Mutations within exon 3 of the @-globin gene are relatively uncommon, and many of these mutations produce a dominant thalassemia-like phenotype. We describe a novel thalassemic hemoglobinopathy caused by a single nucleotide substitution (CTG + CCG) at codon 1 14 resulting in a leucine to proline substitution and designate it @Durham-NC B1 14 Leu + Pro]. The mutation producing this thalassemic h...

A 9-year-old female received an allogeneic stem cell transplant (SCT) from an ABO-incompatible HLA-matched sibling for β-thalassemia major, without achieving a complete donor chimerism. Subsequently, the patient received five donor lymphocyte infusions, without increasing donor chimerism, and autologous SCT. Due to the persistent bone marrow aplasia, the patient received a second allogeneic SCT...

In this study we determined serum IL-8 levels in 18 untransfused patients with beta-thalassemia intermedia and in 14 subjects affected by HbH disease. As reported in polytransfused homozygous beta-thalassemia, untransfused beta-thalassemia and HbH disease show significantly (p < 0.005) higher serum IL-8 levels than normal controls. Our data suggests that there could be an intrinsic cause for th...

The amount and distribution of intranuclear suggested the presence of intranuclear hehemoglobin in erythroblasts of normal submoglobin precipitation in some cells. jects and patients with homozygous Similar characteristic absorption peaks of thalassemia were studied by microspecdenatured ferric hemoglobin were recorded trophotometric methods. The mean intranuover large inclusions of the cytopla...

Background: Extramedullary hematopoiesis (EMH) in the middle ear is exceedingly rare, with fewer than five cases reported. The authors report the first Thai case of middle ear EMH. Case Report: A 32-year-old Thai thalassemic man presented with complaint of right-sided hearing loss from a middle ear mass. The CT/MRI was done and the diagnosis of EMH was confirmed by a pathological examination af...

A prospective case control study was carried out to evaluate the prevalence of HBsAg in 129 thalassemic patients and 113 children as a control group.ELISA was used for serologic investigations. thalassemic  patients were aged 8.5+_3.8 years and children of the control group were aged 7.2+_3.8 years.48.1% of the case group and 36.3% of the control group were female and 51.9% of the case group an...

A 19-year-old thalassemic woman had tissue from one of her ovaries cryopreserved prior to bone marrow transplantation, total body irradiation and sterilizing chemotherapy. As expected, premature ovarian failure resulted from this treatment. Transplantation of her thawed ovarian tissue resulted in return of menstrual cycling and the patient then underwent several IVF cycles. The patient, however...

Spinal cord compression due to extramedullary hematopoiesis is a well-described and rare syndrome encountered in several hematological disorders including β-thalassemia. Hereby, a 37-year old pregnant woman with intermediate β -thalassemia with paraparesis and lower limb hypoesthesia was presented. MRI s...