Immune Status of Thalassemic Patients Receiving Deferiprone or Combined Deferiprone and Desferrioxamine Chelation Treatment

Athanassiou‐Metaxa, Miranda; Tzimouli, Vassiliki; Economou, Marina; Taparkou, Antonia; Tourkantoni, N.; Kanakoudi-Tsakalidou, F

doi:10.1159/000074235

Cited by 8 publications

(3 citation statements)

References 7 publications

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“…Accordingly, the reason for the reduced neutrophil count and agranulocytosis during DFP treatment is doubtful, but they occurred significantly more often in nonsplenectomized patients and in patients treated with a DFO-combined regimen [2][3][4][5][6][7][8][9], as in the case of our patient. However, concerns regarding potential immunosuppressive effects of DFP itself have been raised from results of animal studies and case reports in humans but no change in immunological status following also combined regimen administration had been clearly demonstrated [12][13][14]. Unfortunately CD8?, CD4?…”

Section: Discussionmentioning

confidence: 99%

Hepatitis B virus reactivation during combined therapy with deferiprone and desferioxamine in a hepatitis B surface antigen thalassemic carrier

Ricchi¹,

Cinque²,

Galeota³

et al. 2008

Int J Hematol

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In this report we firstly describe a case of reactivation of hepatitis B virus (HBV) replication occurred in a patient affected by Thalassemia major which underwent a combined chelation therapy with desferioxamine (DFO) and deferiprone (DFP). Clinical symptom and increase in alanine aminotransferase (ALT) level were detected when the prescription of DFO (30 mg/kg) was increased from 3 to 5 days/week; a raise in HBV-DNA levels of greater than or equal to tenfold compared with baseline was thereafter detected. Diagnosis was troublesome because increasing ALT levels, first suggested toxicity to DFP administration. However, HBV reactivation in our patient cannot be definitively attributed to combined regimen administration: the patient was on regular transfusion therapy and either coincidental further infection or spontaneous reactivation of HBV could not be completely ruled out. Furthermore, a background of immunologic abnormalities had been previously reported in thalassemia and postulated to be secondary to iron overload or DFO therapy itself.

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Section: Discussionmentioning

confidence: 99%

Hepatitis B virus reactivation during combined therapy with deferiprone and desferioxamine in a hepatitis B surface antigen thalassemic carrier

Ricchi¹,

Cinque²,

Galeota³

et al. 2008

Int J Hematol

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“…Furthermore, the modulation of activity of drugs and also of natural dietary or other molecules with iron binding properties has a major impact on therapeutic outcomes [ 212 , 213 , 214 , 215 , 270 ]. This is shown, for example, by the introduction of the L1/DF combination in the treatment of thalassaemia, which took many years to be developed from the original concept [ 50 , 97 , 271 , 272 ]. In this context, several other combination options between L1, DF, and DFRA are under investigation at present [ 103 , 105 , 273 , 274 , 275 , 276 , 277 and 278 ].…”

Section: Future Challenges and Potential New Clinical Uses Of Deferip...mentioning

confidence: 99%

“…Funding:Informed Consent Statement: Not applicable.Data Availability Statement: Hematol. 2019,12,[265][266][267][268][269][270][271][272] …”

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confidence: 99%

The Vital Role Played by Deferiprone in the Transition of Thalassaemia from a Fatal to a Chronic Disease and Challenges in Its Repurposing for Use in Non-Iron-Loaded Diseases

Kontoghiorghes¹

2023

Pharmaceuticals

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The iron chelating orphan drug deferiprone (L1), discovered over 40 years ago, has been used daily by patients across the world at high doses (75–100 mg/kg) for more than 30 years with no serious toxicity. The level of safety and the simple, inexpensive synthesis are some of the many unique properties of L1, which played a major role in the contribution of the drug in the transition of thalassaemia from a fatal to a chronic disease. Other unique and valuable clinical properties of L1 in relation to pharmacology and metabolism include: oral effectiveness, which improved compliance compared to the prototype therapy with subcutaneous deferoxamine; highly effective iron removal from all iron-loaded organs, particularly the heart, which is the major target organ of iron toxicity and the cause of mortality in thalassaemic patients; an ability to achieve negative iron balance, completely remove all excess iron, and maintain normal iron stores in thalassaemic patients; rapid absorption from the stomach and rapid clearance from the body, allowing a greater frequency of repeated administration and overall increased efficacy of iron excretion, which is dependent on the dose used and also the concentration achieved at the site of drug action; and its ability to cross the blood–brain barrier and treat malignant, neurological, and microbial diseases affecting the brain. Some differential pharmacological activity by L1 among patients has been generally shown in relation to the absorption, distribution, metabolism, elimination, and toxicity (ADMET) of the drug. Unique properties exhibited by L1 in comparison to other drugs include specific protein interactions and antioxidant effects, such as iron removal from transferrin and lactoferrin; inhibition of iron and copper catalytic production of free radicals, ferroptosis, and cuproptosis; and inhibition of iron-containing proteins associated with different pathological conditions. The unique properties of L1 have attracted the interest of many investigators for drug repurposing and use in many pathological conditions, including cancer, neurodegenerative conditions, microbial conditions, renal conditions, free radical pathology, metal intoxication in relation to Fe, Cu, Al, Zn, Ga, In, U, and Pu, and other diseases. Similarly, the properties of L1 increase the prospects of its wider use in optimizing therapeutic efforts in many other fields of medicine, including synergies with other drugs.

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Oral deferiprone for iron chelation in people with thalassaemia

Roberts

Brunskill

Dorée

et al. 2007

Cochrane Database of Systematic Reviews

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Immune Status of Thalassemic Patients Receiving Deferiprone or Combined Deferiprone and Desferrioxamine Chelation Treatment

Cited by 8 publications

References 7 publications

Hepatitis B virus reactivation during combined therapy with deferiprone and desferioxamine in a hepatitis B surface antigen thalassemic carrier

Hepatitis B virus reactivation during combined therapy with deferiprone and desferioxamine in a hepatitis B surface antigen thalassemic carrier

The Vital Role Played by Deferiprone in the Transition of Thalassaemia from a Fatal to a Chronic Disease and Challenges in Its Repurposing for Use in Non-Iron-Loaded Diseases

Oral deferiprone for iron chelation in people with thalassaemia

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