Peculiar Hepatocellular Carcinoma Clinical Findings in patients with Thalassemia Syndromes.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 5108-5108
Author(s):  
Gaetano Restivo ◽  
Franco Valenza ◽  
Fabio D'Amato ◽  
Angela Vitrano ◽  
Paolo Rigano ◽  
...  

Abstract Abstract 5108 Background Hepatocellular carcinoma (HCC) is the most common primary hepatic cancer, and the fifth and eighth most common malignancy worldwide in men and women, respectively. Hepatitis B and C, primary and secondary hemochromatosis and cirrhosis have been identified as major risk factors. Hepatitis C or B virus infection and secondary hemochromatosis are the major cause of morbidity and mortality. In the last years, HCC on cirrhosis is a common reported complication in patients with thalassemia syndromes1,2,3. In non thalassemia patients HCC is a late complication of cirrhosis and prognosis depends on suitability for treatment. In this group, the last is low (40%) considering that cirrhosis severe stage C, according to Child-Pugh score is generally observed (personal communication). Aim of the study describing, in a homogeneous cohort of thalassemia patients, the main clinical findings of HCC. Patients and Methods All subjects with risk factors for HCC as iron overloading, HCV or HBV chronic infection, histological evidence of cirrhosis were undergone every six months to an ultrasound evaluation associated with alphaphetoprotein determination. Nine new cases, since January 2000 until July 2009, were detected. Table I shows the main clinical findings. Mean value of serum ferritin levels, in the last 2 years before diagnosis, was 1049±721. All patients except one were anti-HCV positive (Table I). Seven of them were HCV-RNA positive (Table I). None of them had previous HBV infection. Results Only one patient had severe stage C, according to Child-Pugh score. None of the patients had significant high levels of alphaphetoprotein (AFP) at diagnosis. However, a slightly increase of AFP levels was shown, during the 6 months before diagnosis, in 50% of cases. Three patients had a delayed diagnosis because of atypical imaging for HCC at TC scan or a negative previous histology for HCC after liver biopsy. Five patients had multifocal HCC at the diagnosis (Table I). Four showed an unifocal HCC, developing two of them multifocal HCC during the follow-up (Table I). Four patients died during the follow-up for decompensated cirrhosis. Five patients are alive after treatment. Main used treatments are shown on Table I. The overall mean survival was 28±25 months (range 3-64). Conclusions 1) almost all talassemia patients with HCC are suitable for treatment ; 2) baseline liver function is the most main factor conditioning survival and suitability for treatment ; 3) periodical serum AFP determnations can reveal a slight increase; 3) iron overloading alone can be related to the development of HCC in a liver with still good functional reserve, giving more opportunity for treatment 4) monitoring for HCC by liver ultrasound evaluation, every six months, in at risk patients with thalassemia syndromes should be mandatory. Disclosures No relevant conflicts of interest to declare.

2010 ◽  
Vol 28 (30) ◽  
pp. 4587-4593 ◽  
Author(s):  
Lee Mei-Hsuan ◽  
Hwai-I Yang ◽  
Sheng-Nan Lu ◽  
Chin-Lan Jen ◽  
Shiou-Hwei Yeh ◽  
...  

Purpose Hepatitis C virus (HCV) contributes to one third of hepatocellular carcinoma cases worldwide. Long-term predictors for HCV-related hepatocellular carcinoma are essential for early intervention. Serum HCV RNA and ALT levels and HCV genotype were assessed for their predictability of hepatocellular carcinoma risk. Methods A prospective cohort of 925 participants positive for antibodies against HCV and age 30 to 65 years was recruited and followed from 1991 to 2006. Serum HCV RNA and ALT levels and HCV genotypes at enrollment and during follow-up were examined. Newly developed hepatocellular carcinoma was identified by health examination and computerized linkage with national cancer registration and death certification profiles. Multivariate adjusted hazard ratios with 95% CIs were estimated using Cox regression models. Results Fifty-five participants newly developed hepatocellular carcinoma during 8,476 person-years of follow-up, giving an incidence rate of 648.9 per 100,000 person-years. The cumulative hepatocellular carcinoma risk increased from 1.1% for HCV RNA seronegative status to 6.4% for low HCV RNA levels and to 14.7% for high HCV RNA levels (P < .001). The cumulative risk also increased with elevated serum ALT levels from 1.7% for persistently ≤ 15 U/L to 4.2% for ever more than 15 U/L but never more than 45 U/L and to 13.8% for ALT ever ≥ 45 U/L (P < .001). Having HCV genotype 1 was associated with a higher cumulative hepatocellular carcinoma risk (12.6%) than not having HCV genotype 1 (4.5%; P < .001). Conclusion Elevated serum levels of HCV RNA and ALT and HCV genotype 1 infection are independent risk predictors of hepatocellular carcinoma. These findings have strong implications for the management of chronic HCV.


Biomedicines ◽  
2020 ◽  
Vol 8 (6) ◽  
pp. 175
Author(s):  
Sara Kishta ◽  
Ashraf Tabll ◽  
Tea Omanovic Kolaric ◽  
Robert Smolic ◽  
Martina Smolic

Although hepatitis C virus (HCV) RNA may be eliminated from blood circulation by direct-acting antivirals (DAA) therapy as assessed by real-time polymerase chain reaction (PCR), HCV RNA can still be present in liver tissue, and this is known as occult HCV. There has been a lot of controversy surrounding the recurrence of hepatocellular carcinoma (HCC) after DAA treatment of hepatic cells infected with chronic HCV. One of the main risk factors that leads to de novo HCC is the chronicity of HCV in hepatic cells. There are many studies regarding the progression of HCV-infected hepatic cells to HCC. However, there is a lack of research on the different molecular mechanisms that lead to the progression of chronic HCV infection to HCC, as well as on the effect of HCV on the alteration of DNA ploidy, which eventually leads to a recurrence of HCC after DAA treatment. In this review article, we will address some risk factors that could lead to the development/recurrence of HCC after treatment of HCV with DAA therapy, such as the role of liver cirrhosis, the alteration of DNA ploidy, the reactivation of hepatitis B virus (HBV), the role of cytokines and the alteration of the immune system, concomitant non- alcoholic fatty liver disease (NAFLD), obesity, alcohol consumption and also occult HCV infection/co-infection. Clinicians should be cautious considering that full eradication of hepatocarcinogenesis cannot be successfully accomplished by anti-HCV treatment alone.


2010 ◽  
Vol 20 (10) ◽  
pp. 2397-2404 ◽  
Author(s):  
Min Jung Park ◽  
Young-sun Kim ◽  
Won Jae Lee ◽  
Hyo K. Lim ◽  
Hyunchul Rhim ◽  
...  

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 4991-4991
Author(s):  
Andrés López ◽  
Roser Solans-Laque ◽  
Josep A Bosch-Gil ◽  
Mercedes Gironella ◽  
Noelia Purroy ◽  
...  

Abstract Abstract 4991 Background Primary Sjögren Symdrome (PSS) is the second common systemic autoimmune rheumatic disease whose main histological feature is a focal infiltration of the exocrine glands by activated B and T lymphocytes. PSS is considered a benign autoimmune condition with a chronic indolent course. However, several reports have noted an increased incidence of malignant lymphoma with an estimated risk of up to 44 times greater than in the normal population. The aims of the present study are to assess the cumulative incidence of lymphoma in patients diagnosed of PSS, as well as to identify risk factors for developing this condition. Methods All patients diagnosed since 1988 and followed-up for longer than 2 years at our institution, were included. The pathological, immunological, and clinical features of each case of PSS were retrospectively examined. These data were cross-examined with the cumulative incidence of lymphoma in this group of patients, in order to determine common findings in patients later developing lymphoid malignancies. Results 272 patients had been diagnosed as having a PSS in this time period, of which 244 [9 males, 235 females; median age 58 years (range 17 to 88)] had been followed for more than 2 years. After a median follow up of 9.6 years, 11 (4.5%) patients developed a lymphoma, after 6 to 171 months from diagnosis. The cumulative risk for developing lymphoma at 5, 10 and 15 years was 3.4, 4.1, and 9.3%, respectively. Risk factors associated with the development of lymphoma were skin purpura/vasculitis, lymphopenia and low levels of C3 and C4 complement fractions. Low levels of C3 and C4 were also found to be associated with an earlier development of lymphoma. Conversely, skin purpura/vasculitis and lymphopenia were both associated with the development of lymphoma at a later stage. The hystological analysis showed mucosal-associated lymphoid tissue lymphoma in 5 patients, diffuse large B cell in 3, and follicular lymphoma in 3 cases. Clinically, lymphomas had extranodal presentation in 8 out of the 11 cases, involving the salivary glands in 5, lacrimal glands in 1, and soft tissues and lung in 1 case, respectively. Only 3 patients had received immunosuppressive therapy prior to the diagnosis of lymphoma, and EBV was not found in the histological examination of any of these cases. Conclusion Patients with PSS are at a higher risk for developing lymphoma. Our study suggests low levels of C3 or C4, as well as the presence of purpura or lymphopenia delimitates a group of patients at a higher risk, so that such patients may benefit from a closer follow up. Disclosures No relevant conflicts of interest to declare.


Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 5065-5065
Author(s):  
Marco Montanaro ◽  
Roberto Latagliata ◽  
Enrico Montefusco ◽  
Michele Cedrone ◽  
Nicoletta Villivà ◽  
...  

Abstract Abstract 5065 The Latium is a region of central Italy that counts approximatively 5.600.000 of residents. We reported herein a preliminary analysis of 1572 patients affected by MPN diagnosed in the last 20 years and treated in 9 of the Centers belonging to our group. Our centers are mainly located in Rome (7 centers) and neighboring districts (2 centers, Latina and Viterbo). The diagnosis was performed according to PVSG criteria until 2001 and then according to the WHO criteria: the majority of the patients underwent bone marrow biopsy. 218 patients were affected by primary myelofibrosis (PMF), 779 by Essential thrombocytemia (ET) and 575 by Policytemia Vera (PV). Epidemiological and clinical findings of all patients at diagnosis as well as thrombotic complications and evolution are reported in the table:ET (779 pts)PV (575 pts)PMF (218 pts)M/F288/491350/225134/84Age (yrs)59 (r: 20-93)60 (r: 19-91)67 (r: 30-86)WBC (x109/L)9192 (+/− 3483 SD)10423 (+/− SD 4307)13313 (+/−5220)Hb (g/dL)13, 9 (+/− 1,8)18,1 (+/− 2,3)11,7 (+/− 2,8)Plts (x 109/L)877 (+/− 353)445 (+/−247)457 (+/− 358)Splenomegaly (%)193579Bone marrow biopsy (performed/total))557/779 (71,5%)254/575 (44,2%)207/218 (95%)JAK-2 V617F mut (%)284/484 (59%)256/369 (69,4%)69/116 (59,5 %)Jak-2 allele burdenPerformed in 188/284 cases 26,49% (SD +/− 24)Performed in 199/256 cases: 59,4 % (SD +/−30,9)Performed in 51/69 cases 57,5% (SD +/− 29,4)Median Follow-up (years)7,157,863,89Thrombosis pre-diagnosis of MPNN. of patients (%)108/779 (13,8%)100/575 (17,4%)29/218 (13,3%)(arterial+venous)74+3476+2424+5Thrombosis post diagnosis of MPNN. of patients (%)71/779 (9,1%)66/575 (11,5%)20/218(9,17%)(arterial+venous)42+2942+2412+8EvolutionMyelofibrosis9/779 (1,1%)24/575 (4,1%)AML9/779 (1,1%)13/575 (2,3%)17/143 (11,8%) Comments: In our opinion, this casistic of patients with different types of MPNs reflects clinical presentation and evolution of three variants of the same disease. Many clinical findings in our unselected cohort of patients are similar to those reported in literature; in particular, thrombotic events were seen in about 13 – 17% of patients, without any correlation with Jak-2 status in both pre-diagnosis and follow-up. However, 2 main differences were noted: a lower incidence of JAK-2 V617F mutation among our PV patients and a lower rate of evolution in myelofibrosis and AML among our ET and PV patients. Both these features warrant further insights to be fully elucidated. Disclosures: No relevant conflicts of interest to declare.


Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 1735-1735
Author(s):  
Serena Rupoli ◽  
Gaia Goteri ◽  
Picardi Picardi ◽  
Lucia Canafoglia ◽  
Giorgia Micucci ◽  
...  

Abstract Abstract 1735 Background: Essential Thrombocytemia (ET) is a myeloproliferative neoplasm characterized by increased risk of vascular events. Established thrombosis risk factors are age and previous vascular events. The clinical and prognostic relevance of WHO histologic criteria for ET and prefibrotic/early Primary Myelofibrosis (PMF) has been well recognized. Our aim was to evaluate the correlation between histologic interpretation and vascular events in our series of thrombocytemias. Material and methods: From our files, we retrieved all patients consecutively diagnosed as having ET with complete clinical data (N = 283) who had undergone to a bone marrow trephine biopsy before any treatment at or within 1 year of diagnosis (N= 133). The histologic slides were reviewed in order to separate true ET cases from early/prefibrotic PMF; vaso-occlusive events at diagnosis and in the follow-up were than compared in the two groups. Results: Histologic review reclassified 61 cases as ET and 72 cases as prefibrotic/early PMF. Prefibrotic/early PMF showed a significant higher prevalence of thrombosis history and thrombotic events at diagnosis, and an increased leukocyte count than ET (22% vs 8%, 15.2% vs 1.6%, 8389/mmc vs 7500/mmc, respectively); furthermore, venous thromboses (mainly atypical) were relatively common in PMF, as opposed to WHO-defined ET. During follow-up, patients with prefibrotic PMF, although younger, showed a significant higher risk of developing thrombosis: the 15-year risk of thrombosis was 48% in prefibrotic PMF (grade 0), 16% in early PMF (grade 1, 2) and 17% in ET. Multivariate analysis confirmed that age and histopathology are independent risk factors for thrombosis during follow-up. Patients older than 60 or with prefibrotic PMF are high risk patients whereas those younger and with non prefibrotic PMF or ET should be considered at low risk (20-year risk of thrombosis 47% vs 4%, p=0.005). Conclusion: The results of present study indicate prefibrotic PMF as a myloproliferative neoplasm with the highest tendency to develop vascular events compared to early PMF and ET. Therefore we suggest to include histopathology interpretation in the risk stratification of so-called ET patients. Disclosures: No relevant conflicts of interest to declare.


Blood ◽  
2001 ◽  
Vol 97 (5) ◽  
pp. 1196-1201 ◽  
Author(s):  
Georgia B. Vogelsang

Allogeneic stem cell transplantation (SCT) is now a commonplace procedure. Clinicians who care for patients with hematologic malignancies and aplastic anemia are almost certain to follow up patients after SCT. This review is intended to help clinicians observe patients for probably the most important late complication of SCT, chronic graft-versus-host disease (GVHD). It reviews the pathophysiology, risk factors, clinical manifestations, evaluation, treatment, and supportive care of chronic GVHD.


2018 ◽  
Vol 31 (Supplement_1) ◽  
pp. 173-173
Author(s):  
Francesco Puccetti ◽  
Paolo Parise ◽  
Uberto Fumagalli Romario ◽  
Andrea Cossu ◽  
Stefano De Pascale ◽  
...  

Abstract Background Oesophagectomy is the mainstay of curative treatment for oesophageal cancer and post-oesophagectomy diaphragmatic hernia (PODH) represents a potentially life-threatening surgical complication characterized by an underestimated occurrence rate and unknown related risk factors. This study analyses the experience of two tertiary designated centers in order to evaluate key elements concerning development and treatment of PODH. Methods A cohort of consecutive patients affected by a clinically resectable oesophageal cancer (any T, any N and M0) underwent Ivor-Lewis oesophagectomy between March 1997 and April 2017 according to three different approaches: totally open incision procedure (OILO), hybrid (HILO) and totally mininvasive to esophagectomy (MILO). All patients were retrospectively observed in the context of a postoperative calendarised follow-up in order to record the incidence and postrepair results of PODH. Results 414 patients underwent Ivor-Lewis oesophagectomy for cancer and 22 (5.3%) developed PODH within a median follow-up period of 16 months (6 - 177). Surgical repair was generally applied by the mean of laparoscopic cruroplasty (77%) with a conversion rate of 24%. Postoperative morbidity did not include early recurrences but exclusively cardio-pulmonary complications (5 patients) with one case of respiratory failure leading to death. The discharge was reached after a median hospital stay of 6 days (2 - 95) while 3 recurrences (14%) occurred over a median follow-up period of 10.1 months. A wide univariate analysis identified statistically significant associations between PODH occurrence and the administration of preoperative chemoradiotherapy, the complete pathological response (CPR) and a lymph node harvest (LNH) larger than 33 stations (p-value of 0.016, 0.001 and 0.024 respectively). The strong influence of an extended LNH was confirmed by the multivariable analyses (0.026) along with CPR which should however be considered as longer survival-related bias. Conclusion The minimally invasive surgery and the neoadjuvant chemoradiotherapy represent a considerable part of multimodal treatment for oesophageal cancer presenting a not statistically significant association with PODH development while a LNH including more than 33 nodes resulted to be an independent risk factor mirroring the extent of surgical demolition in oesophagectomy. Disclosure All authors have declared no conflicts of interest.


1995 ◽  
Vol 10 (5) ◽  
pp. 226-233
Author(s):  
Kamal G. Shaker ◽  
Helen M Hollingsworth ◽  
Richard S. Irwin ◽  
Cynthia B. Umali

Tracheobronchial injuries, once rare complications of blunt trauma to the anterior neck and chest, are becoming more common. Traffic accidents account for most of these injuries. There are several possible mechanisms for airway rupture, the site of which is dictated by the location of the trauma and the points of airway fixation. The vast majority of cervical tracheal injuries occur above the fourth tracheal ring, whereas thoracic tracheal and bronchial lesions tend to occur in the vicinity of the carina. The presence of respiratory distress and signs of air leak, such as subcutaneous emphysema and persistent pneumothorax despite thoracostomy tube drainage, characterize many of these injuries. In some patients, however, the paucity of clinical findings leads to a delay in diagnosis until a late complication, such as lung collapse or suppuration; occurs. Flexible bronchoscopy remains the most valuable tool for diagnosis. Early surgical repair is recommended except for some minor injuries, which can be managed expectantly and with close follow-up. Patients who reach the hospital alive have a good prognosis.


Sign in / Sign up

Export Citation Format

Share Document