Il <i>Tratatello delle indulgentie de Terra Sancta</i> secondo il ms. 1106 della biblioteca Augusta di Perugia: edizione e note linguistiche

The research focused on the study of ms. 1106 (= F), one of the witnesses of the Treaty of the Holy Land and the East of the Franciscan missionary Francesco Suriano (Venice 1450-Assisi 1529ca.). The main objective of research was the realization of the scientific edition of F (yet unpublished) and detailed analysis of the language of the text. The work involved, at first, the transcription of 157 cc. of the codex, produced in the monastery of St. Mary Monteluce of Perugia and now kept in the Augusta Library of Perugia. Secondly, the realization of the description of manuscript; the results of the comparison between F and contemporaries codices of Monteluce; handwriting analysis of F; the study of textual tradition; a comprehensive inventory of mss. of Monteluce and S. Lucia di Foligno; a brief biography of the author; a collation of F with other witnesses yet known of the Treaty, another ms. (P) and the princeps (B). Finally, we have conducted in deep linguistics investigations on F, which made possible to point out, next to strong local aspects consistent with the area of origin of the ms., Umbria, other elements which are attributable to its complex layering, to explain the articulated composition story of the text. In particular, there were (mainly in the phonetic) features that tracked back to the language of northern Italy, to explain the venetian origin of Suriano

The HLA Variant rs6903608 Is Associated with Disease Onset and Relapse of Immune-Mediated Thrombotic Thrombocytopenic Purpura in Caucasians

Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare, life-threatening thrombotic microangiopathy caused by severe ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin motifs 13) deficiency, recurring in 30-50% of patients. The common human leukocyte antigen (HLA) variant rs6903608 was found to be associated with prevalent iTTP, but whether this variant is associated with disease relapse is unknown. To estimate the impact of rs6903608 on iTTP onset and relapse, we performed a case-control and cohort study in 161 Italian patients with a first iTTP episode between 2002 and 2018, and in 456 Italian controls. Variation in rs6903608 was strongly associated with iTTP onset (homozygotes odds ratio (OR) 4.68 (95% confidence interval (CI) 2.67 to 8.23); heterozygotes OR 1.64 (95%CI 0.95 to 2.83)), which occurred over three years earlier for each extra risk allele (β -3.34, 95%CI -6.69 to 0.02). Of 153 survivors (median follow-up 4.9 years (95%CI 3.7 to 6.1)), 44 (29%) relapsed. The risk allele homozygotes had a 46% (95%CI 36 to 57%) absolute risk of relapse by year 6, which was significantly higher than both heterozygotes (22% (95%CI 16 to 29%)) and reference allele homozygotes (30% (95%CI 23 to 39%)). In conclusion, HLA variant rs6903608 is a risk factor for both iTTP onset and relapse. This newly identified biomarker may help with recognizing patients at high risk of relapse, who would benefit from close monitoring or intensified immunosuppressive therapy

The features of acquired thrombotic thrombocytopenic purpura occurring at advanced age

Introduction: Acquired thrombotic thrombocytopenic purpura (aTTP) is a rare life-threatening thrombotic microangiopathy (TMA) affecting more frequently women of 30–50 years of age. There is scarce information on the clinical features of aTTP occurring in the elderly. Our goal was to evaluate the impact of an elderly-onset disease on the expression, severity and management of aTTP. Materials and methods: We performed a cross-sectional study of patients enrolled in the Milan TTP Registry (www.ttpdatabase.org) after a first acute episode of aTTP from January 2002 to March 2018. The aTTP diagnosis was suspected on the basis of the presence of thrombocytopenia and microangiopathic hemolytic anemia with no alternative causes, and was confirmed centrally by a severe plasma deficiency of ADAMTS13 activity (&lt;10%). Triggers, clinical manifestations, laboratory parameters, management and outcome of the first acute events of elderly-onset aTTP patients (≥65 years) were compared with those of younger patients. Results: Among 143 eligible patients, 16 (11%) were elderly at onset. In comparison with younger cases they showed a lower rate of bleeding symptoms and severe anemia (30% and 18%), with a trend towards a higher rate of neurological and renal signs and symptoms. These patients were less frequently treated with plasma exchange and corticosteroids and more often with plasma infusion. No difference for gender, triggers and episode outcomes was observed. Conclusions: Older patients with aTTP differed from younger patients mainly for being treated less frequently with plasma exchange and corticosteroids, perhaps for the perceived risks associated with these treatments in the elderly