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- COVID- 19 in Patients Affected by Red Blood Cell Disorders, Results From the European Registry ERN-EuroBloodNet.Publication . Puyo, Pablo; Christou, Soteroula; Campisi, Saveria; Rodríguez-Sánchez, Maria A; Reidel, Sara; Perez-Hoyo, Santiago; Mota, Miriam; Savvidou, Irene; Rekleiti, Anna; Salvo, Alessandra; Voi, Vincenzo; Ferrero, Giovanni Battista; Mandrile, Giorgia; Gaglioti, Carmen Maria; Cela, Elena; Ponce-Salas, Beatriz; Bardón-Cancho, Eduardo J; Flevari, Pagona; Voskaridou-Dimoula, Ersi; Nur, Erfan; Biemond, Bart J; Delaporta, Polynexi; Beneitez-Pastor, David; Collado Gimbert, Anna; Spasiano, Anna; Besse-Hammer, Tatiana; Lafiatis, Ioannis G; Dedeken, Laurence; Raso, Simona; Ruiz-Llobet, Anna; Bagnato, Sabrina; Labarque, Veerle; Glenthøj, Andreas; Ruffo, Giovan Battista; Guerzoni, Maria Elena; Hafraoui, Kaoutar; Pistoia, Laura; Rosso, Rosamaria; Tagliaferri, Laura; Gonzalez-Urdiales, Paula; Benghiat, Fleur Samantha; de Montalembert, Mariane; Teles, Maria Jose; Vanderfaeillie, Anna; Bertoni, Elisa; Cuzzubbo, Daniela; Ferreira, Teresa; Saunders, Christopher J; Stiakaki, Eftichia; Van de Velde, Ann L; Diamantidis, Michael D; Kerkhoffs, Jean-Louis H; Oliveira, Marisa I; Quota, Alessandra; Russo, Roberta; Van Damme, An; Argüello Marina, María; Lorite Reggiori, Mikael; Rijneveld, Anita W; Rodríguez Gallego, Alexis; Colombatti, Raffaella; Iolascon, Achille; Taher, Ali; Gulbis, Béatrice; Roy, Noémi B A; Mañú-Pereira, María Del MarBackground: Despite several publications covering patients from multiple centers, no international registry covered all patients with red blood cell diseases (RBCD) affected by COVID- 19. The ERN-EuroBloodNet's registry provided real-time registration of SARS-CoV- 2 patients with RBCD, promoting timely disease-specific knowledge sharing during the pandemic's early stages. Procedures: The study evaluated patient distribution, the infection across different RBCDs, and severity risk factors across similar healthcare systems, using data collected from the ERN-EuroBloodNet's REDCap platform. Results: From April 2020 to April 2023, 681 infections were recorded among 663 patients, of which 373 had transfusion-dependent thalassemia or non-transfusion-dependent thalassemia (TDT/NTDT), and 269 had sickle cell disease (SCD). SCD patients had a higher incidence of COVID- 19 than those with TDT/NTDT (10.5 vs. 4.8 COVID/100 patients). Notably, 92% of the cases were mild, with neither age nor the specific RBCD affecting severity. The number of comorbidities, notably obesity and hypertension, that patients had prior to infection was associated with more severe COVID- 19. During the infection, the presence of vaso-occlusive crises, acute chest syndrome, kidney failure, and ground-glass opacities on chest tomography scans were associated with a more severe clinical picture. The vaccination rate (32%) mirrored that of the general population and showed a protective effect against severe COVID- 19. The observed mortality rate was 0.7%, aligning with Europe's general population. Conclusion: SARS-CoV- 2 infection in SCD and TDT/NTDT patients is mild and without higher mortality than the general population. The ERN-Eurobloodnet's registry collaborative structure exemplifies the power of international cooperation in tackling rare diseases, especially during health emergencies.
- Innovations in Cancer Nursing Education Across EuropePublication . McInally, W; Taylor, V; Diez de los Rios, C; Sulosaari, V; Dowling, M; Trigoso, E; Rodrigues Gomes, SM; Cesario Dias Ycn, AR; Piskorjanac, S; Tanay, MA; Hálfdánardóttir, H
- Enablers, Barriers and Strategies to Build Resilience Among Cancer Survivors: a Qualitative Study ProtocolPublication . Fernandes, JB; Domingos, J; Almeida, AS; Castro, C; Simões, A; Fernandes, S; Vareta, D; Bernardes, C; Fonseca, J; Vaz, C; Dias, AR; Fernandes, T; Godinho, CCancer is a life-threatening illness affecting all dimensions of a person's health. Cancer survivors must build resilience to face this adversity and continue their life projects. The present study explores the enablers, barriers, and strategies to build resilience among cancer survivors. This qualitative, descriptive exploratory study will use purposive sampling to recruit cancer survivors and healthcare professionals from two hospital centers in Lisbon and Tagus Valley. Interviews will be conducted until data saturation occurs. Data analysis will be performed using an inductive content analysis process with the help of the QDA Miner Lite database. The findings from this study will generate knowledge that may help stakeholders to identify effective strategies to build resilience among cancer survivors. By implementing strategies to foster resilience, healthcare professionals can potentially promote positive adaptations to cancer by strengthening resilience enablers and reducing the impact of barriers.
- Oral Azacitidine Maintenance Therapy for Acute Myeloid Leukemia in First RemissionPublication . Wei, A; Döhner, H; Pocock, C; Montesinos, P; Afanasyev, B; Dombret, H; Ravandi, F; Sayar, H; Jang, JH; Porkka, K; Selleslag, D; Sandhu, I; Turgut, M; Giai, V; Ofran, Y; Kizil Çakar, M; Botelho de Sousa, A; Rybka, J; Frairia, C; Borin, L; Beltrami, G; Čermák, J; Ossenkoppele, G; La Torre, I; Skikne, B; Kumar, K; Dong, Q; Beach, C; Roboz, GBackground: Although induction chemotherapy results in remission in many older patients with acute myeloid leukemia (AML), relapse is common and overall survival is poor. Methods: We conducted a phase 3, randomized, double-blind, placebo-controlled trial of the oral formulation of azacitidine (CC-486, a hypomethylating agent that is not bioequivalent to injectable azacitidine), as maintenance therapy in patients with AML who were in first remission after intensive chemotherapy. Patients who were 55 years of age or older, were in complete remission with or without complete blood count recovery, and were not candidates for hematopoietic stem-cell transplantation were randomly assigned to receive CC-486 (300 mg) or placebo once daily for 14 days per 28-day cycle. The primary end point was overall survival. Secondary end points included relapse-free survival and health-related quality of life. Results: A total of 472 patients underwent randomization; 238 were assigned to the CC-486 group and 234 were assigned to the placebo group. The median age was 68 years (range, 55 to 86). Median overall survival from the time of randomization was significantly longer with CC-486 than with placebo (24.7 months and 14.8 months, respectively; P<0.001). Median relapse-free survival was also significantly longer with CC-486 than with placebo (10.2 months and 4.8 months, respectively; P<0.001). Benefits of CC-486 with respect to overall and relapse-free survival were shown in most subgroups defined according to baseline characteristics. The most common adverse events in both groups were grade 1 or 2 gastrointestinal events. Common grade 3 or 4 adverse events were neutropenia (in 41% of patients in the CC-486 group and 24% of patients in the placebo group) and thrombocytopenia (in 22% and 21%, respectively). Overall health-related quality of life was preserved during CC-486 treatment. Conclusions: CC-486 maintenance therapy was associated with significantly longer overall and relapse-free survival than placebo among older patients with AML who were in remission after chemotherapy. Side effects were mainly gastrointestinal symptoms and neutropenia. Quality-of-life measures were maintained throughout treatment. (Supported by Celgene; QUAZAR AML-001 ClinicalTrials.gov number, NCT01757535.).
- Diffuse Large B-Cell Lymphoma with Axillary Cutaneous Invasion in a HIV Positive PatientPublication . Dias, A; Saraiva, F; Maltez, F
- A Double Philadelphia Chromosome-Positive Chronic Myeloid Leukemia Patient, Co-Expressing P210 BCR-ABL1 and P195 BCR-ABL1 IsoformsPublication . Vinhas, R; Lourenço, A; Santos, S; Ribeiro, P; Silva, M; Botelho de Sousa, A; Baptista, P; Fernandes, A
- Screening a Targeted Panel of Genes by Next-Generation Sequencing Improves Risk Stratification in Real World Patients with Acute Myeloid LeukemiaPublication . Matos, S; Bernardo, P; Esteves, S; Botelho de Sousa, A; Lemos, M; Ribeiro, P; Silva, M; Nunes, A; Lobato, J; Frade, MJ; Gomes da Silva, M; Chacim, S; Mariz, J; Esteves, G; Raposo, J; Espadana, A; Carda, J; Barbosa, P; Martins, V; Carmo-Fonseca, M; Desterro, JAlthough mutation profiling of defined genes is recommended for classification of acute myeloid leukemia (AML) patients, screening of targeted gene panels using next-generation sequencing (NGS) is not always routinely used as standard of care. The objective of this study was to prospectively assess whether extended molecular monitoring using NGS adds clinical value for risk assessment in real-world AML patients. We analyzed a cohort of 268 newly diagnosed AML patients. We compared the prognostic stratification of our study population according to the European LeukemiaNet recommendations, before and after the incorporation of the extended mutational profile information obtained by NGS. Without access to NGS data, 63 patients (23%) failed to be stratified into risk groups. After NGS data, only 27 patients (10%) failed risk stratification. Another 33 patients were re-classified as adverse-risk patients once the NGS data was incorporated. In total, access to NGS data refined risk assessment for 62 patients (23%). We further compared clinical outcomes with prognostic stratification, and observed unexpected outcomes associated with FLT3 mutations. In conclusion, this study demonstrates the prognostic utility of screening AML patients for multiple gene mutations by NGS and underscores the need for further studies to refine the current risk classification criteria.
- Bone Marrow Granulomatosis in Acute Q FeverPublication . Azevedo Carvalho, J; Pereira, S; Boavida, L; Gião, N; Bastos Furtado, AReported cases of Q fever in people living in urban areas after occasional contact with farm animals or infected pets such as dogs and cats have been increasing. The diagnosis of Q fever is usually laborious due to unspecific and variable clinical manifestations. The most common clinical presentation is an influenza-like illness with varying degrees of pneumonia and hepatitis. Acute hepatitis is more frequent than pneumonia in countries where the disease is endemic, such as in Portugal. We report a case of acute Q fever with hepatic and bone marrow involvement presented as fever of unknown origin (FUO) in a 56-year-old sportive hunter man. Typical fibrin ring granulomas (doughnut granulomas) were found in the bone marrow biopsy and were essential for the diagnosis. Bone marrow involvement is considered a rare manifestation of Q fever. Coxiella infection activates a granulomatous inflammatory response that can lead to persistent immune cell activation. Doughnut granulomas are not pathognomonic but they are highly specific for the diagnosis of Q fever.
- POEMS Syndrome: a Rare Cause of Adrenal Insufficiency in a Young MalePublication . Prokop, J; Estorninho, J; Marote, S; Sabino, T; Botelho de Sousa, A; Silva, E; Agapito, ASummary: POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein and Skin changes) is a rare multisystemic disease. Clinical presentation is variable, the only mandatory criteria being polyneuropathy and monoclonal gammapathy in association with one major and one minor criterion. Primary adrenal insufficiency is rarely reported. We describe a case of a 33-year-old patient, in whom the presenting symptoms were mandibular mass, chronic sensory-motor peripheral polyneuropathy and adrenal insufficiency. The laboratory evaluation revealed thrombocytosis, severe hyperkalemia with normal renal function, normal protein electrophoresis and negative serum immunofixation for monoclonal protein. Endocrinologic laboratory work-up confirmed Addison's disease and revealed subclinical primary hypothyroidism. Thoracic abdominal CT showed hepatosplenomegaly, multiple sclerotic lesions in thoracic vertebra and ribs. The histopathologic examination of the mandibular mass was nondiagnostic. Bone marrow biopsy revealed plasma cell dyscrasia and confirmed POEMS syndrome. Axillary lymphadenopathy biopsy: Castleman's disease. Gluco-mineralocorticoid substitution and levothyroxine therapy were started with clinical improvement. Autologous hematopoietic cell transplantation (HCT) was planned, cyclophosphamide induction was started. Meanwhile the patient suffered two ischemic strokes which resulted in aphasia and hemiparesis. Cerebral angiography revealed vascular lesions compatible with vasculitis and stenosis of two cerebral arteries. The patient deceased 14 months after the diagnosis. The young age at presentation, multiplicity of manifestations and difficulties in investigation along with the absence of serum monoclonal protein made the diagnosis challenging. We report this case to highlight the need to consider POEMS syndrome in differential diagnosis of peripheral neuropathy in association with endocrine abnormalities even in young patients. Learning points: POEMS syndrome is considered a 'low tumor burden disease' and the monoclonal protein in 15% of cases is not found by immunofixation. Neuropathy is the dominant characteristic of POEMS syndrome and it is peripheral, ascending, symmetric and affecting both sensation and motor function. Endocrinopathies are a frequent feature of POEMS syndrome, but the cause is unknown. The most common endocrinopathies are hypogonadism, primary hypothyroidism and abnormalities in glucose metabolism. There is no standard therapy; however, patients with disseminated bone marrow involvement are treated with chemotherapy with or without HCT.
- New Prospects for the Management of Cardiovascular Effects of Tyrosine Kinase Inhibitors in Patients with Chronic Myeloid LeukemiaPublication . Almeida, A; Almeida, A; Melo, T; Guerra, L; Lopes, L; Ribeiro, P; Duarte, M; Mota, A; Fontes-Carvalho, RThe use of tyrosine kinase inhibitors (TKIs) for the treatment of chronic myeloid leukemia has significantly altered the prognosis of this disease, enabling close to normal life expectancy. Despite their undeniable benefits, the use of TKIs is associated with an increased risk of side effects on the cardiovascular system, particularly of atherothrombotic events. It is therefore necessary to understand and prevent the adverse effects of these drugs, in order to enable antileukemic therapy to continue and to minimize patients' toxic exposure. This multidisciplinary consensus document, developed through a collaboration between hematologists and cardiologists, aims to review the cardiovascular toxicity associated with various TKIs and to establish recommendations for the follow-up of these patients. Measures are also proposed for the assessment and reduction of cardiovascular risk in these patients and referral criteria, and relevant drug interactions are discussed.
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