A new insight on postural tachycardia syndrome in 102 adults with hypermobile Ehlers-Danlos Syndrome/hypermobility spectrum disorder

There is an association between joint hypermobility, hypermobile Ehlers-Danlos syndrome (hEDS) and different forms of orthostatic intolerance. Objective: to explore autonomic profile in a large cohort of adults with hEDS and hypermobility spectrum disorder (hEDS/HSD) with a multimodal approach. In this observational retrospective study, heart rate, blood pressure and baroreflex sensitivity were estimated in 102 hEDS/HSD subjects during deep breathing, Valsalva maneuver, standing up: 30-15 ratio, Head-Up Tilt and sustained handgrip. Abnormal results and head-up tilt test were common and included postural orthostatic tachycardia syndrome (POTS; 48%), orthostatic intolerance (25.5%) and hypotension (3.9%). Baroreflex sensitivity was significantly different in individuals with POTS compared to the others. This study confirms the high rate and heterogeneity of abnormal autonomic regulation in hEDS/HSD, and suggests the baroreflex sensitivity might distinguish comorbid POTS from other profiles in this subgroup of patients. Abnormal autonomic regulation is common in adults with hEDS/HSD and should be regularly assessed for tailoring the management approach

Case report: A Saprochaete clavata (Magnusiomyces clavatus) severe infection effectively treated with granulocyte transfusion in a young patient with myeloid sarcoma

Myeloid sarcoma is a hematologic malignancy consisting of extramedullary tissue involvement by myeloid blasts, usually considered as acute myeloid leukemia and treated accordingly. The disease itself, together with chemotherapy and disease-associated factors, may have an impact in increasing the risk of developing severe and frequently life-threatening infections. Herein, we describe the case of a patient with a right breast skin lesion, histologically diagnosed myeloid sarcoma, who developed a severe disseminated fungal infection by Saprochaete clavata (Magnusiomyces clavatus), during the first consolidation course of chemotherapy. Despite maximum antifungal therapy, the infection progressed and the fungus continued to be isolated until granulocyte transfusion therapy was initiated. Our experience suggests that patients with profound and long-lasting neutropenia could benefit from granulocyte transfusions as additional therapy in severe fungal infections resistant to broad-spectrum antimicrobial therapy

P499: APPLICABILITY OF 2022 CLASSIFICATIONS OF ACUTE MYELOID LEUKEMIA IN THE REAL-WORLD SETTING

Background: The increasing knowledge of molecular characterization in acute myeloid leukemia (AML) led to the necessity to fully evaluate the genetic profile also for clinical purposes. These efforts resulted in the release of 2022 new editions of AML classification and prognostication systems, including the 5th edition of The World Health Organization (WHO) classification, the International Consensus Classification (ICC), and the European LeukemiaNet (ELN) recommendations for AML prognosis. Aims: We aimed to provide a real-world application of the WHO 2022, ICC and ELN 2022 classifications in the real-world setting, to unravel differences and similarities, and to test their implementation in clinical AML diagnosis. We particularly focused on secondary AML, myelodysplasia (MDS) related. Methods: We selected a cohort of 1001 cases diagnosed with AML according to the WHO 2016 and the ELN 2017 classifications. Where available (44.9% of cases), information concerning a previous history of an antecedent MDS or MDS/Myeloproliferative neoplasm (MPN), as well as a previous exposure to cytotoxic therapies were considered for defining secondary AML (s-AML) and therapy-related AML (t-AML), respectively. Survival outcome was available for 84.4% patients. Results: The overall diagnostic changes between the WHO 2016, compared to WHO 2022 and ICC classifications were 22.8% and 23.7% respectively, with a 13.1% difference in patients’ distribution between ICC and WHO 2022. The “not otherwise specified” (NOS) by ICC and “defined by differentiation” by WHO 2022 categories shrank compared to WHO 2016 (24.1% and 26.8% respectively, vs 38.7%), particularly due to an expansion of MDS-related categories. The 92.7% and the 74.4% of RUNX1-mutated AML were re-classified respectively by the ICC into AML with MDS-related gene mutations and by WHO 2022 into the AML myelodysplasia related (MR) category, although the latter considers RUNX1 mutations lacking of sufficient unifying characteristics. Of 397 cases with a MDS-related AML according to ICC, 55.9% were definable by the presence of a MDS-related karyotype. More than 75.0% of s-AML and t-AML cases presented a MDS-related genetic profile according to both new 2022 diagnostic classifications. The overall re-stratification between ELN 2017 and 2022 accounted for 12.9% (4.0% favorable to intermediate and 8.1% intermediate to adverse risk). The majority of s-AML and t-AML (83.1%) fell into the ELN 2022 adverse risk group. Stratifying the 213 AML classified as favorable risk by ELN 2017, the difference in OS between ELN 2022-defined favorable and intermediate risk groups was statistically significant (p<0.01). We also focused on the heterogeneous group of patients with normal karyotype and adverse risk mutations according to the ELN 2022: the survival outcome was significantly inferior in patients with multiple versus single MDS-related gene mutations (p<0.05). Summary/Conclusion: The 2022 revisions of AML classification led to a significant improvement of diagnostic schemes. In the real-world setting, conventional cytogenetics, usually easily available and less expensive than molecular characterization, correctly stratified 56% of AML MDS-related, thereby maintaining a diriment diagnostic role. Although the secondary nature of AML (prior MDS or MDS/MPN and therapy-related) is now applied as “diagnostic qualifiers”, it maintains a predictive role for defining an adverse outcome according to the ELN 2022. Considering the similarities between WHO and ICC diagnostic schemes, a tentative to generate a unified model taking into account practical and socio-economic issues is desirable

FEVER OF UNKNOWN ORIGIN AND MULTIDRUG-RESISTANT ORGANISMS COLONIZATION IN AML PATIENTS.

Abstract. Background: Colonization by multidrug-resistant organisms (MDRO) is a frequent complication in hematologic departments, which puts patients at risk of life-threatening bacterial sepsis. Fever of unknown origin (FUO) is a condition related to the delivery of chemotherapy in hematologic malignancies, in which the use of antibiotics is debated. The incidence, risk factors, and influence on the outcome of these conditions in patients with acute myeloid leukemia (AML) are not clearly defined. Methods: We retrospectively analyzed 132 consecutive admissions of non-promyelocytic AML patients at the Hematology Unit of the University Tor Vergata in Rome between June 2019 and February 2022. MDRO swab-based screening was performed in all patients on the day of admission and once weekly after that. FUO was defined as fever with no evidence of infection. Results: Of 132 consecutive hospitalizations (69 AML patients), MDRO colonization was observed in 35 cases (26%) and resulted independently related to a previous MDRO colonization (p=0.001) and length of hospitalization (p=0.03). The colonization persistence rate in subsequent admissions was 64%. MDRO-related bloodstream infection was observed in 8 patients (23%) and correlated with grade III/IV mucositis (p=0.008) and length of hospitalization (p=0.02). FUO occurred in 68 cases (51%) and correlated with an absolute neutrophilic count <500μ/L at admission (0.04). Conclusion: In our experience, MDRO colonization is a frequent and difficult-to-eradicate condition that can arise at all stages of treatment. Prompt discharge of patients as soon as clinical conditions allow could limit the spread of MDRO. In addition, the appropriate use of antibiotics, especially in the case of FUO, and the contraction of hospitalization length, when feasible, are measures to tackle the further spread of MDRO

Piccoli musei d'arte in Umbria

Il volume rappresenta il segmento di un programma di ricerca finanziato dal Murst (1996) e cofinanziato per il 1997 dal titolo 'Piccoli musei d'arte in Piemonte, Liguria, Emilia Romagna, Umbria, Sicilia' (coordinatore nazionale Antonio Piva del Politecnico di Milano, responsabile dell'unità di ricerca Bruno Toscano, Dipartimento di studi storico-artistici, archeologici e sulla conservazione di Roma Tre). Costituisce la prima ricognizione sistematica dei musei d'arte in Umbria dopo il sisma del 1997, sulla spinta della necessità di verificarne le condizioni, le urgenze di ristrutturazione, restauro e riallestimento. Il termine 'piccoli' applicato ai musei presi in considerazione - una trentina in tutto - non è stato inteso nel senso di meno importanti: l'Umbria è caratterizzata da una rete di musei talmente fitta da meritare la definizione di 'museo diffuso'.L'analisi della situazione post-terremoto ha costituito la base di partenza per un'azione di valorizzazione delle potenzialità dei piccoli musei in quanto luoghi di ricerca, di studio e di riqualificazione culturale in un contesto attento anche alla promozione economica e turistica

Effects of propofol or sevoflurane anesthesia induction on hemodynamics in patients undergoing fiberoptic intubation for cervical spine surgery: A randomized, controlled, clinical trial

Background and Aims: In patients undergoing surgery for cervical myelopathy, induction of general anesthesia can induce systemic arterial hypotension that may worsen spinal cord hypoperfusion and precipitate spinal injury. In this randomized, controlled, clinical trial study, we compared the hemodynamic changes related to anesthesia induction with intravenous (IV) propofol- and sevoflurane-based inhalational induction in patients undergoing fiberoptic intubation for cervical spine surgery. Material and Methods: A total of 72 patients were studied. Hemodynamic effects were assessed measuring mean arterial pressure (MAP), and the echocardiographic evaluation of the left ventricular function. A Student's t-test with Bonferroni correction or Chi-squared test was used, when appropriate, to assess differences in hemodynamic (extent of MAP drop and incidence of episodes of severe arterial hypotension) and other variables (occurrence and duration of episodes of apnea). Results: Patients assigned to total IV anesthetic approach had a lower MAP, and more significant changes in cardiac function compared to those who received the inhalational approach (68.1 ± 9.3 mmHg vs. 75.5 ± 10.3 mmHg; 25% vs. 5.5%). Conclusion: Anesthesia induction with both propofol or sevoflurane is safe and effective. However, total IV anesthesia induction is associated with more pronounced MAP drop which can worsen spinal cord hypoperfusion

Applicability of 2022 classifications of acute myeloid leukemia in the real-world setting

The increasing knowledge of molecular genetics of acute myeloid leukemia (AML) urged the update of previous diagnostic and prognostic schemes, which resulted in the development, in 2022, of the World Health Organization (WHO), the International Consensus Classification (ICC) and the new European LeukemiaNet (ELN) recommendations. We aimed to provide a real-world application of the new models, to unravel differences and similarities, and to test their implementation in clinical AML diagnosis. A total of 1001 patients diagnosed with AML were re-classified according to the new schemes. The overall diagnostic changes between the WHO 2016, compared to WHO 2022 and ICC classifications were 22.8% and 23.7%, respectively, with a 13.1% difference in patients' distribution between ICC and WHO 2022. The 2022 ICC "not otherwise specified" and WHO "defined by differentiation" AML categories shrank as compared to WHO 2016 (24.1% and 26.8% respectively, vs 38.7%), particularly due to an expansion of the myelodysplasia (MDS)-related group. Of 397 patients with a MDS-related AML according to the ICC, 55.9% were defined by the presence of a MDS-related karyotype. The overall re-stratification between ELN 2017 and 2022 was 12.9%. The 2022 AML classifications led to a significant improvement of diagnostic schemes. In the real-world setting, conventional cytogenetics, usually rapidly available and less expensive than molecular characterization, stratified 56% of secondary AML, still maintaining a powerful diagnostic role. Considering the similarities between WHO and ICC diagnostic schemes, a tentative to generate a unified model is desirable

Mechanical ventilation in neurocritical care patients: a systematic literature review

none5Introduction: Neurocritical care (NCC) patients often require prolonged mechanical ventilation, and they are at high risk of respiratory complications. Therefore, the potential benefit role of protective lung ventilation (PLV), which demonstrated to reduce postoperative complications in patients with acute distress respiratory syndrome, has been suggested even on NCC patients. However, PLV can increase intracranial pressure as result of permissive hypercapnia and of high airway pressures during recruitment maneuvers. The aim of this review (PROSPERO registration number: CRD42015027011) is to describe the ventilatory strategies, and in particular PLV, commonly used in NCC patients. Areas covered: We selected a total of 16 clinical studies, searching on PubMed and EMBASE databases, reporting original information on the MV on patients receiving NCC after acute brain injury, published in the last 10 years, in English language. Some of the included studies report data on a limited sample size. Expert commentary: The use of PLV techniques (PEEP, recruitment maneuvers, etc) in NCC patients is controversial. There is a wide variability among different centers in the treatment strategies and respiratory management of NCC patients, and there is the need for shared diagnostics and therapeutic studies, in order to improve the patients’ outcome.Borsellino, Beatrice; Schultz, Marcus J.; Gama de Abreu, Marcelo; Robba, Chiara; Bilotta, FedericoBorsellino, Beatrice; Schultz, Marcus J.; Gama de Abreu, Marcelo; Robba, Chiara; Bilotta, Federic