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Toplam kayıt 25, listelenen: 1-10
Dysplastic Changes in Idiopathic Thrombocytopenic Purpura and The Effect of Corticosteroids to Increase Dysplasia and Cause Hyperdiploid Macropolycytes
(Wiley-Liss, 2000)
This study evaluates the dysplastic hematological changes in nine patients with idiopathic thrombocytopenic purpura (ITP) in 11 attacks, before and after corticosteroid treatment. The pretreatment blood smears of patients ...
Lad-1/Variant Syndrome Is Caused by Mutations in Fermt3
(Amer Soc Hematology, 2009)
Leukocyte adhesion deficiency-1/variant (LAD1v) syndrome presents early in life and manifests by infections without pus formation in the presence of a leukocytosis combined with a Glanzmann-type bleeding disorder, resulting ...
Hypertrichosis: The Possible Side Effect Of Cyclosporin In An Infant With Hemophagocytic Lymphohistiocytosis Receiving Hlh-2004 Chemotherapy Protocol
(Aves Yayincilik, 2009)
Hemophagocytic lymphohistiocytosis is a life-threatening condition of severe hyperinflammation that results from an uncontrolled proliferation of activated lymphocytes and histiocytes secreting high amounts of inflammatory ...
A Complex Splicing Defect Associated With Homozygous Ankyrin-Deficient Hereditary Spherocytosis
(Amer Soc Hematology, 2007)
Defects in erythrocyte ankyrin are the most common cause of typical, dominant hereditary spherocytosis (HS). Detection of ankyrin gene mutations has been complicated by allelic heterogeneity, large gene size, frequent de ...
The Plasma Levels Of Prostanoids And Plasminogen Activator Inhibitor-1 In Primary And Secondary Thrombocytosis
(Sage Publications Inc, 2005)
An elevated platelet count is a common finding in both hospitalized and ambulatory patients. Thrombosis and bleeding complications are more frequently observed in patients with clonal thrombocytosis than secondary ...
Novel Hax1 Mutations In Patients With Severe Congenital Neutropenia Reveal Isoform-Dependent Genotype-Phenotype Associations
(Amer Soc Hematology, 2008)
Homozygous mutations in HAX1 cause an autosomal recessive form of severe congenital neutropenia (CN). By screening 88 patients with CN, we identified 6 additional patients with HAX1 mutations carrying 4 novel mutations. ...
The Mutational Spectrum Of Ptpn11 In Juvenile Myelomonocytic Leukemia And Noonan Syndrome/Myeloproliferative Disease
(Amer Soc Hematology, 2005)
Germ line PTPN11 mutations cause 50% of cases of Noonan syndrome (NS). Somatic mutations in PTPN11 occur in 35% of patients with de novo, nonsyndromic juvenile myelomonocytic leukemia (JIMML). Myeloproliferative disorders ...
Myeloprotective Effect Of Short-Course High-Dose Methylprednisolone Treatment Before Consolidation Therapy In Children With Acute Myeloblastic Leukemia
(Wiley, 2005)
In our previous studies, short-course high-dose methylprednisolone (HDMP) has been shown to shorten the chemotherapy-induced neutropenic period by stimulating the CD34(+) hematopoietic progenitor cells in children with ...
Natural History And Early Diagnosis Of Lad-1/Variant Syndrome
(Amer Soc Hematology, 2007)
The syndrome of leukocyte adhesion deficiency (LAD) combined with a severe Glanzmann-type bleeding disorder has been recognized as a separate disease entity. The variability in clinical and cell biological terms has remained ...
Life-Threatening Neurological Complications after Bone Marrow Transplantation in Children
(Nature Publishing Group, 2005)
Neurological complications may occur in BMT recipients (11-59%), frequently contributing to morbidity or mortality. They are the main causes of death in 10-15%. Life-threatening neurological complications were seen in 11 ...