Correlation between Primary Myelofibrosis and the Association of Portal Thrombosis with Portal-Biliary Cavernoma: US, MDCT, and MRI Features

Abstract Objective Myelofibrosis is a rare chronic myelolymphoproliferative disease and is associated with increased risk of venous thromboembolism. The objective of this study is to retrospectively evaluate patients with primary myelofibrosis who underwent abdominal US, MDCT and MRI, in order to identify the development of portal thrombosis and its correlation with portal-biliary cavernoma. Methods We evaluated 125 patients with initial diagnosis of primary myelofibrosis and nonspecific abdominal pain who had undergone US with color Doppler. In 13 patients (8 men, 5 females; age: 45–85), US detected portal thrombosis with associated portal-biliary cavernoma. All patients subsequently underwent contrast-enhanced MDCT and MRI and 4 patients MR-cholangiography. The correlation between primary myelofibrosis and portal thrombosis and cavernoma respectively was calculated using χ2 test. Results About 10% of patients with primary myelofibrosis preliminary evaluated with US had partial (8 pts) or complete (5 pts) portal thrombosis associated with portal-biliary cavernoma with a χ2 = 0. In all patients, US detected a concentric thickening of main bile duct (MBD) wall (mean value: 7 mm); color Doppler always showed dilated venous vessels within the thickened wall of the biliary tract. Contrast-enhanced CT and MRI confirmed thickening of MBD walls with their progressive enhancement and allowed better assessment of the extent of the portal system thrombosis. MR-cholangiography showed a thin appearance of the MBD lumen with evidence of ab extrinsic compression. Conclusions The evidence of portal thrombosis and portal-biliary cavernoma in 10% of the patients with primary myelofibrosis indicates a close correlation between the two diseases. In the detection of portal thrombosis and portal-biliary cavernoma, US with color Doppler is the most reliable and economical diagnostic technique while contrast-enhanced MDCT and MRI allow better assessment of the extent of the portal vein thrombosis and of the complications of myelofibrosis

Risk analysis on the introduction into free territories of vaccinated animals from restricted zones

Between August 2000 and 14 May 2001 (defined as the 2000-2001 epidemic) bluetongue (BT) wasreported in three regions of Italy: Sardinia, Sicily and Calabria. During the 2001-2002 epidemic(between 15 May and 14 April 2002), the disease spread to five additional regions (Puglia, Basilicata,Campania, Latium and Tuscany). In May 2001 the Italian Ministry of Health decided to restrictanimal movements and to vaccinate all susceptible domestic ruminant populations in infected andin neighbouring regions. This action was taken to reduce virus circulation with the aim ofdecreasing direct losses in sheep and goats due to the disease, and indirect losses in cattlepopulations due to movement restrictions. Furthermore, the Italian authorities implemented anepidemiological surveillance system to monitor the spread of the virus and to provide moreeffective movement controls. In 2002, the vaccination campaign reached the set goal of vaccinatingmore than 80% of susceptible domestic ruminants in Abruzzo, Sardinia and Tuscany. Thevaccination campaign successfully reduced clinical disease in Sardinia and Tuscany. Before theadvent of BT, cattle had always been moved from Sardinia, Sicily and the southern regions forfattening and slaughter in northern Italy. During the tracing of animals that had left infected areasin 2000 it was found that 10 957 cattle had been exported from Sardinia between June and August2000 and were scattered throughout continental Italy. In addition, most cows selected for cullingfrom the southern regions and the islands were sent to northern Italy for slaughter. However, sinceAugust 2000 the animal trade between infected and free areas has come to a complete standstill.Sardinia, in particular, due to the climatic and epidemiological conditions (vectors survive almostthroughout the year), was no longer able to export any ruminants to the mainland. Long-termstandstill therefore led to heavy economic losses and had even greater social consequences. Asfarmers are not compensated, it is impossible to enforce these restrictions indefinitely. The Italianauthorities and the European Commission thus decided to adopt a policy of risk managementallowing some animal movement. This paper presents an analysis that assesses the risk associatedwith animal movement from restricted areas, according to the level of immunity of susceptibleanimal populations due to vaccination in the same areas. Results of the analysis indicate that whenmore than 80% of the susceptible population in the territory of origin is vaccinated, the riskassociated with the movement of vaccinated animals to free areas appears acceptable and can bemitigated further by adopting ancillary control measures.[...

Acquired haemophilia in severe pelvic endometriosis: a new association?

[No abstract available

Low Cell Bioenergetic Metabolism Characterizes Chronic Lymphocytic Leukemia Patients with Unfavorable Genetic Factors and with a Better Response to BTK Inhibition

Chronic Lymphocytic Leukemia (CLL) is an indolent malignancy characterized by the accumulation of quiescent mature B cells. However, these cells are transcriptionally and translationally active, implicating an active metabolism. The recent literature suggests that CLL cells have an oxidative-type phenotype. Given the role of cell metabolism, which is able to influence the outcome of treatments, in other neoplasms, we aimed to assess its prognostic role in CLL patients by determining the ex vivo bioenergetic metabolic profile of CLL cells, evaluating the correlation with the patient clinical/biological characteristics and the in vivo response to BTK inhibitor treatment. Clustering analysis of primary samples identified two groups, characterized by low (CLL low) or high (CLL high) bioenergetic metabolic rates. Compared to the CLL high, CLL with lower bioenergetic metabolic rates belonged to patients characterized by a statistically significant higher white blood cell count and by unfavorable molecular genetics. More importantly, patients in the CLL low cluster displayed a better and more durable response to the BTK inhibitor ibrutinib, thus defining a bioenergetic metabolic subgroup that can benefit the most from this therapy

Attention-related changes in short-term cortical plasticity help to explain fatigue in multiple sclerosis

Background: In multiple sclerosis (MS), pathophysiology of fatigue is only partially known. Objective: The aim of this study was to investigate whether the attention-induced modulation on shortand long-term cortical plasticity mechanisms in primary motor area (M1) is abnormal in patients with MS-related fatigue. Methods: All participants underwent 5-Hz repetitive transcranial magnetic stimulation (rTMS), reflecting short-term plasticity, and paired associative stimulation (PAS), reflecting long-term plasticity, and were asked to focus their attention on the hand contralateral to the M1 stimulated. A group of agematched healthy subjects acted as control. Results: In patients with MS, 5-Hz rTMS and PAS failed to induce the normal increase in motor-evoked potential (MEP). During the attention-demanding condition, 5-Hz rTMS- and PAS-induced responses differed in patients with MS with and without fatigue. Whereas in patients with fatigue neither technique induced the attention-induced MEP increase, in patients without fatigue they both increased the MEP response, although they did so less efficiently than in healthy subjects. Attention-induced changes in short-term cortical plasticity inversely correlated with fatigue severity. Conclusion: Short-term and long-term plasticity mechanisms are abnormal in MS possibly owing to widespread changes in ion-channel expression. Fatigue in MS reflects disrupted cortical attentional networks related to movement control

Low absolute lymphocyte count is a poor prognostic factor in diffuse-large-B-cell-lymphoma

The prognostic value of absolute lymphocytic count (ALC), has been a recent matter of debate in non-Hodgkin-lymphoma (NHL). We assessed prospectively the value of ALC at diagnosis and also after the completion of immuno-chemotherapy in 101 diffuse-large-B-cell-lymphoma (DLBCL). Analysis of prognostic factors with respect to overall survival (OS), event free survival (EFS) and progression free survival (PFS) was done by two-tailed log-rank test. The ALC cut-off value was calculated as < 0.84 x 10(9)/L at diagnosis: this was a strong negative prognostic factor for OS (p = 0.0004), EFS (p < 0.00001) and PFS (p < 0.00001) and in multivariate analysis was independent from the revised-international-prognostic-index (R-IPI). ALC after chemo-immunotherapy was not of prognostic value. As R-IPI and ALC < 0.84 x 10(9)/L, were the factors better discriminating poor prognosis, a new trichotomous score (ALC/R-IPI) was built up: (1) low risk: R-IPI = very good or good and ALC < 0.84 x 10(9)/L; (2) intermediate risk: patients with at least one risk factor (R-IPI = poor or ALC < 0.84 x 10(9)/L). (3) high risk: patients with both risk factors. This new prognostic score was highly significant in univariate analysis for OS (p = 0.0002), EFS (p < 0.00001) and PFS (p < 0.00001). In multivariate analysis ALC/R-IPI was the most predictive factor for OS (OR = 2.954; p = 0.002) and EFS (OR = 2.381; p < 0.00001) and the only predictive factor for PFS (OR = 4.018; p < 0.00001). Our data, show that ALC at diagnosis has a strong prognostic relevance and is independent from the R-IPI. The new score including both values proved the most powerful predictor at multivariate analysis

Central nervous system immune reconstitution inflammatory syndrome after autologous stem cell transplantation

No abstract availabl

Identification of a Potential "Hotspot" DNA Region in the RUNXI Gene Targeted by Mitoxantrone in Therapy-Related Acute Myeloid Leukemia with t(16;21) Translocation

Genes Chromosomes Cancer. 2009 Mar;48(3):213-21

A rare BCR-ABL1 transcript in Philadelphia-positive acute myeloid leukemia: case report and literature review

Abstract Background Philadelphia (Ph) chromosome results from the reciprocal translocation t(9;22)(q34.1;q11.2) and is diagnostic for chronic myeloid leukemia (CML). However, this translocation is also found in acute lymphoid leukemia (ALL), as well as in rare cases of acute myeloid leukemias (AML). Most patients with CML harbor either the e13a2 or the e14a2 BCR-ABL fusion product, while a small subset of the cases expresses e1a2 or e19a2 transcripts. Moreover, several atypical BCR-ABL1 transcripts, beside the most common e1a2, e13a2 and e14a2, have been described, mainly in patients with CML. However, ALL and de novo AML may also carry BCR-ABL1 atypical transcripts which will confer a poor prognosis. Case presentation A 78-years old male was admitted at our hospital with clinical and laboratory features allowing to make the diagnosis of AML. No evidence of a preceding CML (splenomegaly or basophilia) was found. The karyotype on G-banded metaphases was 46,XY, t(9;22)(q34;q11). While the molecular analysis was ongoing, the patient started treatment based on hydroxyurea followed by 5-aza-2′-deoxycytidine. The molecular biology analysis revealed the simultaneous presence of the common p190 e1a2 and the rare e6a2 isoforms. Because of persistent pancytopenia and presence of blasts, according to the molecular data, he was then switched to tyrosine kinase inhibitors (TKIs) treatment. Nevertheless, after 2 months, the patient was still refractory to second line treatment dying because of a pulmonary infection. Conclusion The atypical p190 e6a2 transcript seems to be associated in AML with aggressive disease. TKI therapy alone does not seem to control the disease. Prompt observations on these patients carrying rare BCR-ABL1 transcripts may help to establish optimal treatment approaches on these aggressive BCR-ABL1 phenotypes in different setting of patients