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F Dore

Publications and source records attributed to F Dore.

52 records · Page 3Linked to original sources
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[Differential diagnosis of thalassemia syndromes].

In Sardinia, as in other areas with a high incidence of thalassemia syndromes, a prevention program based on the detection of healthy carriers through mass screening and on prenatal diagnosis in the at-risk couples has been in course for several years. The commonly adopted beta-thalassemia flow-chart consists of a first operative step involving simple and widely standardized tests: the estimation of red cell indices, the measurement of Hb A2 and Hb electrophoresis. These investigations permit the identification of the majority of the at-risk couples for beta-thalassemia. However, the not infrequent evidence of Hb A2 borderline levels, with or without microcytosis, isolated microcytosis or Hb F increased values, causes some problems in differential diagnosis, because these findings can indicate the presence of silent beta-thalassemic traits or other beta-thalassemic like states. A diagnostic definition of these unusual hematological phenotypes is particularly important for the identification of eventual at-risk couples. In this paper we report our data concerning the voluntary screening for beta-thalassemia carried out in North Sardinia. The operative flow-chart is shown. In a population with a high incidence of phenotypically heterogeneous thalassemic syndromes, such as that of Sardinia, differential diagnosis of thalassemic traits can require molecular studies. This molecular characterization, which could be carried out in specialized reference centers, is today absolutely necessary both for exact identification of at-risk couples and eventual prenatal diagnosis.

Adult↗
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Incidence and clinical study of ectopic erythropoiesis in adult patients with thalassemia intermedia.

We carried out total body computerized tomography (CT) studies and examined the retrospective clinical data of 29 adult patients with thalassemia intermedia (TI) to evaluate the incidence, features and pathogenesis of ectopic erythropoiesis (EE), located chiefly at the paravertebral gutters in the thorax, was present in 65.5% of the patients; 15% of them had severe clinical complications. We found a clear relationship between EE development and early presentation age of thalassemia, splenectomy and the presence of 100% fetal hemoglobin. The frequent occurrence of EE suggests that CT screening of patients with thalassemia intermedia should be mandatory. We also recommend radiotherapy as a preventive measure for the clinical complications of thalassemic patients with EE.

Adolescent↗
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Two cases of chronic lymphoproliferative disorders in psoriatic patients treated with cyclosporine: hairy cell leukemia and Waldenstrom macroglobulinemia.

The real risk of lymphoproliferative disease in psoriatic patients has not yet been defined. Two explanations can be given for the occurrence of these malignancies: the broad immune activation typical of psoriasis and the administration of an immuno-suppressive treatment. A few studies describing the development of non Hodgkin lymphomas in psoriatic patients undergoing cyclosporine A have been published, but data about the occurrence of chronic lymphoproliferative disorders have never been reported. Here we describe the occurrence of hairy cell leukemia and Waldenstrom macroglobulinemia in two psoriatic patients treated with cyclosporine A. It remains unclear in our cases of chronic lymphoproliferative disease, as well as in the reported cases of psoriatic patients who develop lymphomas, whether psoriasis or the immunosuppressive treatment could play a role, although it is not possible to exclude a synergism between these factors.

Adult↗
PubMedDate not supplied

[Role of densitometry and scintigraphy in post-traumatic algodystrophy of the arms. Initial diagnosis and monitoring during treatment with carbocalcitonin].

In 24 patients affected with post-traumatic reflex sympathetic dystrophy syndrome (RSDS) with upper limb involvement following humeral fractures, bone mineral density (BMD, mg/cm2) was measured by means of dual-photon absorptiometry in the distal radius of both the affected and the normal contralateral limbs. Subsequently, all patients underwent dynamic and static scintigraphic exams after i.v. injection of 99mTc-MDP (20 mCi), with gamma camera collimator centered in both limbs. BMD values were significantly lower in the affected sides than in the normal contralateral ones. Time-activity curves with MDP showed increased flow in the involved limbs. Significant increase in blood pool and in bone uptake was also observed. After carbocalcitonin treatment (80 U/q.d. i.m. in 12 cases and 40 U/q.d. i.m. in the other 12 cases for a month) all the patients presented improved clinical symptoms and significant increase in BMD, that was restored to normal values in 7 of the patients who had a longer treatment (40 U/q.d. i.m. for 2 months). Both local blood flow and bone uptake in the affected side significantly decreased after carbocalcitonin therapy while bone avidity index increased in those patients in whom this parameter had been measured. Our results confirm the usefulness of radioisotopic procedures in post-traumatic RSDS for both diagnosis (by demonstrating increased local blood flow and early bone demineralization) and monitoring response to treatment with carbocalcitonin, which seems to play an important role in this condition.

Adult↗
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[Whooping cough].

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Nursing↗
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Interferon-alpha 2a therapy in CML: disappearance of BCR/ABL transcript in a case of long-lasting continuous cytogenetic conversion.

Seventy months after diagnosis, minimal residual disease is undetectable in a patient with Philadelphia chromosome-positive chronic myelogenous leukemia (CML) in long-lasting continuous cytogenetic conversion (CCC), achieved through alpha 2a-interferon (IFN-alpha) therapy. Fluctuating molecular remission, evaluated with the two-stage reverse transcriptase-polymerase chain reaction (RT-PCR) with nested primers, has persisted for two years at the maximum tolerable dose of IFN alpha (1.5 x 10(6) IU per day).

Adult↗
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Serum interleukin-8 levels in thalassemia intermedia.

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 the IL-8 increase in thalassemia intermedia. We think than the hyperactivity of thalassemic macrophages related to chronic hemolysis is the main cause for the increment in cytokines, such as IL-8, found in thalassemic syndromes.

Adolescent↗
PubMedDate not supplied

Serum transferrin receptor levels in patients with thalassemia intermedia during rHuEPO administration.

In this study we determined the levels of circulating soluble transferrin receptor (sTfR) in six untransfused and two transfused patients affected by beta-thalassemia intermedia during low-dose rHuEPO administration. While the majority of the untransfused patients showed a temporary increment during the first month of treatment, a higher and enduring increase in sTfR concentration was observed in the two transfused patients until rHuEPO was discontinued. The transfused patients showed a significant increase in the reticulocyte index together with the rise in sTfR and an improvement of the anemia, as evidenced by the decrease in their transfusional requirement. These data suggest that basal erythropoietic activity is one of the main causes of differences in responsiveness to low doses of rHuEPO given in thalassemia intermedia.

Adult↗