33 research outputs found
Amifostine protects normal pluripotent peripheral blood stem cells against the toxicity of Idarubicin
Hemothorax due to extramedullary erythropoietic masses
We describe a 27-year-old male patient suffering from beta-thalassemia
intermedia who presented with a nontraumatic spontaneous hemothorax due
to extramedullary hemopoietic foci. In reviewing the literature, four
similar reports were found. The details of this unusual entity are
discussed
RANDOMIZED CLINICAL-TRIAL COMPARING CEFTRIAXONE AMIKACIN VERSUS CEFTAZIDIME AMIKACIN AS INITIAL THERAPY OF FEBRILE EPISODES IN NEUTROPENIC PATIENTS
Neutropenic patients with underlying hematologic (usually malignant)
diseases were randomized to receive either 2 g ceftriaxone once daily +
0.5 g amikacin or 2 g ceftazidime twice daily + 0.5 g amikacin b.i.d.
when fever was higher than 38-degrees-C and granulocyte counts less than
0.5 x 10(9)/l. 25 patients were included in each treatment group.
Successful outcome of treatment was observed in 28 (13/15) and in an
additional 5 (2/3) patients after modification of the therapy.
Tolerability was excellent in both groups
Analysis of the A(TA)(n)TAA configuration in the promoter region of the UGT1 A1 gene in Greek patients with thalassemia intermedia and sickle cell disease
Gilbert’s syndrome is characterized by mild unconjugated
hyperbilirubinemia. The molecular basis of this syndrome usually
concerns an additional dinucleotide insertion (TA) in the A(TA)(n)TAA
configuration residing in the promoter region of the UGT1 A1 gene. This
configuration may vary in length; the “n” represents the different
number of TA repeats. The homozygosity A(TA)(7)TAA/A(TA)(7)TAA is
involved in Gilbert’s syndrome. In many cases of patients with
thalassemia intermedia and sickle cell disease considerable variation in
bilirubin levels is observed. In this study we investigated the
contribution of the A(TA)(7)TAA/A(TA)(7)TAA genotype in the variable
unconjugated serum bilirubin levels in 31 Greek patients with
thalassemia intermedia and 27 Greek compound heterozygotes for beta
thalassemia and sickle cell anemia. Analysis of the A(TA)(n)TAA
configuration in the promoter region of the latter patients showed that
those who were carrying the homozygosity A(TA)(7)TAA/A(TA)(7)TAA had
higher levels of unconjugated bilirubin. These findings suggest that the
coexistence of Gilbert’s syndrome in patients with thalassemia
intermedia and sickle cell disease may be the cause of the elevated
values of unconjugated bilirubin, reducing the possibility of excessive
hemolysis in these patients. (C) 2003 Elsevier Science (USA). All rights
reserved
Multiple myeloma in sickle cell syndromes
Multiple myeloma (MM) is rare among patients with sickle cell syndromes
(SCS). We describe six Greek sickle cell patients aged 56 to 65 years:
five haemoglobin S beta (+)thalassaemia (HbS beta (+)thal), one sickle
cell anaemia (HbSS), who developed MM (three IgGK, one IgG lambda, one
IgAK, and one IgGK-IgAK (biclonal). Our HbS beta (+)thal cases,
represent the first reported association of this entity with MM.
Generalized bleeding diathesis, stroke, grand mal seizures, bone marrow
necrosis and other clinical manifestations due to hyperviscosity
aggravated by sickle cell vasoocclusion were treated by plasmaphereses
and exchange blood transfusions. The increase of mean survival in SCS
patients due to the current medical facilities may have an impact on the
incidence of MM among them, if a pathogenetic link between the two
conditions exists. All our patients carried a diagnosis of
cholelithiasis which may predispose to MM; two of them progressed from a
monoclonal gammopathy of undetermined significance (MGUS) to MM. Further
studies are needed in order to understand the relationship between SCS
and MM
ANGIOID STREAKS IN SICKLE-THALASSEMIA
Angioid streaks have been described in a diverse group of diseases
including hemoglobinopathies such as sickle cell anemia and
beta-thalassemia. We investigated the prevalence of angioid streaks and
pseudoxanthoma elasticum in the rare situation of patients who had
compound heterozygous traits for hemoglobin S and beta-thalassemia. We
examined 58 consecutive patients with sickle-thalassemia. Of these, 25
were men and 33 were women, and they ranged in age from 19 to 58 years
(mean, 32.6 years). Angioid streaks were identified in six of 58
patients (10%), and of these three also displayed the cutaneous lesions
of pseudoxanthoma elasticum, which were confirmed by skin biopsy. An
expanded study on several relatives of the patients with angioid streaks
failed to identify any similar cases. Statistical evaluation of the main
hematologic and biochemical parameters in the patients with and without
angioid streaks did not demonstrate any significant differences, except
that the thalassemic component in all six patients with angioid streaks
was beta degrees (that is, did not allow the synthesis of hemoglobin A).
We conclude that angioid streaks and pseudoxanthoma elasticum skin
lesions occur with an increased frequency in patients with
sickle-thalassemia
Unstable angina associated with coronary arterial calcification in a thalassemia intermedia patient with a pseudoxanthoma elasticum-like syndrome
The coexistence of a pseudoxanthoma elasticum (PXE)-like syndrome in
beta-thalassemia and other hemoglobinopathies is a recently established
clinical entity that has been observed with a significant frequency and
related to some severe, even life-threatening complications. We present
here a thalassemia intermedia patient who developed unstable angina in a
setting of severe anemia and PXE-related coronary arterial
calcification. Besides the clinical significance of this PXE-like
syndrome, its acquired nature may introduce some new thoughts regarding
the pathogenesis of atherosclerosis
Iron stores in multi-transfused thalassaemic patients seem not to be influenced by the HLA system
The HLA-A and -B antigens of 99 Greek patients with transfusion
dependent homozygous beta thalassaemia were determined. The HLA antigen
distribution in thalassaemic patients with a severe transfusion
siderosis and in patients without signs of siderosis were compared to
that of 400 healthy unrelated controls from the same population. There
is an increase of HLA-B14 antigen in both groups of thalassaemics as
compared with the controls. No significant difference exists in the
distribution of all the other HLA antigens between the two sub-groups of
thalassaemics or with the controls