15 research outputs found
The candidates’ counselling in the validation of informal and nonformal learning
The validation of the competences acquired other than by formal means is a current preoccupation at national and European policies level, an opportunity for the young and the adult people alike, a desire, but at the same time a reality of everyday life. The optimal conducting of the validation of nonformal and informal learning entails, according to the European documents, the supporting of candidates. The article presents, in its first part three case studies from different national contexts, Romania, France and Italy, focused on the counselling activity, In its second part, the article analyses the perception of a counselling framework possibly to be implemented in the human resources departments of Romanian companies as a complementary manner to the counselling offered by accredited evaluation centres
Association of monoclonal gammopathy and polycythemia vera or essential thrombocythemia: study of a large cohort of patients
Monoclonal gammopathy in human leishmaniasis
A 64-year-old female with IgGk monoclonal components (total 45 g/l) and 30% abnormal plasma cells and plasmoblasts in bone marrow is reported. After the identification of leishmania in the bone marrow, liposomal amphotericin B was used and a progressive resolution of the gammopathy was documented
Splenectomy after portal thrombosis in patients with polycythemia vera and essential thrombocythemia.
Background and Objectives. Polycythemia vera (PV) and
essential thrombocythemia (ET) are two rare acquired
myeloproliferative disorders (MPD) with frequent thrombotic
and hemorrhagic complications. The occurrence of
thrombosis in unusual sites, e.g. splanchnic vasculature,
is a severe complication of these diseases. We describe
a single-institution experience in patients with ET and
PV, diagnosed in agreement with the Polycthemia Vera
Study Group criteria, with portal vein thrombosis who did
or did not undergo splenectomy.
Design and Methods. The medical records and the followup
outcome of 16 MPD patients with portal thrombosis
who underwent splenectomy (group A1) and 16 who did
not (group A2) were evaluated. Their median follow-up
was, respectively, 13.45 and 10.49 years. The overall
survival of these patients was compared with that of a
population of 32 patients with MPD and no portal thrombosis
(group B) matched for sex, age, diagnosis and
duration of follow-up.
Results. In group A1, 2 patients developed deep vein
thrombosis, 1 patient had a surgical hemorrhage and 2
patients died early, one from acute infection, the other
from bone marrow aplasia. Among the survivors, one
male had a deep vein thrombosis and 1 developed a new
portal thrombosis. Four patients died during the followup
(median 9.48 years, range 3.17-25.1; 1 stroke, 2
gastrointestinal bleedings, 1 leukemic conversion). No
difference was observed in the incidence of thrombotic
or hemorrhagic complications or in the rate of deaths
when group A1 was compared to the other groups. The
use of antiplatelets drugs was statistically increased in
group A1 after splenectomy, because portal vein thrombosis
induced per se an increased use of therapeutic
agents. No statistical difference was observed in overall
survival between the different groups.
Interpretation and Conclusions. 1) Bleeding and thrombosis
are the leading causes of morbidity and mortality
in ET and PV patients with portal vein thrombosis both
with or without splenectomy. 2) Portal vein thrombosis,
and sometimes splenectomy, requires increased use of
drugs which may enhance the risk of leukemic transformation.
In spite of this, the patients who survive the first
post-splenectomy period may have a long and safe life
Clonality analysis in essential thrombocythemia (ET): differences between adults and children
V617F Jak2 mutation in children with essential thrombocythemia (ET)
none5noneRANDI M.L.; PUTTI MC; SCAPIN M; PACQUOLA E; FABRIS FRandi, MARIA LUIGIA; Putti, Mc; Scapin, M; Pacquola, E; Fabris, Fabrizi
Hydroxyurea in the treatment of primary thrombocytosis: a retrospective study of a single institution
Splenectomy after portal thrombosis in patients with polycythemia vera and essential thrombocythemia. Haematologica 2002; 87: 1180-1184 3,216
IF 4,19
Normal thrombopoietin and its receptor (c-mpl) genes in children with essential thrombocythemia.
BACKGROUND:Following the observation of thrombopoietin (TPO) gene abnormalities as the cause of familiar cases of thrombocythemia similar derangements of TPO and/or its receptor (c-mpl) might be surmised to be at the root of increased platelet count also in non-familiar (sporadic) cases. Although this was not demonstrated in adults, little data exist about childhood.
PROCEDURES:We studied the molecular biology of TPO and c-mpl in seven children with non-familiar essential thrombocythemia (ET) and one child with secondary thrombocytosis (ST). Plasma TPO content was measured using a commercially available kit. Genomic DNA was extracted from whole blood by standard methods and TPO and c-mpl genes were amplified by polymerase chain reaction (PCR) and sequenced.
RESULTS:Plasma TPO levels were normal in all our patients. No alteration was detected in either coding region, including the flanking intronic sequences of TPO and c-mpl genes. As compared to the published normal sequence of the TPO gene, one allelic base change in a non-coding region of intron 1 was found in all children with ET and ST, but this was reported as a common finding in normal subjects as well.
CONCLUSIONS:High platelet count in our series of sporadic ET of childhood is not due to an abnormality either of TPO or c-mpl gene