. FA transformed into acute lymphoblastic leukemia (ALL) is a rare phenomenon and one of the ...

2015
Naureen Mushtaq Aga Khan Rabia Wali Shaukat Khanum Zehra Fadoo Ali Faisal Saleem

Fanconi anaemia (FA) is an autosomal recessive inherited disorder with progressive bone marrow failure, associated congenital malformation and solid and haematological malignancies. Acute myeloid leukemia is the commonest haematological malignancy followed by myelodysplastic syndrome in children with FA. FA transformed into acute lymphoblastic leukemia (ALL) is a rare phenomenon and one of the ...

2010
Eleftheria Hatzimichael Mark Tuthill

More than 25,000 hematopoietic stem cell transplantations (HSCTs) are performed each year for the treatment of lymphoma, leukemia, immune-deficiency illnesses, congenital metabolic defects, hemoglobinopathies, and myelodysplastic and myeloproliferative syndromes. Before transplantation, patients receive intensive myeloablative chemoradiotherapy followed by stem cell "rescue." Autologous HSCT is...

Journal: :Blood 2014
Ruth Clifford Tania Louis Pauline Robbe Sam Ackroyd Adam Burns Adele T Timbs Glen Wright Colopy Helene Dreau Francois Sigaux Jean Gabriel Judde Margalida Rotger Amalio Telenti Yea-Lih Lin Philippe Pasero Jonathan Maelfait Michalis Titsias Dena R Cohen Shirley J Henderson Mark T Ross David Bentley Peter Hillmen Andrew Pettitt Jan Rehwinkel Samantha J L Knight Jenny C Taylor Yanick J Crow Monsef Benkirane Anna Schuh

SAMHD1 is a deoxynucleoside triphosphate triphosphohydrolase and a nuclease that restricts HIV-1 in noncycling cells. Germ-line mutations in SAMHD1 have been described in patients with Aicardi-Goutières syndrome (AGS), a congenital autoimmune disease. In a previous longitudinal whole genome sequencing study of chronic lymphocytic leukemia (CLL), we revealed a SAMHD1 mutation as a potential foun...

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