Philadelphia-positive chronic myelogenous leukemia with typical bcr/abl molecular features and atypical, prolonged survival.

Despite the major breakthrough in the knowledge of the molecular events underlying the t(9;22) translocation, still no consistent data have been found on the evolution of Ph1 positive CML from the chronic to the accelerated or blastic phase of the disease. In most patients in fact the bcr/abl rearrangements are identical both in chronic phase and in blast crisis, and overall differences in chronic phase duration, related to different location of breakpoints inside the bcr region, were found to be marginal. We approached this problem by studying the molecular features of the bcr/abl abnormality in rare CML patients with very long, atypical chronic phase. The three patients studied, whose chronic phase duration is 17, 19, and 21 years, respectively, have typical genomic bcr rearrangements, and two of them show, hybridizing Northern blots to c-abl, the 8.5 kb mRNA, as that typically present in CML. It seems that genomic alterations within bcr and abl cannot account, alone, for the duration of the chronic phase of Ph1 positive CML and those quantitative and/or qualitative alterations of the p210 bcr/abl protein, unluckily awkward to prove, might be responsible for the atypical clinical features of these CML long survivors.

Enzymatic and osmoregulative alterations in white shrimp Litopenaeus vannamei exposed to pesticides.

Pesticide pollution in coastal ecosystems of Sinaloa, Mexico is considered to be a cause for slow growth, increase of diseases and sometimes massive mortality of shrimp. So it was necessary to develop fast techniques to detect pesticide pollution in shrimp habitats. Enzymatic and osmoregulation tests in shrimp exposed to DDT, Lindane, Chlordane, Lorsban, Gusathion, Folidol, Diazinon and Tamaron were carried out. Activity reductions from 11 to 2 units/ml in acetylcholinesterase and from 1 to 0 units/l in transaminases (GOT and GPT) were detected. Also increases in osmoregulation were observed in shrimp exposed to Folidol, Diazinon and Gusation, whereas decreases with DDT, Lindane and Lorsban at salinity 50/1000. We conclude that pesticides are causing alterations in these biochemical functions and this kind of tests represent a rapid and inexpensive method for pesticide pollution detection.

[Malignant histiocytosis. Histiocytic medullary reticulosis]. / Istiocitosi maligna. Histiocytic medullary reticulosis.

The Authors report a case of malignant histiocytosis apparently localized only in the spleen. The diagnosis of the disease, characterized at the onset by moderate leukopenia and thrombocytopenia, was made possible by the histopathologic examination of the spleen following splenectomy. The disease shows a chronic course. The Authors discuss the clinical and histological features of the case.

[Clinical and lymphographic findings in miliary tuberculosis with tuberculin anergy. Considerations on a case]. / Rilievi clinici e linfografici in corso di tbc miliare con anergia alla tubercolina. Considerazioni su un caso.

Abdominopelvic lymphadenography conducted with due care in a patient with miliary TB and anergy to tuberculin ruled out alterations attributable to retroperitoneal lymphomatosis, and revealed a picture of miliary lymphadenitis. Splenectomy induced positivisation of the reaction to tuberculin and paved the way for a favourable response to anti-TB chemotherapy.

[Anergic miliary tuberculosis, with principally splenic localization simulating hemolymphopathy. Report of 4 cases]. / Tubercolosi miliare anergica a principale localizzazione splenica simulante una emolinfopatia. Descrizione di 4 casi.

Four cases of disseminated tuberculosis with prevailing spleen involvement and lack of tuberculin reactivity are described. The atypical clinical picture justified, at the beginning of the disease, the suspect of a lymphoreticular disorder (malignant lymphoma in 3 cases) or of a pulmonary hemosiderosis (in 1 case). The splenectomy and the following anti-tubercular chemotherapy were fully successful in all 4 patients and the skin reactivity was restored. The Authors discuss the pathogenesis of the observed features and the differential diagnosis of the cases of tuberculosis with only extrapulmonary involvement. These cases represent today about 1/6 of the patients with postprimary tuberculosis.

[Clinico-immunological study of 6 cases of Behçet's disease]. / Studio clinico-immunologico di sei casi di morbo di Behçet.

6 cases of Behçet's disease are reported. Diagnosis was particularly arduous because the typical triad of symptoms (oral aphthae, genital ulcers, uveitis) was masked by secondary disturbances in other organs and systems. Immunology confirmed the presence in these patients of changes in various in vivo and in vitro tests and particularly interesting was the aspecific cutaneous hypersensitivity seen in 5 cases and enhanced lymphocyte blastic transformation. This was seen spontaneously and following PHA. On the basis of the outcome of the studies carried out and the literature data, the possibility that Behçet's disease may contain immunological changes and changes in the mechanisms that regulate quinine, complement and clotting activation is suggested. Therapeutically, confirmation was obtained of the effectiveness, at least temporarily, of corticosteroid treatment and antilymphocyte globulin was experimented for the first time in these patients. This might be used to replace the immune depressant antiblastic substances already successfully employed in patients with Behçet's disease.

Left ventricular performance in the natural history and after corrective surgery of isolated ventricular septal defect.

Left ventricular performance by determination of the systolic time intervals, was analyzed in 34 patients with isolated ventricular septal defect and unidirectional left-to-right shunt. Fourteen of them (group I) with mean pulmonary flow/systemic flow ratio 2.19 +/- 0.3 SD, underwent corrective surgery; the other 20 patients (group II) with mean pulmonary flow/systemic flow ratio 1.43 +/- 0.3 SD were not operated; all the patients were followed up for at least 10 years. The measurements were obtained from simultaneous high speed photographic recordings of electrocardiogram, external carotid pulse and phonocardiogram. The analysis of data in group I showed a significant prolongation of pre-ejection period and an abbreviation of left ventricular ejection time; the degree of abbreviation was related to the magnitude of the shunt. The above abnormalities persisted after corrective surgery, and only several years after the operation these parameters became normal. Instead, the systolic time intervals were always normal in group II during the whole follow-up period. The most likely explanation for the observed abnormalities in ventricular septal defect in group I is that the depressed contractility of the left ventricle, which is secondary to the important volume overload, persists after corrective surgery. Septal construction abnormalities, before and after ventricular septal defect repair, may be another contributing factor in abnormal systolic time intervals.

Systolic time intervals in isolated septal defects before and after corrective surgery. II. Ventricular septal defect.

Systolic time intervals (STI) were analyzed in 34 patients with isolated ventricular septal defect (VSD) and undirectional left-to-right shunt. 14 of the patients who underwent corrective surgery were followed-up for at least 10 years. The measurements were obtained from simultaneous high speed photographic recordings of electrocardiogram, external carotid pulse and phonocardiogram. Before the operation the Q-I sound interval and, consequently, the pre-ejection period were significantly prolonged and the left ventricular ejection time significantly abbreviated, the degree of abbreviation relating with the magnitude of the shunt. The above deviations persisted after corrective surgery, and only several years after the operation the parameters studied became normal. It is concluded that the most likely explanation for these abnormalities is the depressed contractility of the left ventricle, secondary to the long-standing volume overload, which tends to persist after corrective surgery.

Systolic time intervals in isolated septal defects before and after corrective surgery in female patients. I. Atrial septal defect.

Systolic time intervals (STI) were evaluated in 19 female patients with uncomplicated ostium secundum atrial septal defect (ASD), before, shortly after (within 2 months) and a long time after the corrective surgery (mean 13.2 years). The measurements were obtained from simultaneous high speed photographic recordings of electrocardiogram, external carotid pulse and phonocardiogram. Before the operation, a significant prolongation of Q-I heart sound interval was detected, together with a less prominent but statistically significant shortening of the left ventricular ejection time; the above alterations were not correlated with the magnitude of the shunt and disappeared shortly after corrective surgery. The STI were still normal a long time after surgical closure of ASD. In accordance with hemodynamic studies reported by other authors, our results confirm that the deviations of STI observed in ASD are due to a reduced diastolic filling of the left ventricle secondary to an apparent decreased distensibility.

Systolic time intervals in patent ductus arteriosus before and after corrective surgery.

Systolic time intervals (STI) were analyzed in 11 patients with isolated patent ductus arteriosus (PDA) and unidirectional left-to-right shunt, before, shortly after (within 2 months) and a long time (at least 10 years) after the corrective surgery. The measurements were obtained from simultaneous high speed photographic recordings of electrocardiogram, external carotid pulse and phonocardiogram. Before the operation, the left ventricular ejection time (LVET) was significantly prolonged (p less than 0.01) and this abnormality was correlated with Qp/Qs (r = 0.74, p less than 0.01). Shortly after the operation, the LVET was shorter than normal, and became normal only a long time after. It is concluded that the most likely explanation for these abnormalities is: 1) before the operation, the increased stroke volume of the left ventricle secondary to the shunt, and 2) after corrective surgery, the depressed contractility of the left ventricle secondary to the long-standing volume overload which tends to persist after the corrective surgery.