This unusual case of hepatocellular carcinoma presenting as an incidental malignant portal vein thrombosis without any primary liver lesion is extremely rare. Other reported cases of malignant portal vein thrombosis have been in patients with underlying hepatoma, cirrhosis, or with intrabiliary hepatocelluar carcinoma. In the clinical setting of portal vein thrombosis, imaging studies showing enhancement of the thrombus in the arterial phase are important in leading to the diagnosis of malignancy.
4622 Elevated histone deacetylase (HDAC) enzyme levels have been described in patients with carcinoma and leukemia. Using HDAC inhibitor (HDACi) to treat carcinoma has been promising and is currently undergoing intense research. In order to be better in using HDACi to treat chronic lymphocytic leukemia (CLL), HDAC isoenzyme levels were measured in 32 patients with CLL and compared with 17 normal volunteer controls. Peripheral blood CD20+ cells from patients with CLL and normal volunteer controls were isolated by using Progenitor Cell Isolation Kits (Miltenyi Biotec, Auburn, CA). Isolated CD20+ cells were confirmed by flow cytometry studies to have more than 92% purity. Patient and control CD20+ cells were then lysed in denaturation buffer. Total RNA was extracted, then cDNAs were prepared and quantitative RT-PCR was performed using premixed primer and FAM- and TAMRA-labeled probes obtained from ABI. The results showed: 1) HDAC1, -3, -6, -7, -9, and -10 and SIRT1, -2, and -6 were significantly over-expressed, suggesting that, in CLL, elevated HDAC activity are not restricted to one class. Therefore, HDAC inhibitor therapy may need to be directed to more than one specific class of HDAC; 2) Patients with ZAP70+ were associated with more advanced Rai clinical stage and higher HDAC activity than Zap70– patients and HDAC activity were correlated to the expression levels of CD44 and Pin1, suggesting that higher HDAC values may indicate a poor prognosis and more advanced disease stage. Disclosures: No relevant conflicts of interest to declare.
Background: The pathophysiology of sickle cell disease (SCD) is based on increased blood viscosity due to abnormal red blood cells (RBCs), which causes SCD complications, such as chronic hemolytic anemia, vaso-occlusive crisis with tissue hypoxemia, and organ dysfunction. Effective treatment of Sickle Cell Anemia is to reduce the blood concentration of Hemoglobin S (Hb S) RBCs. Exchange transfusion (ET) remains an effective but possibly underutilized therapy for the management of various acute and chronic complications of SCD such as acute chest syndrome, thromboembolic stroke, splenic and hepatic infarction, right upper quadrant syndrome, multi-organ failure syndrome, or in preparation for surgery by reducing HbS to less than 30%. RBC ET quickly replaces abnormal RBCs with normal RBCs, thus improving oxygen transport while reducing overall blood viscosity. Methodology: To determine the effectiveness of ET in SCD in reducing the total number of admissions and total in-hospital Length of Stay (LOS) in patients, admitted with any of acute complications of SCD, we retrospectively reviewed the medical records of 38 patients between June 15, 2007 and June 15, 2008. The eligibility criteria were age above 18 years old and admission to the hospital for any SCD complication. Nineteen patients had ET with Hb A containing RBCs, generally with an average packed RBC exchange volume of 70–80 ml/kg patient’s weight. Nineteen patients were treated with conventional managements. Three patients were excluded from the ET group because of prolonged LOS due to non-SCD- related complications. Four patients in the non-Exchange group signed against medical advice on the first day of admission and were excluded from analysis. Results: Sixteen (42.1%) patients were male and 22 (57.9%) patients were female. Their ages ranged from 19 to 67 years old, mean (SD) 30.2 (10.8). Of eligible patients, 19 (50%) patients received at least one therapeutic ET during the one year period of the study. In an independent-Samples T test analysis, the mean (SD) LOS were 7.5 (0.6) and 4.2 (0.6) days for the groups without ET and with ET respectively (95% CI = −5.2 to 1.5, p=.0011) (Figure 1). In this small studied group, this resulted average 3.3 days shorter in-hospital stay in ET group, could have saved 62 in-hospital days in the group who received conventional treatments. This number could have been easily much greater, since our hospital’s electronically stored data revealed that during year 2007, there had been 278 SCD admissions in all age groups. There was no mortality in the ET group, nor were any transfusion-related complications reported. In another analysis of one year follow up data, the number of admissions for the patients who never received ET ranged from 1 to 14, mean (SD) 1.7 (2.3) times in year 2007, while in patients who received at least one ET, the number of following admissions ranged from 0 to 2, mean (SD) 0.7 (1.3) times during the next year (95% CI = 0.16 to 1.7, p= .020). Hemoglobin level of patients in conventional treatment group on the day of discharge ranged from 6.5 to 10.7, mean (SD) 8.9 (1.9) and in ET group it ranged from 8.4 to 12.4, mean (SD) 10.2 (1.2) gr/dl (p= .045). Conclusion: Patients with SCD are frequently admitted to hospital for vaso-occlusive crisis and other complications. Exchange transfusion is a reliable, safe, and effective therapeutic modality in SCD patients, in particular during a catastrophic event. ET can significantly reduce the number of hospital admissions and in-hospital stay days in these patients. In addition, patients managed with ET have a better hemoglobin level on discharge. Figure Figure
HIV is associated with progressive iron deposition in the bone marrow, liver and other organs. One of the pathways of developing increased iron stores may be due to sequestration of iron in the macrophages because of chronic inflammation. Increased iron stores might favor HIV progression by impairing key mediators in the host response. The aim of this study is to look at the frequency of other common co morbid conditions that exist in HIV positive patients that cause hyperferritinemia. We retrospectively reviewed the available charts of patients with HIV admitted to the Brookdale Hospital and Medical center over a period of 1 year, who had ferritin levels measured during the hospital admission. The lab values including the ferritin level, admitting diagnosis and other coexisting medical conditions were obtained from the medical charts. The results are summarized in table 1. A total of 56 HIV positive patient’s (33 females and 23 males) charts were reviewed and of those, 66% of patients had ferritin levels greater than 350ng/ml and 32% of them had ferritin level >1000ng/ml. The mean ferritin level was 1247ng/ml in males and 582ng/ml in females. Males had ferritin levels >1000ng/ml in 52% of them, compared to 18% in females. The mean ferritin level was 1203ng/ml in patients with CD4 <100 and 467ng/ml in patients with CD4 counts >100. Forty seven percent of the patients with CD4 <100 had ferritin levels >1000ng/ml. The mean ferritin level in patients with renal failure was 1166ng/ml. Forty six percent of the patients with renal failure (both acute and chronic) had ferritin levels >1000 ng/ml. Patients with coexisting Hepatitis C infection had a mean ferritin level of 976ng/ml and ferritin levels were >1000 ng/ml in 45.4% of the patients .The mean ferritin level was 946ng/ml in patients with liver failure and 41.6% of them had ferritin levels greater than 1000ng/ml. Thirty seven percent of the patients with hemoglobin less than 11 gm/dl had high ferritin compared to 16.6% of patients with hemoglobin level greater than 11 gm/dl. Nine patients died and six of them had ferritin level >1000ng/ml. Twenty six percent of the total 56 patients had transferrin saturation less than 15%. Among the patients with high ferritin (greater than 1000ng/ml) 33.3% had transferrin saturation less than 15%. Transferrin saturation was greater than 50% in 11% patients among the patients with high ferritin levels. As per our study results in HIV positive patients, male sex, renal failure, CD4 count <100, Hepatitis C infection, liver failure and low hemoglobin are more frequently associated with hyperferritinemia. Ferritin levels do not correlate with the transferrin saturation and may not be helpful in diagnosing iron deficiency in HIV positive patients but may be a marker for disease severity. Hepcidin may play a role in the regulation of iron metabolism in HIV positive patient’s needs further studies. Table 1 Factors Percentage of Patients with ferritin >1000 ng/ml Mean ferritin level(ng/ml) Males (23) 52.17 % 1247 ± 903 Females(33) 18 % 582 ± 537 CD4<100 (22) 47 % 1203 ± 963 Renal Failure(15) 46.6 % 1166 ± 690 Hepatitis C (11) 45.4 % 976 ± 853 Liver failure (10) 41.6 % 946 ±1021
Background: The heart is frequently involved in Sickle Cell Anemia (SCA). Cardiomegaly is a usual finding, significant arrythmias and sudden death are common, and 30% of patients with both homozygous and heterozygous SCA develop Pulmonary Arterial Hypertension (PAH), a major risk factor for higher mortality in this population. Brain Natriuretic Peptide (BNP) and echocardiographic data could provide important prognostic and diagnostic information about PAH in SCD. High levels of BNP, which is released from ventricular cardiomyocytes in response to their stretch, reflect cardiac chamber volume and pressure overload in various conditions. In patients with PAH, BNP levels correlate with the severity of Pulmonary Artery Pressure (PAP) elevation and right ventricular dysfunction. In human, the half life of BNP is 20 minutes, reflecting the fluctuation of BNP levels during different stages of any acute cardiac pathology. Methodology: The hypothesis of this prospective IRB approved study was to investigate the BNP level and PAP elevation during an acute Sickle Cell Crisis (SCC), in particular in those with intrathoracic structures involvement. Between December 2006 and July 2008, 81 patients were registered after a written informed consent was obtained. We collected the BNP levels and echocardiographic data of patients with SCD and compared them in two group; those who were admitted with Sickle Cell Crisis (SCC) and those who returned to clinic in Steady State (SS) for follow up. The data were obtained on the first day of admission in SCC group. The primary endpoint was the elevation of the BNP level and the secondary endpoint was elevation of the PAP during a SCC, which were compared with SS patients. The inclusion criterion was age above 18 and having one of the sickle cell syndromes, requiring hospital admission. Results: Forty nine patients (59%) were female, and 34 (41%) patients were male. Their ages ranged from 19 to 65, mean (SD) 30.2 (9.7) years. The mean (SD) levels of BNP were significantly higher in patients who were admitted with one of the acute complications or vaso-occlusive crisis of sickle cell, [177.3 (23.4) pg/ml], when compared with its levels in SS, [34.17 (6.1) pg/ml], (95% CI 61.4 to 225.0, p<.001) (Figure 1). An elevated BNP level was defined as levels more than 100 pg/ml. A further subgroup analysis revealed that the BNP levels were even more significantly higher in patients with acute chest syndrome or other intrathoracic events [(n= 17, mean (SD) 363.6 (121.3) pg/ml], when compared with those of simple acute sickle cell crisis, [(n= 35, mean (SD) 167.7 (26.8) pg/ml] (p=.038) (Figure 1). Topographic data about heart chambers’ sizes, volumes, and pressures were obtained by Echocardiography and compared in two groups. While only 23.1% of patients in SS group had elevated PAP with a mean (SD) of 43 (2.1) mmHg, 41.1% (n=21) of patients with SCC had elevated PAP with mean (SD) 45.9 (2.1) mmHg, with no significant difference between two groups with PAH (p=.608). Conclusion: Patients with either homozygous or heterozygous forms of SCA can have cardiac complications, such systolic and diastolic dysfunction. Hypoxemia leads to raised levels of BNP production. In patients with SCA, an elevated BNP level largely reflects the severity of right ventricular dysfunction associated with PAH. Our data revealed that BNP level and PAP are increased during vaso-occlusive crisis of SCA, in particular during those life-threatening complications, such acute chest syndrome. These changes seem to be temporary and with clinical improvement, the majority of patients’ BNP levels and PAP return to the baseline, although some will never normalize. Figure Figure
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.