A bstract : The life expectancy of patients with thalassemia major has significantly increased in recent years, as reported by several groups in different countries. However, complications are still frequent and affect the patients' quality of life. In a recent study from the United Kingdom, it was found that 50% of the patients had died before age 35. At that age, 65% of the patients from an Italian long‐term study were still alive. Heart disease is responsible for more than half of the deaths. The prevalence of complications in Italian patients born after 1970 includes heart failure in 7%, hypogonadism in 55%, hypothyroidism in 11%, and diabetes in 6%. Similar data were reported in patients from the United States. In the Italian study, lower ferritin levels were associated with a lower probability of experiencing heart failure and with prolonged survival. Osteoporosis and osteopenia are common and affect virtually all patients. Hepatitis C virus antibodies are present in 85% of multitransfused Italian patients, 23% of patients in the United Kingdom, 35% in the United States, 34% in France, and 21% in India. Hepatocellular carcinoma can complicate the course of hepatitis. A survey of Italian centers has identified 23 such cases in patients with a thalassemia syndrome. In conclusion, rates of survival and complication‐free survival continue to improve, due to better treatment strategies. New complications are appearing in long‐term survivors. Iron overload of the heart remains the main cause of morbidity and mortality.
// Serena Bonin 1, * , Alessia Parascandolo 2, * , Cinzia Aversa 1 , Renzo Barbazza 1 , Nobuo Tsuchida 3 , Maria Domenica Castellone 4 , Giorgio Stanta 1 and Giancarlo Vecchio 5, 6, 7 1 Dipartimento di Scienze Mediche, Università di Trieste-Cattinara, Trieste, Italy 2 IRCSS SDN, Naples, Italy 3 Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan 4 Istituto di Endocrinologia e Oncologia Sperimentale G.Salvatore, CNR, Naples, Italy 5 Dipartimento di Medicina Molecolare e Biotecnologie Mediche, Università di Napoli Federico II, Naples, Italy 6 Istituto Superiore di Oncologia, Naples, Italy 7 Istituto Superiore di Oncologia, Genoa, Italy * Co-first authors Correspondence to: Giancarlo Vecchio, email: vecchio@unina.it Keywords: breast cancer; luminal B; lymph node metastasis; α-L-Fucosidase-1; immunohistochemistry Received: October 06, 2017 Accepted: February 01, 2018 Epub: February 07, 2018 Published: March 16, 2018 ABSTRACT Background: The lysosomal enzyme α-L-Fucosidase-1 (FUCA-1) catalyzes the hydrolytic cleavage of terminal fucose residues. FUCA-1 gene is down-regulated in highly aggressive and metastatic human tumors as its inactivation perturbs the fucosylation of proteins involved in cell adhesion, migration and metastases. Results: Negativity to FUCA-1 was significantly related to the development of later recurrences in breast cancer patients with lymph node involvement at diagnosis. Cancer specific survival of luminal B LN+ patients was influenced by FUCA-1 expression as luminal B LN+ patients with positive expression had a longer cancer specific survival. FUCA-1 mRNA expression was inversely related to cancer stage and lymph node involvement. WB and qPCR analysis of FUCA-1 expression in breast cancer-derived cell lines confirmed an inverse relationship with tumor aggressiveness. Conclusions: This study shows that, within LN+ breast cancer patients, FUCA-1 is able to identify a sub-set of non recurrent patients characterized by the positive expression of FUCA-1 and that, within luminal B LN+ patients, the expression of FUCA-1 predicts longer cancer specific survival. Methods: We have analyzed FUCA-1 in 305 breast cancer patients by Immunohistochemistry (IHC), and by qPCR in breast cancer patients and in breast cancer cell lines.
Tumor-promoting agents are known to inhibit the specific differentiation processes of several animal cell systems in vitro, including the Friend leukemia cell system. We have examined the effect of 12-O tetradecanoylphorbol-13-acetate (TPA) on the latter system and have investigated its action on Friend virus expression. At a concentration of 16.7 nM, TPA inhibits the dimethyl sulfoxide-induced Friend cell terminal differentiation and, at the same time, enhances the expression of the Friend virus genome, as demonstrated by a 2-fold increase in the amount of reverse transcriptase-containing particles released into the culture fluid and in the levels of virus-specific intracytoplasmic RNA. The greatest effect of TPA is evident after 24 hr of treatment. At this time, TPA exerts also its strongest effect upon the induction of the plasminogen activator. Our results indicate that two specific effects of TPA, i.e., block of differentiation and induction of plasminogen activator, correlate well in the Friend cell system with an extracellular and intracellular increase in virus expression.
Immune thrombocytopenia (ITP) is an autoimmune disorder characterized by isolated thrombocytopenia. Many viruses and some vaccines have been identified as triggering the autoimmune process, including parvovirus, human immunodeficiency virus, Epstein-Barr virus, rubella, and measles. However, ITP in association with coronavirus disease 2019 (COVID-19) vaccination has not been reported so far. We describe the cases of two young girls affected by ITP presenting a quick reduction of platelet count after receiving Pfizer-BioNTech COVID-19 vaccine.
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