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Article ; Online: How I treat thalassemia.

Rachmilewitz, Eliezer A / Giardina, Patricia J

2011 Volume 118, Issue 13, Page(s) 3479–3488

Abstract: The purpose of this article is to set forth our approach to diagnosing and managing the thalassemias, including β-thalassemia intermedia and β-thalassemia major. The article begins by briefly describing recent advances in our understanding of the ... ...

Abstract	The purpose of this article is to set forth our approach to diagnosing and managing the thalassemias, including β-thalassemia intermedia and β-thalassemia major. The article begins by briefly describing recent advances in our understanding of the pathophysiology of thalassemia. In the discussion on diagnosing the condition, we cover the development of improved diagnostic tools, including the use of very small fetal DNA samples to detect single point mutations with great reliability for prenatal diagnosis of homozygous thalassemia. In our description of treatment strategies, we focus on how we deal with clinical manifestations and long-term complications using the most effective current treatment methods for β-thalassemia. The discussion of disease management focuses on our use of transfusion therapy and the newly developed oral iron chelators, deferiprone and deferasirox. We also deal with splenectomy and how we manage endocrinopathies and cardiac complications. In addition, we describe our use of hematopoietic stem cell transplantation, which has produced cure rates as high as 97%, and the use of cord blood transplantation. Finally, we briefly touch on therapies that might be effective in the near future, including new fetal hemoglobin inducers and gene therapy.
MeSH term(s)	Algorithms ; Cardiovascular Diseases/epidemiology ; Cardiovascular Diseases/etiology ; Cardiovascular Diseases/therapy ; Endocrine System Diseases/epidemiology ; Endocrine System Diseases/etiology ; Endocrine System Diseases/therapy ; Humans ; Incidence ; Models, Biological ; Thalassemia/complications ; Thalassemia/epidemiology ; Thalassemia/etiology ; Thalassemia/therapy
Language	English
Publishing date	2011-08-02
Publishing country	United States
Document type	Journal Article ; Review
ZDB-ID	80069-7
ISSN	1528-0020 ; 0006-4971
ISSN (online)	1528-0020
ISSN	0006-4971
DOI	10.1182/blood-2010-08-300335
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Article ; Online: Late complications of mixed chimerism following allogeneic bone marrow transplantation for thalassemia major.

Spitzer, Barbara / Giardina, Patricia J / O'Reilly, Richard J / Boulad, Farid

Pediatric blood & cancer

2015 Volume 62, Issue 7, Page(s) 1303–1304

MeSH term(s)	Bone Marrow Transplantation/adverse effects ; Chimerism ; Humans ; Infant ; Male ; Prognosis ; Transplantation, Homologous ; beta-Thalassemia/complications ; beta-Thalassemia/therapy
Language	English
Publishing date	2015-07
Publishing country	United States
Document type	Case Reports ; Letter
ZDB-ID	2131448-2
ISSN	1545-5017 ; 1545-5009
ISSN (online)	1545-5017
ISSN	1545-5009
DOI	10.1002/pbc.25498
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Article ; Online: An update on the US adult thalassaemia population: a report from the CDC thalassaemia treatment centres.

Chapin, John / Cohen, Alan R / Neufeld, Ellis J / Vichinsky, Elliott / Giardina, Patricia J / Boudreaux, Jeanne / Le, Binh C / Kenney, Kristy / Trimble, Sean / Thompson, Alexis A

British journal of haematology

2021 Volume 196, Issue 2, Page(s) 380–389

Abstract: Thalassaemia is caused by genetic globin defects leading to anaemia, transfusion-dependence and comorbidities. Reduced survival and systemic organ disease affect transfusion-dependent thalassaemia major and thalassaemia intermedia. Recent improvements in ...

Abstract	Thalassaemia is caused by genetic globin defects leading to anaemia, transfusion-dependence and comorbidities. Reduced survival and systemic organ disease affect transfusion-dependent thalassaemia major and thalassaemia intermedia. Recent improvements in clinical management have reduced thalassaemia mortality. The therapeutic landscape of thalassaemia may soon include gene therapies as functional cures. An analysis of the adult US thalassaemia population has not been performed since the Thalassemia Clinical Research Network cohort study from 2000 to 2006. The Centers for Disease Control and Prevention supported US thalassaemia treatment centres (TTCs) to compile longitudinal information on individuals with thalassaemia. This dataset provided an opportunity to evaluate iron balance, chelation, comorbidities and demographics of adults with thalassaemia receiving care at TTCs. Two adult cohorts were compared: those over 40 years old (n = 75) and younger adults ages 18-39 (n = 201). The older adult cohort was characterized by higher numbers of iron-related comorbidities and transfusion-related complications. By contrast, younger adults had excess hepatic and cardiac iron and were receiving combination chelation therapy. The ethnic composition of the younger cohort was predominantly of Asian origin, reflecting the demographics of immigration. These findings demonstrate that comprehensive care and periodic surveys are needed to ensure optimal health and access to emerging therapies.
MeSH term(s)	Adolescent ; Adult ; Age Factors ; Aged ; Aged, 80 and over ; Combined Modality Therapy/adverse effects ; Combined Modality Therapy/methods ; Comorbidity ; Disease Management ; Disease Susceptibility ; Female ; Genetic Predisposition to Disease ; Humans ; Iron Overload/diagnosis ; Iron Overload/etiology ; Iron Overload/therapy ; Male ; Middle Aged ; Public Health Surveillance ; Retrospective Studies ; Sociodemographic Factors ; Thalassemia/diagnosis ; Thalassemia/epidemiology ; Thalassemia/etiology ; Thalassemia/therapy ; United States/epidemiology ; Young Adult
Language	English
Publishing date	2021-11-14
Publishing country	England
Document type	Journal Article ; Research Support, U.S. Gov't, P.H.S.
ZDB-ID	80077-6
ISSN	1365-2141 ; 0007-1048
ISSN (online)	1365-2141
ISSN	0007-1048
DOI	10.1111/bjh.17920
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Article ; Online: The palatability and tolerability of deferasirox taken with different beverages or foods.

Goldberg, Stuart L / Giardina, Patricia J / Chirnomas, Deborah / Esposito, Jason / Paley, Carole / Vichinsky, Elliott

Pediatric blood & cancer

2013 Volume 60, Issue 9, Page(s) 1507–1512

Abstract: Background: Deferasirox is a once-daily, oral iron chelator that was developed out of a need for a long-acting, conveniently-administered chelator for patients with transfusional hemosiderosis. The approved mode of administration requires taking ... ...

Abstract	Background: Deferasirox is a once-daily, oral iron chelator that was developed out of a need for a long-acting, conveniently-administered chelator for patients with transfusional hemosiderosis. The approved mode of administration requires taking deferasirox on an empty stomach with water, apple juice, or orange juice to limit variation in bioavailability. This required administration schedule might not be palatable for patients. Additionally, approximately one-quarter of patients experience mild to moderate gastrointestinal (GI) symptoms, which may pose additional challenges, particularly in the younger and older age ranges. We present a trial to assess the palatability and safety of various administration modes of deferasirox in pediatric and adult patients. Procedures: Participants rated palatability in a 4-week run-in phase, where deferasirox was administered per label. Subsequently, patients rated several administration modes during a 3-month assessment phase. Results: Palatability was more favorable during the assessment phase, with 47% of patient ratings for palatability being favorable while only 38% were favorable during the run-in phase. The most highly rated choice was deferasirox taken with a soft food at breakfast. In addition, there was an indication of improved GI tolerability during the assessment phase (symptoms were reported in 37% of patients during run-in and 32% during the assessment phase; rates of diarrhea decreased significantly). Although trough PK values increased, no major new toxicities were observed. Conclusions: These data indicate that different administration options may improve palatability and GI tolerability, which could have a positive impact on treatment adherence. (ClinicalTrials.gov number, NCT00845871)
MeSH term(s)	Adolescent ; Benzoates/administration & dosage ; Beverages ; Blood Transfusion ; Child ; Child, Preschool ; Female ; Food ; Food-Drug Interactions ; Hematologic Diseases/therapy ; Hemosiderosis/drug therapy ; Hemosiderosis/etiology ; Humans ; Iron Chelating Agents/administration & dosage ; Male ; Triazoles/administration & dosage
Chemical Substances	Benzoates ; Iron Chelating Agents ; Triazoles ; deferasirox (V8G4MOF2V9)
Language	English
Publishing date	2013-09
Publishing country	United States
Document type	Clinical Trial ; Journal Article ; Multicenter Study ; Research Support, Non-U.S. Gov't
ZDB-ID	2131448-2
ISSN	1545-5017 ; 1545-5009
ISSN (online)	1545-5017
ISSN	1545-5009
DOI	10.1002/pbc.24561
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Article ; Online: Epidemiologic and clinical characteristics of nontransfusion-dependent thalassemia in the United States.

Vichinsky, Elliott / Cohen, Alan / Thompson, Alexis A / Giardina, Patricia J / Lal, Ashutosh / Paley, Carole / Cheng, Wendy Y / McCormick, Nora / Sasane, Medha / Qiu, Ying / Kwiatkowski, Janet L

Pediatric blood & cancer

2018 Volume 65, Issue 7, Page(s) e27067

Abstract: Background: Nontransfusion-dependent thalassemia (NTDT) refers to a diverse group of thalassemia mutations and clinical phenotypes that do not require chronic transfusions. It is increasingly prevalent in the United States.: Procedure: This study ... ...

Abstract	Background: Nontransfusion-dependent thalassemia (NTDT) refers to a diverse group of thalassemia mutations and clinical phenotypes that do not require chronic transfusions. It is increasingly prevalent in the United States. Procedure: This study reviews the epidemiology and clinical characteristics of 138 patients with NTDT treated at four US thalassemia centers from 1997 to 2014. Data on laboratory results, transfusions, and clinical complications were collected from patient charts. Results: Overall, 84 patients with α-thalassemia (62 deletional hemoglobin H; 22 nondeletional hemoglobin H), 39 with β-thalassemia (26 with homozygous or double heterozygous β mutations; 13 with single β mutations with or without α triplication), and 15 with E/β-thalassemia (12 E/β Conclusions: NTDT in the United States is a multi-ethnic disease with different genotypic mutations and phenotypic manifestations. A higher than expected proportion of patients was Black/African American. NTDT-related complications are common and increase with age, supporting a need for early diagnosis.
MeSH term(s)	Adolescent ; Adult ; Aged ; Child ; Child, Preschool ; Female ; Genotype ; Humans ; Infant ; Infant, Newborn ; Male ; Middle Aged ; Phenotype ; Thalassemia/complications ; Thalassemia/epidemiology ; Thalassemia/genetics ; United States/epidemiology ; Young Adult
Language	English
Publishing date	2018-04-10
Publishing country	United States
Document type	Journal Article
ZDB-ID	2131448-2
ISSN	1545-5017 ; 1545-5009
ISSN (online)	1545-5017
ISSN	1545-5009
DOI	10.1002/pbc.27067
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Article ; Online: Toward optimizing the use of deferasirox: potential benefits of combined use with deferoxamine.

Grady, Robert W / Galanello, Renzo / Randolph, Rachel E / Kleinert, Dorothy A / Dessi, Carlo / Giardina, Patricia J

Haematologica

2012 Volume 98, Issue 1, Page(s) 129–135

Abstract: Patients with β-thalassemia require iron chelation therapy to protect against progressive iron overload and non-transferrin-bound iron. Some patients fail to respond adequately to deferoxamine and deferasirox monotherapy while others have side effects ... ...

Abstract	Patients with β-thalassemia require iron chelation therapy to protect against progressive iron overload and non-transferrin-bound iron. Some patients fail to respond adequately to deferoxamine and deferasirox monotherapy while others have side effects which limit their use of these drugs. Since combining deferiprone and deferoxamine has an additive effect, placing all patients into net negative iron balance, we investigated the possibility that combining deferasirox and deferoxamine would lead to similar results. We conducted 34-day metabolic iron balance studies in six patients in whom the relative effectiveness of deferasirox (30 mg/kg/day) and deferoxamine (40 mg/kg/day) was compared, alone and in combination. Patients consumed fixed low-iron diets; daily urinary and stool iron excretion were determined by atomic absorption. Red blood cell transfusions were given prior to each drug treatment to minimize the effects of ineffective erythropoiesis. Serial safety measures, hematologic parameters, serum chemistries, ferritin levels and urinalyses were determined. All patients were in negative iron balance when treated with deferoxamine alone while four of six patients remained in positive balance when deferasirox monotherapy was evaluated. Daily use of both drugs had a synergistic effect in two patients and an additive effect in three others. Five of six patients would be in negative iron balance if they used the combination of drugs just 3 days a week. No significant or drug-related changes were observed in the blood work-ups or urinalyses performed. We conclude that supplementing the daily use of deferasirox with 2 - 3 days of deferoxamine therapy would place all patients into net negative iron balance thereby providing a convenient way to tailor chelation therapy to the individual needs of each patient.
MeSH term(s)	Adult ; Benzoates/administration & dosage ; Deferasirox ; Deferoxamine/administration & dosage ; Drug Therapy, Combination ; Female ; Humans ; Iron/blood ; Iron/urine ; Iron Chelating Agents/administration & dosage ; Male ; Triazoles/administration & dosage ; beta-Thalassemia/drug therapy ; beta-Thalassemia/metabolism
Chemical Substances	Benzoates ; Iron Chelating Agents ; Triazoles ; Iron (E1UOL152H7) ; Deferoxamine (J06Y7MXW4D) ; Deferasirox (V8G4MOF2V9)
Language	English
Publishing date	2012-08-08
Publishing country	Italy
Document type	Clinical Trial ; Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't
ZDB-ID	2333-4
ISSN	1592-8721 ; 0017-6567 ; 0390-6078
ISSN (online)	1592-8721
ISSN	0017-6567 ; 0390-6078
DOI	10.3324/haematol.2012.070607
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Article ; Online: Safety and pharmacokinetics of the oral iron chelator SP-420 in β-thalassemia.

Taher, Ali T / Saliba, Antoine N / Kuo, Kevin H / Giardina, Patricia J / Cohen, Alan R / Neufeld, Ellis J / Aydinok, Yesim / Kwiatkowski, Janet L / Jeglinski, Brenda I / Pietropaolo, Keith / Berk, Gregory / Viprakasit, Vip

American journal of hematology

2017 Volume 92, Issue 12, Page(s) 1356–1361

Abstract: Our phase I, open-label, multi-center, dose-escalation study evaluated the pharmacokinetics (PK) of SP-420, a tridentate oral iron chelating agent of the desferrithiocin class, in patients with transfusion dependent β-thalassemia. SP-420 was administered ...

Abstract	Our phase I, open-label, multi-center, dose-escalation study evaluated the pharmacokinetics (PK) of SP-420, a tridentate oral iron chelating agent of the desferrithiocin class, in patients with transfusion dependent β-thalassemia. SP-420 was administered as a single dose of 1.5 (n = 3), 3 (n = 3), 6 (n = 3), 12 (n = 3), and 24 (n = 6) mg/kg or as a twice-daily dose of 9 mg/kg (n = 6) over 14-28 days. There was a near dose-linear increase in the mean plasma SP-420 concentrations and in the mean values for C
Language	English
Publishing date	2017-12
Publishing country	United States
Document type	Journal Article
ZDB-ID	196767-8
ISSN	1096-8652 ; 0361-8609
ISSN (online)	1096-8652
ISSN	0361-8609
DOI	10.1002/ajh.24914
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Article ; Online: Combined iron chelation therapy.

Galanello, Renzo / Agus, Annalisa / Campus, Simona / Danjou, Fabrice / Giardina, Patricia J / Grady, Robert W

Annals of the New York Academy of Sciences

2010 Volume 1202, Page(s) 79–86

Abstract: Patients with thalassemia major accumulate body iron over time as a consequence of continuous red blood cell transfusions which cause hepatic, endocrine, and cardiac complications. Despite the availability of three iron chelators, some patients fail to ... ...

Abstract	Patients with thalassemia major accumulate body iron over time as a consequence of continuous red blood cell transfusions which cause hepatic, endocrine, and cardiac complications. Despite the availability of three iron chelators, some patients fail to respond adequately to monotherapy with any of them. Combination therapy, consisting in the use of two chelators on the same day, has been introduced to increase the efficacy and to induce negative iron balance in patients with severe iron overload. Extensive long-term experience has shown that combined chelation with deferiprone and deferoxamine (DFO) rapidly reduces liver iron, serum ferritin, and myocardial siderosis, improves cardiac function, reverses and prevents endocrine complications, reduces cardiac mortality, and improves survival. Side effects, though significant, are manageable if properly monitored. Preliminary promising results have been obtained using combined chelation with deferasirox and DFO. As more drug combination regimes are evaluated, it should be possible to better tailor iron chelation to the needs of the patients, minimizing toxicity and maximizing efficacy throughout life.
MeSH term(s)	Chelation Therapy/methods ; Deferoxamine/adverse effects ; Deferoxamine/therapeutic use ; Drug Combinations ; Ferritins/blood ; Humans ; Iron/metabolism ; Iron Chelating Agents/adverse effects ; Iron Chelating Agents/therapeutic use ; Iron Overload/etiology ; Pyridones/adverse effects ; Pyridones/therapeutic use ; Transfusion Reaction ; beta-Thalassemia/complications ; beta-Thalassemia/therapy
Chemical Substances	Drug Combinations ; Iron Chelating Agents ; Pyridones ; deferiprone (2BTY8KH53L) ; Ferritins (9007-73-2) ; Iron (E1UOL152H7) ; Deferoxamine (J06Y7MXW4D)
Language	English
Publishing date	2010-08
Publishing country	United States
Document type	Journal Article
ZDB-ID	211003-9
ISSN	1749-6632 ; 0077-8923
ISSN (online)	1749-6632
ISSN	0077-8923
DOI	10.1111/j.1749-6632.2010.05591.x
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Article ; Online: Longitudinal monitoring of cardiac siderosis using cardiovascular magnetic resonance T2* in patients with thalassemia major on various chelation regimens: a 6-year study.

Ambati, Srikanth R / Randolph, Rachel E / Mennitt, Kevin / Kleinert, Dorothy A / Weinsaft, Jonathan W / Giardina, Patricia J

American journal of hematology

2013 Volume 88, Issue 8, Page(s) 652–656

Abstract: Cardiovascular magnetic resonance (CMR) and hepatic magnetic resonance imaging (MRI) have become reliable noninvasive tools to monitor iron excess in thalassemia major (TM) patients. However, long-term studies are lacking. We reviewed CMR and hepatic MRI ...

Abstract	Cardiovascular magnetic resonance (CMR) and hepatic magnetic resonance imaging (MRI) have become reliable noninvasive tools to monitor iron excess in thalassemia major (TM) patients. However, long-term studies are lacking. We reviewed CMR and hepatic MRI T2* imaging on 54 TM patients who had three or more annual measurements. They were managed on various chelation regimens. Patients were grouped according to their degree of cardiac siderosis: severe (T2, <10 msec), mild to moderate (T2 = 10-20 msec), and no cardiac siderosis (T2, >20 msec). We looked at the change in cardiac T2, liver iron concentration (LIC) and left ventricular ejection fraction (LVEF) at years 3 and 5. In patients with severe cardiac siderosis, cardiac T2* (mean ± SD) improved from 6.9 ± 1.6 at baseline to 13.6 ± 10.0 by year 5, mean ΔT2* = 6.7 (P = 0.04). Change in cardiac T2* at year 3 was not significant in the severe group. Patients with mild to moderate cardiac siderosis had mean cardiac T2* of 14.6 ± 2.9 at baseline which improved to 26.3 ± 9.5 by year 3, mean ΔT2* = 1.7 (P = 0.01). At baseline, median LICs (mg/g dry weight) in patients with severe, mild-moderate, and no cardiac siderosis were 3.6, 2.8, and 3.3, whereas LVEFs (mean ± SD) (%) were 56.3 ± 10.1, 60 ± 5, and 66 ± 7.6, respectively. No significant correlation was noted between Δ cardiac T2* and Δ LIC, Δ cardiac T2, and Δ LVEF at years 3 and 5. Throughout the observation period, patients with no cardiac siderosis maintained their cardiac T2 above 20 msec. The majority of patients with cardiac siderosis improve cardiac T2* over time with optimal chelation.
MeSH term(s)	Adolescent ; Adult ; Child ; Female ; Follow-Up Studies ; Heart/diagnostic imaging ; Heart/physiopathology ; Heart Diseases/diagnostic imaging ; Heart Diseases/etiology ; Heart Diseases/metabolism ; Heart Diseases/physiopathology ; Hemosiderosis/diagnostic imaging ; Hemosiderosis/etiology ; Hemosiderosis/physiopathology ; Humans ; Iron Chelating Agents/administration & dosage ; Liver/diagnostic imaging ; Liver/metabolism ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Monitoring, Physiologic/methods ; Myocardium/metabolism ; Radiography ; Stroke Volume ; beta-Thalassemia/complications ; beta-Thalassemia/diagnostic imaging ; beta-Thalassemia/drug therapy ; beta-Thalassemia/metabolism ; beta-Thalassemia/physiopathology
Chemical Substances	Iron Chelating Agents
Language	English
Publishing date	2013-06-28
Publishing country	United States
Document type	Clinical Trial ; Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't
ZDB-ID	196767-8
ISSN	1096-8652 ; 0361-8609
ISSN (online)	1096-8652
ISSN	0361-8609
DOI	10.1002/ajh.23469
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Article: Low bone mass in prepubertal children with thalassemia major: insights into the pathogenesis of low bone mass in thalassemia.

Vogiatzi, Maria G / Autio, Karen A / Schneider, Robert / Giardina, Patricia J

Journal of pediatric endocrinology & metabolism : JPEM

2004 Volume 17, Issue 10, Page(s) 1415–1421

Abstract: Objective: Low bone mass occurs frequently in the aging thalassemic population. However, limited information exists on bone mass in children with thalassemia major (TM) during their first decade of life.: Study design: Spinal bone mineral density ( ... ...

Abstract	Objective: Low bone mass occurs frequently in the aging thalassemic population. However, limited information exists on bone mass in children with thalassemia major (TM) during their first decade of life. Study design: Spinal bone mineral density (BMD) was measured by dual-energy X-ray absorptiometry (DEXA) in 18 children (age 5.8 +/- 1.5 yr; M:F 8:10) with TM on hypertransfusion and iron chelation therapy. Serial BMD measurements were available for 11 of the 18 children. Results: Weight and height z scores were 0.81 +/- 4.2 and -0.47 +/- 1.7 respectively. At the first BMD, four (22.2%) patients presented with BMD z scores less than -2.5, seven (38.8%) had BMD z scores between -1 and -2.5, while the remaining seven (38.8%) had normal BMDs (z score above -1). The mean decline of BMD z score was -0.38/year (p = ns). BMD z scores correlated with height z scores (p = 0.039), but not with liver enzymes, serum ferritin levels, or thalassemia genotypes. Conclusions: Low bone mass is present in most children with TM despite hypertransfusion and optimal chelation, adequate growth and lack of endocrine complications.
MeSH term(s)	Blood Transfusion ; Bone Density ; Bone Diseases, Metabolic/diagnosis ; Bone Diseases, Metabolic/etiology ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Humans ; Male ; Osteoporosis/diagnosis ; Osteoporosis/etiology ; beta-Thalassemia/complications ; beta-Thalassemia/therapy
Language	English
Publishing date	2004-09-03
Publishing country	Germany
Document type	Clinical Trial ; Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't ; Research Support, U.S. Gov't, P.H.S.
ZDB-ID	1231070-0
ISSN	2191-0251 ; 0334-018X
ISSN (online)	2191-0251
ISSN	0334-018X
DOI	10.1515/jpem.2004.17.10.1415
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