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1
Luspatercept: A New Kid on the Block
Dergi Makalesi

Dergi Makalesi
Kaynak :
Taylor and Francis
Dergi:
Hemoglobin
Yayıncı:
Informa UK Limited
Yazar(lar) :
Antonio Piga , Filomena Longo
Konu :
Genetics(clinical), Clinical Biochemistry, Hematology, Biochemistry, medical
Yıl :
2019
Filomena Longo
Yayıncı Web Sitesi
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DOI : 10.1080/03630269.2020.1719592
Anahtar Kelimeler : Erythropoiesis, luspatercept, thalassemia
ISSN: 0363-0269
Sayı: 6
Cilt: 43
Sayfa: 328-328
2
Atrial fibrillation in β-thalassemia Major Patients: Diagnosis, Management and Therapeutic Options.
Dergi Makalesi

Dergi Makalesi
Open Access
Kaynak 1:
PubMed
Kaynak 2:
Taylor and Francis
Dergi:
Hemoglobin
Yazar(lar) :
Umberto Barbero , Federico Fornari , Silvia Guarguagli , Carmen Maria Gaglioti , Filomena Longo , Baldassarre Doronzo , Matteo Anselmino , Antonio Piga
Yıl :
2018
Filomena Longo
Piri Tam Metin Bulucu
Kaydet Alıntıla
The prevalence of atrial fibrillation (AFib) in β-thalassemia major (β-TM) patients has increased in the last few years, reaching up to 33.0%. Several factors may drive this value to even more in the next few years. We summarized the main challenges in the management and therapy of AFib in this very specific group of patients.
DOI : 10.1080/03630269.2018.1488724
Anahtar Kelimeler : Arrhythmia, atrial fibrillation (AFib), iron overload, β-thalassemia (β-thal)
Sınıflandırma :
3
Late Effects of Bone Marrow Transplantation for Thalassemiaa
Dergi Makalesi

Dergi Makalesi
Kaynak :
Wiley Online Library
Dergi:
Annals of the New York Academy of Sciences
Yazar(lar) :
ANTONIO PIGA , FILOMENA LONGO , VINCENZO VOI , SILVIA FACELLO , ROBERTO MINIERO , BERND DRESOW
Konu :
General Biochemistry, Genetics and Molecular Biology, History and Philosophy of Science
Yıl :
1998
FILOMENA LONGO
Yayıncı Web Sitesi
Tam Metin
Kaydet Alıntıla
Abstract: Long term effects of BMT in thalassemia were monitored in 33 patients transplanted between 1987 and 1995 and compared with 155 patients matched for age and treated during the same period with conventional therapy (CT). The incidence of fulminant sepsis and growth impairment was significantly higher in transplanted patients, whereas the occurrence of hypothyroidism, hypogonadism, and cardiopathy was higher in CT patients. For diabetes, liver disease, and severe infections, the differences were not statistically significant. After BMT we performed monthly erythrocytaferesis for iron removal in 23 (70%) patients, obtaining a complete normalization of iron stores in 91% of cases; among untreated patients, 60% had evidence of iron up to 8.3 years after BMT. Protection against poliovirus, tetanus, diphtheria, and hepatitis B has been lost in 74%, 47%, 78%, and 44%, respectively. After BMT a careful follow‐up is needed to monitor and treat late transplant‐related and thalassemia‐related complications.
DOI : 10.1111/j.1749-6632.1998.tb10486.x
ISSN: 0077-8923
Sayı: 1 COOLEY'S ANEM
Cilt: 850
Sayfa: 294-299
4
Deferiprone
Dergi Makalesi

Dergi Makalesi
Kaynak :
Wiley Online Library
Dergi:
Annals of the New York Academy of Sciences
Yazar(lar) :
Antonio Piga , Simona Roggero , Ilaria Salussolia , Davide Massano , Melania Serra , Filomena Longo
Konu :
General Biochemistry, Genetics and Molecular Biology, History and Philosophy of Science
Yıl :
2010
Filomena Longo
Yayıncı Web Sitesi
Tam Metin
Kaydet Alıntıla
Deferiprone (DFP) has been evaluated in a wide range of disorders, but most data come from transfusion‐dependent thalassemia. The safety and tolerability profile includes gastrointestinal complaints, liver enzymes elevation, weight gain, arthropathy, neutropenia, and agranulocytosis. The last requires close monitoring of blood count and precludes the use of DFP in conditions with bone marrow abnormalities. The efficacy profile is similar among the three available chelators. For DFP, the choice of dosage is crucial to optimize the effect on liver iron concentration, according to the iron load degree and transfusional iron input. Growing evidence indicates that DFP, alone or in combination with deferoxamine, is effective in removing cardiac iron and preventing cardiac iron load. The available data consolidate an important role of DFP in the management of iron overload. There is a need to compare directly the relative value of the available chelators in the long‐term prevention of iron toxicity by well‐designed randomized controlled trials.
DOI : 10.1111/j.1749-6632.2010.05586.x
ISSN: 0077-8923
Sayı: 1
Cilt: 1202
Sayfa: 75-78
5
Severe iron overload in Blackfan-Diamond anemia: A case-control study
Dergi Makalesi

Dergi Makalesi
Kaynak :
Wiley Online Library
Dergi:
American Journal of Hematology
Yazar(lar) :
Simona Roggero , Paola Quarello , Tiziana Vinciguerra , Filomena Longo , Antonio Piga , Ugo Ramenghi
Konu :
Hematology
Yıl :
2009
Filomena Longo
Yayıncı Web Sitesi
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Kaydet Alıntıla
Chronic iron overload is a serious complication in transfusion‐dependent patients. Few studies have addressed this issue in Diamond‐Blackfan anemia (DBA). We describe a retrospective analysis of iron overload, and its related complications in 31 transfusion‐dependent Italian DBA patients whose records included one or more evaluation of liver iron concentration (LIC) by means of noninvasive magnetic liver susceptometry with a superconductive quantum interference device (SQUID). This cohort is also matched with a group of transfusion‐dependent β‐thalassemia major patients to look for differences. A severe iron overload was observed in 54% patients, especially among those inadequately chelated. The DBA patients displayed a significantly higher LIC than the regularly chelated β‐thalassemics. This difference may have been attributable to nonoptimal chelation (late onset, type, dose, prescription, and compliance), or an unknown biological mechanism that lead to an early severe iron overload. We therefore suggest that all transfusion patients should have an accurate record of their iron intake, a regular monitoring of iron overload, in order to start chelation when a critical transfusion load is reached, and to test the efficacy/compliance of chelation treatment. Physicians taking care of transfusion‐dependent DBA patients must be concerned about the frequent and early complications such as cardiac toxicity. Am. J. Hematol., 2009. © 2009 Wiley‐Liss, Inc.
DOI : 10.1002/ajh.21541
ISSN: 0361-8609
Sayı: 11
Cilt: 84
Sayfa: 729-732
6
Role of CYP1A1, ABCG2, CYP24A1 and VDR gene polymorphisms on the evaluation of cardiac iron overload in thalassaemia patients.
Dergi Makalesi

Dergi Makalesi
Open Access
Kaynak :
PubMed
Dergi:
Pharmacogenet. Genomics
Yazar(lar) :
Sarah Allegra , Jessica Cusato , Silvia De Francia , Filomena Longo , Elisa Pirro , Davide Massano , Valeria Avataneo , Amedeo De Nicolò , Antonio Piga , Antonio D'Avolio
Yıl :
2018
Filomena Longo
Yayıncı Web Sitesi
 Piri Tam Metin Bulucu
Kaydet Alıntıla
Iron-burden-induced arrhythmia and heart failure are among the leading causes of morbidity and mortality in β-thalassaemia major patients. T2* cardiac magnetic resonance remains the only reliable noninvasive method for the heart iron excess assessment. We explored the role of single nucleotide polymorphisms involved in vitamin D metabolism, transport and activity and in deferasirox (DFX) metabolism on cardiac iron burden.
DOI : 10.1097/FPC.0000000000000348
Sınıflandırma :
7
Monitoring long-term efficacy of iron chelation therapy by deferiprone and desferrioxamine in patients with β-thalassaemia major: application of SQUID biomagnetic liver susceptometry
Dergi Makalesi

Dergi Makalesi
Kaynak :
Wiley Online Library
Dergi:
British Journal of Haematology
Yazar(lar) :
Roland Fischer , Filomena Longo , Peter Nielsen , Rainer Engelhardt , Robert C. Hider , Antonio Piga
Konu :
Hematology
Yıl :
2003
Filomena Longo
Yayıncı Web Sitesi
Tam Metin
Kaydet Alıntıla
Summary. In this non‐randomized prospective study, liver and spleen iron concentrations were monitored annually over a 4‐year period by non‐invasive Superconducting Quantum Interference Device biomagnetometry in 54 β‐thalassaemia major patients (age, 7–22 years) receiving treatment with deferiprone (75 mg/kg/d). Median liver iron concentrations increased significantly from 1456 to 2029 and 2449 µg/gliver at baseline, after 2·0 and 3·2 years respectively. Another group of 51 thalassaemic patients (aged 4–34 years) who received desferrioxamine s.c. for 1·9 years increased their liver iron concentration from 1076 to 1260 µg/gliver. Taking into account the increase of the daily iron input from transfusions of 3·6 mg/d, caused by weight gain in 67% of the patients treated with deferiprone, a larger total body iron elimination rate was achieved after 2 years than at baseline. A negative ferritin change was observed in 51% of the patients. In 15 non‐splenectomized patients, liver iron significantly increased from 1260 to 1937 µg/gliver (P < 0·01), but serum ferritin remained stable at 2100 µg/l, as did the spleen iron concentration at 1200 µg/gspleen. A two‐compartment model may predict an average chelation efficacy for desferrioxamine and deferiprone, with a saturation effect of the latter, for a certain chelation and transfusion regimen by a single liver iron quantification.
DOI : 10.1046/j.1365-2141.2003.04297.x
ISSN: 0007-1048
Sayı: 6
Cilt: 121
Sayfa: 938-948
8
C29G in the iron-responsive element of l-ferritin: a new mutation associated with hyperferritinemia-cataract
Dergi Makalesi

Dergi Makalesi
Kaynak :
Science Direct
Dergi:
Blood Cells, Molecules, and Diseases
Yayıncı:
Elsevier
Yazar(lar) :
Sandra Bosio , Alessandro Campanella , Enrico Gramaglia , Paolo Porporato , Filomena Longo , Laura Cremonesi , Sonia Levi , Clara Camaschella
Yıl :
2004
Filomena Longo
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Hyperferritinemia-cataract syndrome (HHCS) is a dominant disorder characterized by high serum ferritin and early onset of bilateral cataract. The disorder is caused by mutations in the iron-responsive element (IRE) of l-ferritin, which disrupt the postranscriptional control of l-ferritin synthesis. Here, we report a new (C>G) mutation which affects base 29 in the loop (c.-169C>G), previously unrecognized as essential for the stem loop stability. The mutation was identified in two members of an Italian family. Computer modeling and electrophoretic mobility shift assay (EMSA) confirm a decreased affinity of the C29G IRE for IRPs control proteins.
DOI : 10.1016/j.bcmd.2004.04.010
Anahtar Kelimeler : Hyperferritinemia-cataract syndrome, l-Ferritin synthesis, c.-169C>G
ISSN: 1079-9796
Sayı: 1
Cilt: 33
Sayfa: 31-34
Sınıflandırma :
9
Changing patterns of splenectomy in transfusion-dependent thalassemia patients
Dergi Makalesi

Dergi Makalesi
Kaynak :
Wiley Online Library
Dergi:
American Journal of Hematology
Yazar(lar) :
Antonio Piga , Melania Serra , Filomena Longo , Gianluca Forni , Giovanni Quarta , Maria D. Cappellini , Renzo Galanello
Konu :
Hematology
Yıl :
2011
Filomena Longo
Yayıncı Web Sitesi
Tam Metin
Kaydet Alıntıla
DOI : 10.1002/ajh.22102
ISSN: 0361-8609
Sayı: 9
Cilt: 86
Sayfa: 808-810
10
Prevalence and prognostic impact of left ventricular non-compaction in patients with thalassemia
Dergi Makalesi

Dergi Makalesi
Kaynak :
Springer Link
Dergi:
Internal and Emergency Medicine
Yazar(lar) :
Rodolfo Bonamini , Massimo Imazio , Riccardo Faletti , Marco Gatti , Borejda Xhyheri , Marco Limone , Filomena Longo , Antonio Piga
Konu :
Emergency Medicine, Internal Medicine
Yıl :
2019
Filomena Longo
Yayıncı Web Sitesi
Tam Metin
Kaydet Alıntıla
A high incidence of isolated left ventricular non-compaction (LVNC) has been reported in previous studies on smaller cohorts of patients with thalassemia by cardiac MRI but the clinical impact of the finding is unknown. This prospective cohort study evaluates the prevalence and clinical implication of the finding. Prospective cohort study with enrollment of all consecutive cases with thalassemia referred for cardiac MRI from September 2007 to November 2014. The presence of LVNC was assessed according to the Petersen method and the Jacquier method, with the proposed changes by Fazio, Grothoff, and Chiodi. A clinical follow-up was performed in all patients. We included 560 patients with thalassemia (473 with thalassemia major and 87 with thalassemia intermedia: mean age 31.9 ± 10.6 years, male/female = 250/310). A total number of 1683 MRI tests were performed. A diagnosis of LVNC was determined according to adopted MR criteria in 44 patients (7.9%). Patients with LVNC had a significantly lower ejection fraction (52.68 ± 5.17% vs. 56.90 ± 6.34%; p = 0.0005) and greater indexed LV ESV (48.16 ± 10.03 ml/m2 vs. 40.02 ± 10.06 ml/m2; p = 0.0022). After a mean follow-up time was 5.1 years, no significant change of MR parameters was detected as well as no clinical adverse events. LVNC is relatively frequent in patients with thalassemia. However, it is not associated with a worsening of LV function and adverse events after a long-term follow-up.
DOI : 10.1007/s11739-019-02114-3
Anahtar Kelimeler : Thalassemia, Left ventricular non-compaction, Cardiac magnetic resonance, Prognosis
ISSN: 1828-0447 1970-9366
Sayı: 8
Cilt: 14
Sayfa: 1299-1306
11
Left ventricular noncompaction in patients with β-thalassemia: Uncovering a previously unrecognized abnormality
Dergi Makalesi

Dergi Makalesi
Kaynak :
Wiley Online Library
Dergi:
American Journal of Hematology
Yazar(lar) :
Antonio Piga , Filomena Longo , Khaled M. Musallam , Andrea Veltri , Francesca Ferroni , Amedeo Chiribiri , Rodolfo Bonamini
Konu :
Hematology
Yıl :
2012
Filomena Longo
Yayıncı Web Sitesi
Tam Metin
Kaydet Alıntıla
Left ventricular noncompaction (LVNC) is a rare cardiomyopathy with potentially serious outcomes. It results in multiple and excessive trabeculations, deep intertrabecular recesses, and a thickened ventricular myocardium with two distinct layers, compacted and noncompacted. The condition is most commonly congenital; however, acquired forms have also been described. A recent report of LVNC detected in a β‐thalassemia twin suggested an association with cardiac siderosis. In a cross‐sectional study of 135 transfusion‐dependent patients with β‐thalassemia (130 major and 5 intermedia, mean age 29.6 ± 7.7 years, 49.6% males) presenting for cardiac iron assessment by magnetic resonance imaging (MRI), we evaluated the prevalence and risk factors for LVNC. None of the patients had neuromuscular or congenital heart disease. Eighteen patients (13.3%; 95% confidence interval [CI] = 8.6–20.1) fulfilled the preassigned strict criteria for LVNC on cardiac MRI. There were no statistically significant differences between patients with and without LVNC with respect to demographics; hemoglobin levels; splenectomy status; systemic, hepatic, and cardiac iron overload indices; hepatic disease and infection studies; or iron chelator type. Patients with LVNC were more likely to have heart failure (adjusted odds ratio = 1.77; 95% CI = 0.29–10.89); although with high uncertainty. Patients with β‐thalassemia have a higher prevalence of LVNC than normal individuals. As this finding could not be explained by conventional risk factors in this patient population, further investigation of the underlying mechanisms of LVNC is warranted. This remains crucial for an entity with adverse cardiac outcomes, especially in patients with β‐thalassemia where cardiac disease remains a primary cause of mortality. Am. J. Hematol., 2012. © 2012 Wiley Periodicals, Inc.
DOI : 10.1002/ajh.23323
ISSN: 0361-8609
Sayı: 12
Cilt: 87
Sayfa: 1079-1083
Sınıflandırma :
12
High nontransferrin bound iron levels and heart disease in thalassemia major
Dergi Makalesi

Dergi Makalesi
Kaynak :
Wiley Online Library
Dergi:
American Journal of Hematology
Yazar(lar) :
Antonio Piga , Filomena Longo , Lorena Duca , Simona Roggero , Tiziana Vinciguerra , Roberto Calabrese , Chaim Hershko , Maria Domenica Cappellini
Konu :
Hematology
Yıl :
2009
Filomena Longo
Yayıncı Web Sitesi
Tam Metin
Kaydet Alıntıla
Although the presence of nontransferrin bound plasma iron (NTBI) in transfusional iron overload is well documented, knowledge about its clinical significance is limited. We assessed NTBI levels in a large and homogeneous series of thalassemia patients on regular transfusion and chelation and explored the hypothesis that NTBI levels may be associated with relevant clinical outcomes: in particular, heart disease. Among 174 patients with thalassemia major and intermedia, we showed the presence NTBI in 145 of 174 or 83.3% of cases. NTBI levels correlated with transferrin saturation, age, and ALT, and not with serum ferritin or liver iron concentrations. At a multiple regression analysis, transferrin saturation and heart disease but not age was independent predictors of NTBI. Patients with heart disease had NTBI levels significantly higher than those without. All patients with heart disease had transferrin saturation above 70%, and all were NTBI positive. Conversely, none of the patients without NTBI and/or with transferrin saturation less than 70% had preclinical or clinical heart disease. To our knowledge, this is the first documentation of a link between the presence of NTBI in thalassemic patients with transfusional iron overload and heart disease. Further investigation from these preliminary findings may clarify whether NTBI assessment may have a role in evaluating the risks and optimizing treatment for transfusion‐dependent patients. Am. J. Hematol., 2009. © 2008 Wiley‐Liss, Inc.
DOI : 10.1002/ajh.21317
ISSN: 0361-8609
Sayı: 1
Cilt: 84
Sayfa: 29-33
13
Deferasirox for cardiac siderosis in β-thalassaemia major: a multicentre, open label, prospective study
Dergi Makalesi

Dergi Makalesi
Kaynak :
Wiley Online Library
Dergi:
British Journal of Haematology
Yazar(lar) :
Antonio Piga , Filomena Longo , Raffaella Origa , Simona Roggero , Francesca Pinna , Antonietta Zappu , Chiara Castiglioni , Maria Domenica Cappellini
Konu :
Hematology
Yıl :
2014
Filomena Longo
Yayıncı Web Sitesi
Tam Metin
Kaydet Alıntıla
DOI : 10.1111/bjh.12987
ISSN: 0007-1048
Sayı: 3
Cilt: 167
Sayfa: 423-426
14
Deferasirox pharmacokinetics evaluation in a woman with hereditary haemochromatosis and heterozygous β-thalassaemia
Dergi Makalesi

Dergi Makalesi
Kaynak :
Science Direct
Dergi:
Biomedicine & Pharmacotherapy
Yayıncı:
Elsevier
Yazar(lar) :
Sarah Allegra , Silvia De Francia , Filomena Longo , Davide Massano , Jessica Cusato , Arianna Arduino , Elisa Pirro , Antonio Piga , Antonio D'Avolio
Yıl :
2016
Filomena Longo
Yayıncı Web Sitesi
Tam Metin
Kaydet Alıntıla
We present the deferasirox pharmacokinetics evaluation of a female patient on iron chelation, for the interesting findings from her genetic background (hereditary haemochromatosis and heterozygous β-thalassaemia) and clinical history (ileostomy; iron overload from transfusions). Drug plasma concentrations were measured by an HPLC-UV validated method, before and after ileum resection. Area under deferasirox concentration curve over 24 h (AUC) values were determined by the mixed log-linear rule, using Kinetica software. AUC was low also with high deferasirox dose as well as tolerability. Non invasive tissue iron quantification by magnetic resonance imaging or superconducting quantum interference device were prevented by a metal hip replacement. Good efficacy and normalisation of iron markers was obtained on long term. Therapeutic drug monitoring in patient in critical conditions may help to understand reasons for non response and set individualised treatment.
DOI : 10.1016/j.biopha.2016.11.041
Anahtar Kelimeler : Deferasirox, Dfx, Iron overload, Iron chelation, Haemochromatosis, Ileostomy
ISSN: 0753-3322
Cilt: 84
Sayfa: 1510-1512
15
Risk factors for heart disease in transfusion-dependent thalassemia: serum ferritin revisited.
Dergi Makalesi

Dergi Makalesi
Open Access
Kaynak :
PubMed
Dergi:
Intern Emerg Med
Yazar(lar) :
Giorgio Derchi , Carlo Dessì , Patrizio Bina , Maria Domenica Cappellini , Antonio Piga , Silverio Perrotta , Immacolata Tartaglione , Marianna Giuditta , Filomena Longo , Raffaella Origa , Antonella Quarta , Valeria Pinto , Gian Luca Forni
Yıl :
2018
Filomena Longo
Yayıncı Web Sitesi
 Piri Tam Metin Bulucu
Kaydet Alıntıla
Heart disease remains a leading cause of morbidity and mortality in transfusion-dependent thalassemia (TDT), which can be attributed to several factors but primarily develops in the setting of iron overload. This was a retrospective cohort study utilizing Webthal® patient data from five major centers across Italy. Patients without heart disease were followed-up for 10 years (2000-2010) and data were collected for demographics, splenectomy status, serum ferritin and hemoglobin levels, and comorbidities associated with heart disease. Among 379 patients analyzed (mean age 22.9 ± 5.1 years, 47.8% men), 44 (cumulative incidence: 11.6%) developed heart disease during the period of observation. Splenectomy (p = 0.002) and serum ferritin level (p < 0.001) were the only risk factors with significant association with heart disease. A serum ferritin threshold of ≥ 3000 ng/mL was the best predictor for the development of heart disease (86.4% sensitivity and 92.8% specificity, AUC: 0.912, 95% CI 0.852-0.971, p < 0.001). On multivariate analysis, only a serum ferritin level ≥ 3000 ng/mL remained significantly and independently associated with increased risk of heart disease (HR: 44.85, 95% CI 18.85-106.74), with a 5- and 10-year heart disease-free survival of 58 and 39%. The association between iron overload and heart disease in patients with TDT is confirmed, yet a new serum ferritin level of 3000 ng/mL to flag increased risk is suggested.
DOI : 10.1007/s11739-018-1890-2
Anahtar Kelimeler : Heart disease, Iron overload, Serum ferritin, Thalassemia
16
Clinical relevance of deferasirox trough levels in β-thalassemia patients
Dergi Makalesi

Dergi Makalesi
Kaynak :
Wiley Online Library
Dergi:
Clinical and Experimental Pharmacology and Physiology
Yazar(lar) :
Sarah Allegra , Davide Massano , Silvia De Francia , Filomena Longo , Francesca Piccione , Elisa Pirro , Jessica Cusato , Antonio D'Avolio , Antonio Piga
Yıl :
2017
Filomena Longo
Yayıncı Web Sitesi
Tam Metin
Kaydet Alıntıla
We evaluated the role of deferasirox therapeutic drug monitoring in order to avoid toxicity or treatment failure. Plasma concentrations, measured between two consecutive liver iron determinations, were determined at the end of dosing interval. Fifty‐four β‐thalassemic adult patients were enrolled: 50% were males; median age was 32.3 years (IQR 19.1‐41.7 years) and median body mass index was 22.25 kg/m2 (IQR 20.24‐23.75 kg/m2). The mean deferasirox dose was 28.6 ± 6.3 mg/kg/d and mean plasma concentration was 17.3 ± 16.8 μg/mL. Drug levels showed lower results in males. Deferasirox concentration was significantly correlated with serum creatinine levels (P = .01) and serum ferritin (P < .0001). The assessment of deferasirox therapeutic drug monitoring could help clinicians to predict patient responses and to optimize the therapy.
DOI : 10.1111/1440-1681.12879
ISSN: 0305-1870
Sayı: 2
Cilt: 45
Sayfa: 213-216
17
Pharmacological and clinical evaluation of deferasirox formulations for treatment tailoring
Dergi Makalesi

Dergi Makalesi
Kaynak :
Springer Nature
Dergi:
Scientific Reports
Yayıncı:
Springer Science and Business Media LLC
Yazar(lar) :
Andrea Piolatto , Paola Berchialla , Sarah Allegra , Silvia De Francia , Giovanni Battista Ferrero , Antonio Piga , Filomena LongoAndrea Piolatto , Paola Berchialla , Sarah Allegra , Silvia De Francia , Giovanni Battista Ferrero , Antonio Piga , Filomena Longo
Yıl :
2021
Filomena Longo
Yayıncı Web Sitesi
Tam Metin
Kaydet Alıntıla
AbstractDeferasirox (DFX) is the newest among three different chelators available to treat iron overload in iron-loading anaemias, firstly released as Dispersible Tablets (DT) and more recently replaced by Film-Coated Tablets (FCT). In this retrospective observational study, pharmacokinetics, pharmacodynamics, and safety features of DFX treatment were analyzed in 74 patients that took both formulations subsequently under clinical practice conditions. Bioavailability of DFX FCT compared to DT resulted higher than expected [Cmax: 99.5 (FCT) and 69.7 (DT) μMol/L; AUC: 1278 (FCT) and 846 (DT), P < 0.0001]. DFX FCT was also superior in scalability among doses. After one year of treatment for each formulation, no differences were observed between the treatments in the overall iron overload levels; however, DFX FCT but not DT showed a significant dose–response correlation [Spearman r (dose-serum ferritin variation): − 0.54, P < 0.0001]. Despite being administered at different dosages, the long-term safety profile was not different between formulations: a significant increase in renal impairment risk was observed for both treatments and it was reversible under strict monitoring (P < 0.002). Altogether, these data constitute a comprehensive comparison of DFX formulations in thalassaemia and other iron-loading anaemias, confirming the effectiveness and safety characteristics of DFX and its applicability for treatment tailoring.
DOI : 10.1038/s41598-021-91983-w
ISSN: 2045-2322
Sayı: 1
Cilt: 11
Sınıflandırma :
18
Effectiveness of bibliographic searches performed by paediatric residents and interns assisted by librarians. A randomised controlled trial
Dergi Makalesi

Dergi Makalesi
Kaynak :
Wiley Online Library
Dergi:
Health Information & Libraries Journal
Yazar(lar) :
Paolo Gardois , Roberto Calabrese , Nicoletta Colombi , Annamaria Deplano , Carla Lingua , Filomena Longo , Maria C. Villanacci , Roberto Miniero , Antonio Piga
Konu :
Health Informatics, Health Information Management, Library and Information Sciences
Yıl :
2011
Filomena Longo
Yayıncı Web Sitesi
Tam Metin
Kaydet Alıntıla
Background: Considerable barriers still prevent paediatricians from successfully using information retrieval technology.
DOI : 10.1111/j.1471-1842.2011.00957.x
ISSN: 1471-1834
Sayı: 4
Cilt: 28
Sayfa: 273-284
19
Relative contribution of iron burden, HFE mutations, and insulin resistance to fibrosis in nonalcoholic fatty liver
Dergi Makalesi

Dergi Makalesi
Kaynak :
Wiley Online Library
Dergi:
Hepatology
Yazar(lar) :
Elisabetta Bugianesi , Paola Manzini , Sergio D'Antico , Ester Vanni , Filomena Longo , Nicola Leone , Paola Massarenti , Antonio Piga , Giulio Marchesini , Mario Rizzetto
Yıl :
2004
Filomena Longo
Yayıncı Web Sitesi
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Kaydet Alıntıla
The mechanism(s) determining the progression from fatty liver to steatohepatitis is currently unknown. Our goal was to define the relative impact of iron overload, genetic mutations of HFE, and insulin resistance on the severity of liver fibrosis in a population of subjects with nonalcoholic fatty liver disease (NAFLD) who had low prevalence of obesity and no overt symptoms of diabetes. In a cohort of 263 prospectively enrolled patients with NAFLD, 7.4% of patients had signs of peripheral iron overload and 9% had signs of hepatic iron overload, but 21.1% had hyperferritinemia. The prevalence of C282Y and H63D HFE mutations was similar to the general population and mutations were not associated with iron overload. Although subjects were on average only moderately overweight, insulin sensitivity, measured both in the fasting state and in response to oral glucose, was lower. Univariate analysis demonstrated that the presence of severe fibrosis was independently associated with older age, female sex, overweight, aspartate/alanine aminotransferase ratio, serum ferritin level, fasting glucose and insulin levels, decreased insulin sensitivity, and with histologic features (degree of necroinflammation and steatosis). After adjustment for body mass index (BMI), age, sex, and degree of steatosis, ferritin levels (odds ratio [OR] = 1.77; 95% CI = 1.21‐ 2.58; P = .0032) and the oral glucose insulin sensitivity (OR = 0.53; CI = 0.33‐0.87; P = .0113) were independent predictors of severe fibrosis. In conclusion, the current study indicates that insulin resistance is a major, independent risk factor for advanced fibrosis in patients with NAFLD. Increased ferritin levels are markers of severe histologic damage, but not of iron overload. Iron burden and HFE mutations do not contribute significantly to hepatic fibrosis in the majority of patients with NAFLD. (HEPATOLOGY 2004;39:179–187.)
DOI : 10.1002/hep.20023
ISSN: 0270-9139
Sayı: 1
Cilt: 39
Sayfa: 179-187
20
Iron Overload in Patients Receiving Allogeneic Hematopoietic Stem Cell Transplantation: Quantification of Iron Burden by a Superconducting Quantum Interference Device (SQUID) and Therapeutic Effectiveness of Phlebotomy
Dergi Makalesi

Dergi Makalesi
Kaynak :
Science Direct
Dergi:
Biology of Blood and Marrow Transplantation
Yayıncı:
Elsevier
Yazar(lar) :
Alessandro Busca , Michele Falda , Paola Manzini , Sergio D'Antico , Adriano Valfrè , Franco Locatelli , Roberto Calabrese , Annalisa Chiappella , Stefano D'Ardia , Filomena Longo , Antonio Piga
Yıl :
2010
Filomena Longo
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Kaydet Alıntıla
Iron overload (IO) is a known adverse prognostic factor in patients who undergo allogeneic hematopoietic stem cell transplantation (HSCT) for thalassemia and appears to play a similar role in patients with other hematologic disorders. The estimation of IO is based primarily on serum ferritin level; however, many confounding factors can result in ferritin overestimation, especially in HSCT recipients. The aim of the present study was to quantify IO after HSCT using a superconducting quantum interference device (SQUID), and to evaluate the impact of IO on hepatic function and infections. In addition, the feasibility of iron depletion was investigated. A total of 102 consecutive allogeneic HSCT recipients admitted to our outpatient department between December 2005, and December 2007, were analyzed. Primary diagnosis included acute leukemia/myelodysplastic syndrome in 61% of cases. Assessment of IO after HSCT included serum ferritin; in those with hyperferritinemia (ferritin > 1000 ng/mL), liver iron concentration (LIC) was evaluated by SQUID magnetic susceptometry. Iron removal therapy was offered to patients with moderate IO (LIC 1000-2000 μg Fe/g wet weight [ww]) or severe IO (LIC >2000 μg Fe/g ww). Fifty-seven patients had a ferritin level <1000 ng/mL: the median time between HSCT and assessment of ferritin level was 1006 days (range, 93-5239 days), significantly different from the median time of 183 days (range, 78-2957 days) in the 45 patients with a ferritin level >1000 ng/mL. Out of 42 patients evaluated by SQUID, 29 had moderate to severe IO (median LIC value, 1493 μg Fe/g ww [range, 1030-3253]). In a multivariate analysis, a significant correlation was found between a ferritin level >1000 ng/mL and the presence of at least one abnormal liver function test (LFT) ORo = 6.8; 95% CI = 2.2-20.6). In addition, the rate of proven/probable invasive fungal disease was significantly higher in the patients with hyperferritinemia (13% vs 0%; P = .006). Nineteen of the 24 patients considered eligible for iron-depletion therapy underwent regular phlebotomy; 13 completed the program in a median of 287 days (range, 92-779 days), reaching the target of a ferritin level < 500 ng/mL; LIC was significantly reduced (median, 1419 μg Fe/g ww to 625 μg Fe/g ww; P < .001) in 8 of the 9 patients who were revaluated by SQUID at the end of the iron-depletion program. In conclusion, the measurement of LIC obtained by SQUID documented the presence of moderate/severe IO in 69% of the patients with a high ferritin level. Our data showed that in HSCT recipients, high ferritin level is an independent risk factor for abnormal LFTs, and IO may be considered a potential risk factor for fungal infections. A phlebotomy program may be feasible in two-thirds of the patients who might benefit from iron depletion.
DOI : 10.1016/j.bbmt.2009.09.011
Anahtar Kelimeler : Allogeneic stem cell transplant, Iron overload, SQUID
ISSN: 1083-8791
Sayı: 1
Cilt: 16
Sayfa: 115-122
Sınıflandırma :
Hematoloji %100
21
Agreement of liver iron quantification measurements with low Tc-SQUID biosusceptometers in Oakland, Torino and Hamburg
Dergi Makalesi

Dergi Makalesi
Kaynak :
Science Direct
Dergi:
International Congress Series
Yayıncı:
Elsevier
Yazar(lar) :
Rainer Engelhardt , Ellen B. Fung , Filomena Longo , Marco Borri , Zahra Pakbaz , Herbert Opitz , Robert L. Fagaly , Antonio Piga , Roland Fischer , Paul Harmatz
Yıl :
2007
Filomena Longo
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. Accurate measurement of liver storage iron is important for the management of iron overload from regular red blood cell transfusion therapy. In clinical trials testing iron chelator efficacy in iron overloaded patients, with different centers involved, it is mandatory to standardize the procedures of liver iron concentration (LIC) measurements. The agreement (precision and bias) of the SQUID biosusceptometer in Oakland/USA (CHO) and Torino/Italy (TOR) was evaluated in comparison to the validated System in Hamburg/Germany (UKE). In a blinded round-robin test, liver iron concentrations were quantified within 1 month by the 3 SQUID biosusceptometer systems, in 20 patients with thalassemia, sickle cell disease or CDA type-1 and in 10 normal subjects. Mean LIC values were determined from 5 vertical scans at each of two, different positions. Prediction of LIC at CHO and TOR in comparison with UKE was excellent, with coefficients of determination between sites of R2 = 0.97, resulting in inter-site standard deviations of 276 and 315 μg/g liver, respectively. A significant proportional underestimation of 27% was noted for the TOR system. A systematically lower skin–liver distance of 1.0 mm at the TOR site could only account for 10% of the underestimated LIC. Intra-site precision was within an acceptable range for repeated measurements in the majority of iron overloaded subjects.
DOI : 10.1016/j.ics.2007.01.048
Anahtar Kelimeler : Biosusceptometry, SQUID, Liver, Iron
ISSN: 0531-5131
Cilt: 1300
Sayfa: 279-282
Sınıflandırma :
22
Iron-related MRI images in patients with pantothenate kinase-associated neurodegeneration (PKAN) treated with deferiprone: Results of a phase II pilot trial
Dergi Makalesi

Dergi Makalesi
Kaynak :
Wiley Online Library
Dergi:
Movement Disorders
Yazar(lar) :
Giovanna Zorzi , Federica Zibordi , Luisa Chiapparini , Enrico Bertini , Lidia Russo , Antonio Piga , Filomena Longo , Barbara Garavaglia , Domenico Aquino , Mario Savoiardo , Alessandra Solari , Nardo Nardocci
Yıl :
2011
Filomena Longo
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DOI : 10.1002/mds.23751
ISSN: 0885-3185
Sayı: 9
Cilt: 26
Sayfa: 1755-1759
23
Assessment and management of iron overload in β-thalassaemia major patients during the 21st century: a real-life experience from the Italian Webthal project
Dergi Makalesi

Dergi Makalesi
Kaynak :
Wiley Online Library
Dergi:
British Journal of Haematology
Yazar(lar) :
Antonio Piga , Filomena Longo , Khaled M. Musallam , Maria Domenica Cappellini , Gian Luca Forni , Giovanni Quarta , Francesco Chiavilli , Francesca Commendatore , Sergio Mulas , Vincenzo Caruso , Renzo Galanello
Konu :
Hematology
Yıl :
2013
Filomena Longo
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We conducted a cross‐sectional study on 924 β‐thalassaemia major patients (mean age 30·1 years) treated at nine Italian centres using the webthal software, to evaluate real‐life application of iron overload assessment and management standards. Serum ferritin <2500 ng/ml was a risk factor for never having liver iron concentration (LIC) measurement, while absence of cardiac disease and siderosis were risk factors for a delay in LIC measurement >2 years. Patients who never had a cardiac MRI (CMR) T2* measurement were <18 years, had iron intake ≤0·4 mg/kg per day, or a serum ferritin <2500 ng/ml. A history of normal CMR T2* was the main risk factor for a delay in subsequent assessment of >2 years. Deferoxamine (22·8%) was more commonly used in patients with Hepatitis C Virus or high serum creatinine. Deferiprone (20·6%) was less commonly prescribed in patients with elevated alanine aminotransferase; while a deferoxamine + deferiprone combination (17·9%) was more commonly used in patients with serum ferritin >2500 ng/ml or CMR T2* <20 ms. Deferasirox (38·3%) was more commonly prescribed in patients <18 years, but less commonly used in those with heart disease or high iron intake. These observations largely echoed guidelines at the time, although some practices are expected to change in light of evolving evidence.
DOI : 10.1111/bjh.12340
ISSN: 0007-1048
Sayı: 6
Cilt: 161
Sayfa: 872-883
24
Fontan circulation causes early, severe liver damage. Should we offer patients a tailored strategy?
Dergi Makalesi

Dergi Makalesi
Kaynak :
Science Direct
Dergi:
International Journal of Cardiology
Yayıncı:
Elsevier
Yazar(lar) :
Gabriella Agnoletti , Gaetana Ferraro , Roberto Bordese , Davide Marini , Simona Gala , Laura Bergamasco , Francesca Ferroni , Pier Luigi Calvo , Claudio Barletti , Fabio Cisarò , Filomena Longo , Carlo Pace Napoleone
Yıl :
2016
Filomena Longo
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Background In patients with Fontan circulation, the liver is profoundly affected by chronic venous stasis. Little is known about early hepatic changes in this population. Methods We performed echocardiography, abdominal ultrasound, liver elastography, cardiac catheterization, esophago-gastro-duodenoscopy and calculated MELD-XI score in 64 Fontan patients (69% minors), at an interval of 1–15 years since Fontan. Results Cardiac output remained stable in the first 5 years after Fontan, then significantly decreased (r = − 0.45, p(r = 0) = 0.003). NYHA class significantly increased after Fontan. Patients in NYHA class II/III (n = 21, 14 minors) had significantly higher hepatic pressures, but normal ventricular function and pulmonary vascular resistances (PVR). Patients with pulmonary arterial pressure (PAP) ≥ 15 mm Hg (n = 12, 6 minors) and those with PVR ≥ 2 WU*m2 (n = 27, 25 minors), had higher hepatic pressures (p < 0.0001), a higher incidence of liver collaterals and/or esophageal varices (p < 0.0001) and splenomegaly (p < 0.02). Liver stiffness (LS) was elevated in most patients (median, 25th–75th percentile:17.3 KPa, 14.1–21.4). It rapidly increased during the first 5-years after Fontan, compared to the following 5-years (from 12.2 KPa, 9.8–14.1 to 17.5 KPa, 14.3–24.5, p = 0.007), then remained stable (19.1 KPa, 16.9–22.6, p = 0.60). MELD-XI score increased linearly with the time interval since Fontan (r = 0.31, p(r = 0) = 0.01). For patients above 12 years we found a linear correlation between LS and MELD-XI score in the 6–15 years period after Fontan (r = 0.40. p(r = 0) = 0.04). The overall incidence of established liver cirrhosis was 22%. Conclusions This is the largest study showing that Fontan circulation prompts early, progressive and eventually irreversible liver damage. Precautions should be taken immediately after Fontan, to protect this fragile population.
DOI : 10.1016/j.ijcard.2016.02.041
Anahtar Kelimeler : Congenital heart disease, Fontan, Liver
ISSN: 0167-5273
Cilt: 209
Sayfa: 60-65
Sınıflandırma :
25
SARS‐CoV ‐2 infection in beta thalassemia: Preliminary data from the Italian experience
Dergi Makalesi

Dergi Makalesi
Kaynak :
Wiley Online Library
Dergi:
American Journal of Hematology
Yazar(lar) :
Irene Motta , Margherita Migone De Amicis , Valeria M. Pinto , Manuela Balocco , Filomena Longo , Federico Bonetti , Barbara Gianesin , Giovanna Graziadei , Maria D. Cappellini , Lucia De Franceschi , Antonio Piga , Gian L. Forni
Konu :
Hematology
Yıl :
2020
Filomena Longo
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DOI : 10.1002/ajh.25840
ISSN: 0361-8609
Sayı: 8
Cilt: 95

26
Transient elastography for non-invasive evaluation of post-transplant liver graft fibrosis in children
Dergi Makalesi

Dergi Makalesi
Kaynak :
Wiley Online Library
Dergi:
Pediatric Transplantation
Yazar(lar) :
Tiziana Vinciguerra , Andrea Brunati , Ezio David , Filomena Longo , Michele Pinon , Fulvio Ricceri , Luisa Castellino , Antonio Piga , Maria Teresa Giraudo , Francesco Tandoi , Fabio Cisarò , Dominic Dell Olio , Giuseppe Isolato , Renato Romagnoli , Mauro Salizzoni , Pier Luigi Calvo
Yıl :
2018
Filomena Longo
Yayıncı Web Sitesi
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Kaydet Alıntıla
As graft survival in pediatric LT is often affected by progressive fibrosis, numerous centers carry out protocol liver biopsies. Follow‐up biopsy protocols differ from center to center, but all biopsies are progressively spaced out, as time from transplant increases. Therefore, there is a need for non‐invasive techniques to evaluate graft fibrosis progression in those children who have no clinical or serological signs of liver damage. Indirect markers, such as the APRI, should be relied on with caution because their sensitivity in predicting fibrosis can be strongly influenced by the etiology of liver disease, severity of fibrosis, and patient age. A valid alternative could be TE, a non‐invasive technique already validated in adults, which estimates the stiffness of the cylindrical volume of liver tissue, 100‐fold the size of a standard needle biopsy sample. The aims of this study were to evaluate the reliability of TE in children after LT and to compare both the TE and the APRI index results with the histological scores of fibrosis on liver biopsies. A total of 36 pediatric LT recipients were studied. All patients underwent both TE and biopsy within a year (median interval ‐0.012 months) at an interval from LT of 0.36 to 19.47 years (median 3.02 years). Fibrosis was assessed on the biopsy specimens at histology and staged according to METAVIR. There was a statistically significant correlation between TE stiffness values and METAVIR scores (P = .005). The diagnostic accuracy of TE for the diagnosis of significant fibrosis (F ≥ 2) was measured as the area under the curve (AUROC = 0.865), and it demonstrated that the method had a good diagnostic performance. APRI was not so accurate in assessing graft fibrosis when compared to METAVIR (AUROC = 0.592). A liver stiffness cutoff value of 5.6 kPa at TE was identified as the best predictor for a significant graft fibrosis (METAVIR F ≥ 2) on liver biopsy, with a 75% sensitivity, a 95.8% specificity, a 90% positive predictive value, and an 88.5% negative predictive value. These data suggest that TE may represent a non‐invasive, reliable tool for the assessment of graft fibrosis in the follow‐up of LT children, alerting the clinicians to the indication for a liver biopsy, with the aim of reducing the number of protocol liver biopsies.
DOI : 10.1111/petr.13125
ISSN: 1397-3142
Sayı: 2
Cilt: 22
Sayfa: e13125
27
Epigenetic therapy for Friedreich ataxia
Dergi Makalesi

Dergi Makalesi
Kaynak :
Wiley Online Library
Dergi:
Annals of Neurology
Yazar(lar) :
Elisabetta Soragni , Wenyan Miao , Marco Iudicello , David Jacoby , Stefania De Mercanti , Marinella Clerico , Filomena Longo , Antonio Piga , Sherman Ku , Erica Campau , Jintang Du , Pablo Penalver , Myriam Rai , Joseph C. Madara , Kristopher Nazor , Melinda O'Connor , Anton Maximov , Jeanne F. Loring , Massimo Pandolfo , Luca Durelli , Joel M. Gottesfeld , James R. Rusche
Yıl :
2014
Filomena Longo
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DOI : 10.1002/ana.24260
ISSN: 0364-5134
Sayı: 4
Cilt: 76
Sayfa: 489-508
28
Hepatocellular carcinoma in thalassaemia: an update of the Italian Registry
Dergi Makalesi

Dergi Makalesi
Kaynak :
Wiley Online Library
Dergi:
British Journal of Haematology
Yazar(lar) :
Caterina Borgna-Pignatti , Maria Chiara Garani , Gian Luca Forni , Maria Domenica Cappellini , Elena Cassinerio , Carmelo Fidone , Vincenzo Spadola , Aurelio Maggio , Gaetano Restivo Pantalone , Antonio Piga , Filomena Longo , Maria Rita Gamberini , Paolo Ricchi , Silvia Costantini , Domenico D'Ascola , Paolo Cianciulli , Maria Eliana Lai , Maria Paola Carta , Angela Ciancio , Paola Cavalli , Maria Caterina Putti , Susanna Barella , Giovanni Amendola , Saveria Campisi , Marcello Capra , Vincenzo Caruso , Grazia Colletta , Stefano Volpato
Konu :
Hematology
Yıl :
2014
Filomena Longo
Yayıncı Web Sitesi
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The risk of developing hepatocellular carcinoma (HCC) in patients with thalassaemia is increased by transfusion‐transmitted infections and haemosiderosis. All Italian Thalassaemia Centres use an ad hoc form to report all diagnoses of HCC to the Italian Registry. Since our last report, in 2002, up to December 2012, 62 new cases were identified, 52% of whom were affected by thalassaemia major (TM) and 45% by thalassaemia intermedia (TI). Two had sickle‐thalassaemia (ST). The incidence of the tumour is increasing, possibly because of the longer survival of patients and consequent longer exposure to the noxious effects of the hepatotropic viruses and iron. Three patients were hepatitis B surface antigen‐positive, 36 patients showed evidence of past infection with hepatitis B virus (HBV). Fifty‐four patients had antibodies against hepatitis C virus (HCV), 43 of whom were HCV RNA positive. Only 4 had no evidence of exposure either to HCV or HBV. The mean liver iron concentration was 8 mg/g dry weight. Therapy included chemoembolization, thermoablation with radiofrequency and surgical excision. Three patients underwent liver transplant, 21 received palliative therapy. As of December 2012, 41 patients had died. The average survival time from HCC detection to death was 11·5 months (1·4–107·2 months). Ultrasonography is recommended every 6 months to enable early diagnosis of HCC, which is crucial to decrease mortality.
DOI : 10.1111/bjh.13009
ISSN: 0007-1048
Sayı: 1
Cilt: 167
Sayfa: 121-126
29
Thalassaemia is paradoxically associated with a reduced risk of in‐hospital complications and mortality in COVID‐19: Data from an international registry
Dergi Makalesi

Dergi Makalesi
Kaynak :
Wiley Online Library
Dergi:
Journal of Cellular and Molecular Medicine
Yazar(lar) :
Ibrahim Elu2010Battrawy , Filomena Longo , Iván J. Núñez Gil , Mohammad Abumayyaleh , Barbara Gianesin , Vicente Estrada , Álvaro Aparisi , Ramón Arroyou2010Espliguero , Manuela Balocco , Susanna Barella , Andrea Beccaria , Federico Bonetti , Maddalena Casale , Elisa De michele , Anna Rita Denotti , Carmelo Fidone , Monica Fortini , Maria Rita Gamberini , Giovanna Graziadei , Roberto Lisi , Antonella Massa , Alessia Marcon , Bryan Rubinski , Maurizio Miano , Irene Motta , Valeria Maria Pinto , Alberto Piperno , Raffaella Mariani , Maria Caterina Putti , Alessandra Quota , Michela Ribersani , Marco Marziali , Domenico Roberti , Rosamaria Rosso , Immacolata Tartaglione , Angelantonio Vitucci , Vincenzo Voi , Marco Zecca , Rodolfo Romero , Charbel Marouneld , Inmaculada Fernándezu2010Rozas , Carolina Espejo , Wulandewi Marhaeni , Marcos Garcia Aguado , Maria Domenica Cappellini , Silverio Perrotta , Lucia De Franceschi , Antonio Piga , Gian Luca Forni , Ibrahim Akin
Yıl :
2022
Filomena Longo
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Although numerous patient‐specific co‐factors have been shown to be associated with worse outcomes in COVID‐19, the prognostic value of thalassaemic syndromes in COVID‐19 patients remains poorly understood. We studied the outcomes of 137 COVID‐19 patients with a history of transfusion‐dependent thalassaemia (TDT) and transfusion independent thalassaemia (TIT) extracted from a large international cohort and compared them with the outcomes from a matched cohort of COVID‐19 patients with no history of thalassaemia. The mean age of thalassaemia patients included in our study was 41 ± 16 years (48.9% male). Almost 81% of these patients suffered from TDT requiring blood transfusions on a regular basis. 38.7% of patients were blood group O. Cardiac iron overload was documented in 6.8% of study patients, whereas liver iron overload was documented in 35% of study patients. 40% of thalassaemia patients had a history of splenectomy. 27.7% of study patients required hospitalization due to COVID‐19 infection. Amongst the hospitalized patients, one patient died (0.7%) and one patient required intubation. Continuous positive airway pressure (CPAP) was required in almost 5% of study patients. After adjustment for age‐, sex‐ and other known risk factors (cardiac disease, kidney disease and pulmonary disease), the rate of in‐hospital complications (supplemental oxygen use, admission to an intensive care unit for CPAP therapy or intubation) and all‐cause mortality was significantly lower in the thalassaemia group compared to the matched cohort with no history of thalassaemia. Amongst thalassaemia patients in general, the TIT group exhibited a higher rate of hospitalization compared to the TDT group (p = 0.001). In addition, the rate of complications such as acute kidney injury and need for supplemental oxygen was significantly higher in the TIT group compared to the TDT group. In the multivariable logistic regression analysis, age and history of heart or kidney disease were all found to be independent risk factors for increased in‐hospital, all‐cause mortality, whereas the presence of thalassaemia (either TDT or TIT) was found to be independently associated with reduced all‐cause mortality. The presence of thalassaemia in COVID‐19 patients was independently associated with lower in‐hospital, all‐cause mortality and few in‐hospital complications in our study. The pathophysiology of this is unclear and needs to be studied in vitro and in animal models.
DOI : 10.1111/jcmm.17026
ISSN: 1582-1838

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