Elio Cacciola

Antithrombin III Concentrate for Treatment of Chronic Leg Ulcers in Sickle Cell-Beta Thalassemia: A Pilot Study

Excerpt Chronic leg ulcers are a common distressing complication in patients with sickle cell-beta thalassemia and are difficult to heal. The pathogenic mechanism of this complication is not known ...

A leftward deletional α+ thalassemia found in East Sicily in conjunction with heterozygous β-thalassemia

SummaryTwo types of α+-antitrypsin thalassemia (-α/) have been described, respectively termed leftward and rightward, which correspond to nonhomologous crossing-over in different homology zones X and Z within the α-globin gene cluster. Up to now the leftward type has been described only in Asiatic populations, whereas the rightward type is universally distributed. We report here a first case of leftward deletion observed in a Sicilian male. This raises the question of an identical or not crossing-over event.

Multiple molecular bases for thalassemia intermedia in east Sicily.

The existence of forms of P-thalassemia with intermediate severity has been long known, and the name thalassemia intermedia is used to describe a widespread spectrum of patients. The criteria used were both clinical and hematological or, frequently, based on moderate transfusional needs. Nevertheless, characterization of molecular defect(s) in these cases has been only sporadic, made in individual cases or in short, ethnically homogeneous series. The principles of such characterizations are based on the knowledge that the factor determining the severity of the disease is the degree of globin chain imbalance, due to the deleterious effect of excess unpaired a chains on erythroid cells.’ In theory at least, it is clear that there are three ways of reducing this effect: a reduction in a-chain production, the intrinsically mild nature of the &gene defect, or an unusually high production of y-chains able to combine with the excess a-chains. Examples have been given of all of these three mechanisms. In short, local series, there was apparently a predominance of a single pathophysiology.’ ’

Acute Respiratory Distress During Sclerotherapy for Esophageal Varices

Excerpt To the editor: Connors and coworkers (1) report that the adult respiratory distress syndrome, which may occur after endoscopic variceal sclerotherapy using sodium morrhuate, was not depende...

T lymphocyte subpopulations in the bone marrow and peripheral blood of untreated patients with myeloma.

SummaryThe quantitative distribution of the two main T lymphocyte subsets, recognizable by the ‘high’ and ‘low-affinity’ E rosette-forming cell technique of West, was studied in both the bone marrow and peripheral blood from ten untreated multiple myeloma (MM) patients. A reduced total T lymphocyte count, with a relative predominance of ‘low-affinity’ T lymphocytes (putative suppressor T cells), was found in the peripheral blood. Within a normal total T lymphoid cell count, a predominance of the T lymphocyte subset with ‘low-affinity’ characteristics was also observed in the bone marrow. An inverse correlation, that was statistically significant, was seen between the monoclonal malignant cellular B component and the ‘low-affinity’ T cell percentage in all cases. It is concluded, therefore, that such an imbalance between the ‘high’ and ‘low-affinity’ T subsets, with the latter predominating, could be of importance in the regulation of the growth rate of the monoclonal cellular B component.

Low-Dose Cytarabine in Myelodysplastic Syndromes and Acute Leukemia

Low-Dose Cytarabine in Myelodysplastic Syndromes and Acute Leukemia A Comment E. Elio Cacciola F. Francesco Di Raimondo P. Patrizia Guglielmo G. Giuseppe Milone R. Rosario Giustolisi Elio Cacciola, MD, Francesco Di Raimondo, MD, Patrizia Guglielmo, MD, Giuseppe Milone, MD, Rosario Giustolisi, MD, Institute of Medical Semeiotic I and Hematology, University of Catania, Via S. Citelli No. 6, 1-95124 Catania (Italy) We have read with interest the paper of A. Inbal et al. [1] and we would like to make some comments. There are a few unclear data. The authors reported in table II that patient No. 2 had a granulocyte count of 5.98 × 10/1 before treatment with low-dose cytarabine (LDC) and a granulocyte count of 5.28 × 10V1 after treatment. It is also reported that patient No. 1 did not have any abnormal chromosomal pattern. Nevertheless, in the ‘Results’ and ‘Discussion’ sections they asserted that’... in 3 patients (No. 1, 2, 3) with refractory anemia (RA) normal peripheral blood was obtained after one course of treatment’. But patient No. 2 had before treatment with LDC a granulocyte count of 5.98 × 10/1 and of 5.28 × 10V1 after treatment; is this a mistake? Furthermore, the authors reported in the ‘Discussion’: ‘... therapy with LDC was associated with beneficial responses in 5 of the 8 patients, 3 of whom (namely the first three ones showed in the table) achieved normal peripheral blood counts’. However, patient No. 2 had yet prior to treatment a normal granulocyte count, whereas platelet count and Hb level were low. With regard to patient No. 1 the chromosomal analysis was normal (see table II), but in the ‘Discussion’ (p. 73) the authors reported as follows: ‘In our 3 patients with refractory anemia (namely patients No. 1, 2, 3), despite excellent responses to cytarabine, the bone marrow metaphases remained 100% abnormal’. But if patient No. 1 had a normal chromosomal pattern before treatment, how could one explain 100% of metaphases abnormal? Is this a mistake? In addition, we would like to comment some suggestions of the authors with regard to the mechanism of action of LDC which, in our opinion, appears to be conflicting. They firstly reported that the LDC regimen results in inducing a suppression of the leukemic clone, so allowing the suppressed normal hematopoietic one to maturate. The authors later reported that the excellent response to cytarabine regards a differentiating effect on the leukemic clone, as demonstrated by the persistence of abnormal bone marrow metaphases. This could be the case in the patient with acute leukemia (even if one had a normal karyotype), but it is questionable in RA patients. In RA patients the percent of bone marrow blast cells is very small (5%); therefore, it is difficult to think that the myeloid matu-rative effect of LDC corresponds to a differentiative stimulus on ‘leukemic clone’ rather than on ‘suppressed normal hemopoietic line’. The finding of a persistent abnormal chromosomal pattern after LDC does not seem enought to support this view because it might concern the persistence of residual leukemic cells. On the other hand, the