Acute promyelocyte leukemia arose from CALR 1 mutated post essential thrombocythemia- myelofibrosis with splanchnic vein thrombosis: A case report.

Major disease complications for patients with essential thrombocythemia (ET) include thrombosis and fibrotic or leukemic transformation. Calreticulin (CALR) mutation type 1 frequencies in ET are estimated between 7% and 11% and ET patients carrying CALR type 1 mutation are associated with lower risk of thrombosis but higher risk of myelofibrosis transformation compared to ET patients with JAK2 mutation. Leukemic transformation rates at 20 years are estimated at less than 5% for ET and risk factors for leukemic transformation are advanced age, thrombosis history, leukocytosis, and anemia. Amongst the subtypes of blast phase myeloproliferative neoplasms, acute promyelocytic leukemia is extremely rare. Herein, we present a case of a promyelocytic blast crisis of post-ET myelofibrosis with associated life-threatening splanchnic vein thrombosis. This case suggests that inflammation plays a key role in thrombotic events and fibrotic/leukemic transformation in ET patients, regardless the molecular landscape.

Putative occurrence of lysine decarboxylase isoforms in soybean (Glycine max) seedlings.

The activity of lysine decarboxylase was studied in 3-day-old soybean (Glycine max (L.) Meer cv. Sakai) seedlings also in relation to light conditions. Lysine decarboxylase activity was mainly localized in the roots and to a lesser extent in the hypocotyls and was detectable in both the soluble and particulate fractions. The enzyme activity levels were similar during germination under light and dark conditions. With respect to lysine concentration, the initial decarboxylation rate of the soluble fraction showed a saturating curve. Conversely, the initial decarboxylation rate of the particulate fraction showed a sigmoidal curve. These results could suggest that at least two isoforms of lysine decarboxylase are present in different organs of soybean seedlings. In the root soluble fraction, the suicide inhibitor alpha-difluoromethyl-lysine suppressed the activity of lysine decarboxylase and of ornithine decarboxylase to the same extent, but had no effect on arginine decarboxylase activity.

Polyamine metabolism and biosynthetic gene expression in Arabidopsis thaliana under salt stress.

In the present study we analysed polyamine metabolism in Arabidopsis thaliana (ecotype Columbia) rosette leaves collected at vegetative and reproductive stages from plants germinated and grown under increasing salt stress (0-75 mM NaCl) conditions. The expression level of the different isoforms of polyamine biosynthetic enzymes was analysed by reverse transcriptase-polymerase chain reaction (RT-PCR) and the polyamine biosynthetic enzyme activities were determined both in supernatant and pellet fractions. Free and perchloric acid (PCA)-conjugated (soluble and insoluble) polyamines, were measured. At vegetative stage, plants were able to adapt up to 50 mM NaCl, showing a significant growth inhibition only at 75 mM NaCl. At this growth stage and NaCl concentration there was an up-regulation of spermine biosynthesis. At reproductive stage, plants were able to flower up to 50 mM NaCl, even if with a delay of 7 days. On the contrary, at 75 mM NaCl two different phenotypes were isolated: 75/01 (salt sensitive) and 75/02 (salt tolerant). The sensitive plants (75/01) showed a severely stressed phenotype, compared to the tolerant ones (75/02), and the polyamine metabolism was up-regulated, with the increase of free putrescine and spermine.

Factors influencing hospital infection control policies in Italian hospitals.

A study was undertaken to determine the resources available in Italian hospitals for the control of nosocomial infections and the factors favouring a successful approach. During January-May 2000 a questionnaire about infection control was sent to the hospital health director of all Italian National Health System hospitals treating acute patients and with more than 3500 admissions in 1999. An active programme was defined as a hospital infection control committee (HICC) meeting at least four times in 1999, the presence of a doctor with infection control responsibilities, a nurse employed in infection control and at least one surveillance activity and one infection control guideline issued or updated in the past two years. There was a response rate of 87.5% (463/529). Almost fifteen percent (69/463) of hospitals had an active programme for Infection Control and 76.2% (353/463) had a HICC. Seventy-one percent (330/463) of the hospitals had a hospital infection control physician and 53% (250/463) had infection control nurses. Fifty-two percent (242/463) reported at least one surveillance activity and 70.8% (328/463) had issued or updated at least one guidance document in the last two years. The presence of regional policies [odds ratio (OR) 8.7], operative groups (OR 4.2), at least one full-time nurse (OR 4.6) and a hospital annual plan which specified infection control (OR 2.1) were statistically associated with an active programme in the multivariate analysis.

Biosynthesis, oxidation and conjugation of aliphatic polyamines in higher plants.

This chapter will focus on polyamine biosynthesis, oxidation, conjugation processes, mainly to hydroxycinnamic acids, and compartmentation of enzymes, substrates and products, giving an overview about recent results especially in higher plants. New research advances regarding the cloning of the main cDNA encoding for polyamine biosynthetic and oxidative enzymes, will be taken into consideration.

Characterization of spermidine binding to solubilized plasma membrane proteins from zucchini hypocotyls

In this work [14C]spermidine binding to total proteins solubilized from plasma membrane purified from zucchini (Cucurbita pepo L.) hypocotyls was investigated. Proteins were solubilized using octyl glucoside as a detergent. Specific polyamine binding was thermolabile, reversible, pH dependent with an optimum at pH 8.0, and had a Kd value of 5 &mgr;M, as determined by glass-fiber-filter assays. Sephadex G-25 M gel-filtration assays confirmed the presence of a spermidine-protein(s) complex with a specific binding activity. By sodium dodecyl sulfate-polyacrylamide gel electrophoresis and native polyacrylamide gel electrophoresis of collected fractions having the highest specific spermidine-binding activity, several protein bands (113, 75, 66, and 44 kD) were identified. The specificity of spermidine binding was examined by gel-filtration competition experiments performed using other polyamines and compounds structurally related to spermidine. Partial purification on Sephadex G-200 led to the identification of 66- and 44-kD protein bands, which may represent the putative spermidine-binding protein(s) on the plasmalemma.

Polyamine Binding to Plasma Membrane Vesicles Isolated from Zucchini Hypocotyls.

The general features of [14C]spermidine binding to plasmalemma vesicles isolated from zucchini (Cucurbita pepo L.) etiolated hypocotyls are reported in the present paper. The specific interaction of the polyamine with the plasma membranes was reversible and thermolabile, since it decreased by about 50% in the assay performed at 40[deg]C compared to that carried out on ice. On the contrary, nonspecific binding was unaffected by temperature. Specific spermidine binding showed a pH dependence with a maximum at pH 8.0 and it reached saturation between 0.75 and 1 mM external spermidine concentration. The value of the dissociation constant calculated from Scatchard analysis was 4.4 x 10-5 M. Specific spermidine interaction appeared to be sensitive to detergents and was markedly reduced by the presence of divalent cations, such as Mg2+ and Ca2+, whereas it was stimulated by monovalent cations. Polyamine binding sites were highly sensitive to pronase treatment. Competition experiments, performed using a series of compounds structurally related to spermidine, may provide some indication of the characteristics of spermidine binding sites. The results presented here suggest that specific spermidine binding occurs mainly with the protein component of the plasma membrane.

[Values and limitations of radioimmunoscintigraphy with MoAb 225-28S in the diagnosis of posterior uveal melanoma]. / Valeurs et limites de la radio-immuno-scintigraphie avec MoAb 225-28S dans le diagnostic des mélanomes uvéaux postérieurs.

When evaluating a clinical situation in which a posterior uveal melanoma (PUM) is suspected, we have to use all available diagnostic procedures because it may be difficult to distinguish between amelanotic tumours and/or small melanomas. Early diagnosis of a small lesion is mandatory for successful conservative therapy. Radioimmunoscintigraphy (RIS) of malignant melanoma has become an encouraging tool in Nuclear Medicine. 99mTc labelled F (ab')2 fragments of MoAb 225-28S raised against cutaneous melanoma were used. Nine patients were examined: the clinical diagnosis was PUM in 7 cases and choroidal metastases in 2 cases. Due to the size and/or site of the tumor, six eyes were enucleated. In all of these cases histopathology revealed a malignant melanoma. One patient died with disseminated hepatic metastases, 2 months after observation. RIS was positive in 5 of 7 PUM (71.4%) and a false negative result was obtained in 2 patients. Two true negative scintigraphies were observed in the patients with breast carcinomas and choroidal metastases. On the basis of these results, RIS may be valuable to diagnose ocular malignant melanoma, but specificity of MoAb 225-28S needs to be assessed.

[Retinographic and angiographic study of ocular lesions detected in AIDS]. / Contribution à l'étude rétinographique et angiographique des lésions oculaires dépistées au cours du SIDA.

We examined 42 patients with the acquired immune deficiency syndrome: 11 of these had retinal lesions. In this study we reported five cases; all were examined within several weeks of admission to the Maggiore Hospital, and at various intervals thereafter. Ophthalmic evaluation included determination of visual acuity, slit-lamp examinations, direct ophthalmoscopy with the pupil dilated, fundus photography and fluorescein angiography. Peripheral blood mononuclear cells were isolated during gradient density centrifugation. Lymphocyte subsets were determined by immunofluorescence with monoclonal antibodies of the OKT series. All patients demonstrated a reduction of the ratio of T-helper to T-suppressor. Patient no. 1 was a 41 year-old hemophiliac man became ill (ARC) in June 1986; initially the patient had no ocular lesions. After five months developed bilateral retinal cotton wool spots. The patient's clinical conditions progressively worsened during the ensuing months and after fifteen months he felt a decreased vision in left eye: retinal examination disclosed a presumed CMV retinitis. Patient no. 2 was a 30 year-old intravenous drug-user man. At the time of the admission (AIDS) ophthalmologic evaluation revealed multiple cotton wool spots in both eyes. These changed of number and size during the following months. This patient was treated with ganciclovir (dihydroxy propoxymethyl guanine) because developed a CMV encephalopathy. We noted after this treatment a strong reduction of the cotton wool spots without an improvement of the general conditions and he died a month later. Patient no. 3 was a 26 year-old drug-user woman admitted for ARC. Ophthalmologic evaluation disclosed in right eye a presumed CMV retinitis with vascular sheathing and hemorrhages, and in left eye a little white retinal lesion (an initial retinitis) and cotton wool spots. The general and the retinal conditions rapidly worsened and she died three months later. Patient no. 4 was a 24 year-old women, intravenous drug-user, admitted for AIDS complicated by central nervous system infection by Toxoplasmic Gondii. Ocular examination revealed in right eye retinal cotton wool spots, and in left eye a white chorioretinal lesion with vitritis attributed to Toxoplasma Gondii. This retinochoroiditis improved after empiric therapy with sulfonamides and pyrimethamine. Patient no. 5 was a 37 year-old bisexual man admitted for AIDS. The findings on ophthalmologic examination were: CMV retinitis in right eye and retinal cotton wool spots in left eye. Treatment with ganciclovir resulted in an improvement of general symptoms.(ABSTRACT TRUNCATED AT 400 WORDS)

Contribution to the differentiation of peripheral versus central tinnitus via auditory brain stem response evaluation.

Auditory brain stem response (ABR) parameters were evaluated in 54 subjects with unilateral idiopathic subjective tinnitus in order to verify the possibility of detecting its site of origin. All the subjects had normal hearing or a symmetrical bilateral sensorineural hearing loss. All the cases in whom middle or inner ear disease had been diagnosed, were excluded. Subjects, classified on the basis of their mean auditory threshold and masking curves according to Feldmann, underwent a residual inhibition (RI) test and ipsilateral narrow-band noise masking before a second ABR test was performed. Patients with positive RI made up the A+ group, while those with negative RI made up the A- group. The main characteristics observed were an increase of the mean latency values of wave I in the tinnitus ear in the A+ group while, after masking, the values of the affected and unaffected ears almost overlapped. An increase in the latency values of wave V, unaffected by the masking procedure, could be observed in A- patients. The occurrence of waves I and III was often affected in the tinnitus ears in both groups, but it increased, after masking, only in the A- patients. Based on the obtained data, the authors conclude that there may be a substantial difference concerning ABR parameters between the patients in whom residual tinnitus masking is demonstrable compared with those in whom it is not.

Auditory brainstem responses (ABR) in multiple sclerosis.

Thirty-two multiple sclerosis (MS) patients, 10 males and 22 females, aged between 21 and 55, underwent pure-tone audiometry and testing of auditory brainstem response (ABR). Thirteen were classified as 'definite', 12 as 'probable' and 7 as 'possible', according to the McAlpine criteria. Each ear of each patient was tested monoaurally. The most common alterations were seen in the parameters of the cross-correlation between the normal template and the template of the individual MS patient. The second most altered parameters were those concerning the V wave which was absent in most cases but, when present, rarely showed latency-amplitude values falling outside the 90% confidence limit ellipses. Test-retest replicability and stimulations with increasing numbers of stimuli per second were useful in detecting intra-individual variability of waveform characteristics. When considering all the tests performed, 88% of the 'definite', 71% of 'probable' and 64% of 'possible' MS patients' ears showed ABR abnormalities. We stress the importance of a separate evaluation of the two ears due to the frequent unilateral alterations, and of an accurate evaluation of the ABR characteristics. We conclude that brainstem audiometry is a more reliable test for detecting demyelinating processes than is usually described in the literature.

Middle-latency auditory components in response to clicks and low- and middle-frequency tone pips (0.5-1 kHz).

Middle-latency auditory components (MLC) in response to clicks and tone pips have been recorded in 20 normal subjects, aged between 26 and 32 years, in order to verify their reliability in response to frequency-specific stimuli (0.5 and 1kHz). The results indicate a good reliability of MLC obtained when using tone pips. The responses show the conventionally labeled Po, Na, Nb, Pb waves. The latencies of these waves tend to be greater than those of the corresponding waves elicited by clicks and their amplitudes are smaller. This is probably due to an asynchrony of the responses. The Po and Pa waves are the most resistant to decreasing stimulus intensity, as both are clearly detectable down to 20 dB nHL, but Po is the best threshold index because at 20 dB it has a more clear-cut shape than Pa. According to the latency values obtained for MLC elicited by both clicks and tone pips, the Po wave is probably generated at the inferior colliculus level. The latency shift towards the click-elicited Jewett wave V is mainly due to the different filter settings employed. The morphology of MLC elicited by tone pips is less affected by changes in stimulus frequency than that of corresponding auditory brainstem responses. Thus, MLC are a reliable indicator for defining low- and middle-frequency auditory thresholds.

Nasal polyps: a comparative study of morphologic and etiopathogenetic aspects.

16 nasal polyps belonging to 10 male subjects aged between 40 and 70, have been removed during the same number of ethmoidectomies; the polyps have been sectioned and specimens of mucosa corresponding to the pedicle and to the different parts of the body have been collected. During the operation parts of the mucosa of the inferior turbinates were removed. All the specimens have been prepared and observed at the scanning electron microscope. The epithelium of the inferior turbinates, of the small polyps and of the apical part of all polyps were almost normal. Marked alterations of the epithelial layer have been observed in bigger polyps. Surface characteristics of nasal polyps seem to be influenced by their relationship with neighbouring structures and by their position in the nasal cavity, but mostly by their size. On the basis of these findings the authors discuss the most recent etiopathogenetic theories of nasal polyps. They finally describe some peculiar findings concerning the mucosal layer of the polyps and of the inferior turbinates of three confirmed allergic subjects and discuss their possible clinical implications.