Defera Tablet

Thalassaemia, Iron overload, Sickle cell disease

Transfusional Iron Overload Iron chelator indicated for the treatment of transfusional iron overload caused by thalassemia syndromes when current chelation therapy is inadequate Approval based on serum ferritin level reduction; no controlled trials demonstrating a direct treatment benefit (eg, improvement in disease-related symptoms, functioning, or increased survival) Initial: 25 mg/kg (actual body weight) PO TID Maximum dose: 100 mg/kg/day Round the dose to the nearest 250 mg

Transfusional Iron Overload Iron chelator indicated for treatment of transfusional iron overload in children aged >3 years caused by thalassemia syndromes, sickle cell disease, or other anemias Approval based on serum ferritin level reduction; no controlled trials demonstrating a direct treatment benefit (eg, improvement in disease-related symptoms, functioning, or increased survival) <3 years: Safety and efficacy not established 500-mg tablet (aged >8 yr) Initial: 25 mg/kg (actual body weight) PO TID Maximum dose: 100 mg/kg/day Round the dose to the nearest 250 mg

Renal impairment Mild-to-severe (eGFR 15-89 mL/min/1.73 m2): No dose adjustment necessary ESRD: Unknown

Clinical studies of deferiprone did not include sufficient numbers of subjects aged 65 and over to determine whether they respond differently from younger subjects. Other reported clinical experience has not identified differences in responses between the elderly and younger patients.

Hepatic impairment Mild or moderate (Child Pugh A or B): No dose adjustment necessary Severe: (Child Pugh C): Unknown

May be taken with or without food.

Agranulocytosis, pregnancy and lactation.

Deferiprone can cause agranulocytosis that can lead to serious infections and death. Neutropenia may precede the development of agranulocytosis. Measure the absolute neutrophil count (ANC) before starting Deferiprone and monitor regularly while on therapy. Interrupt Deferiprone therapy if neutropenia develops. Interrupt Deferiprone if infection develops and monitor the ANC more frequently. Advise patients taking Deferiprone to report immediately any symptoms indicative of infection. Liver Enzyme Elevations: Monitor monthly and discontinue for persistent elevations. Zinc Deficiency: Monitor during therapy and supplement for deficiency. Embryo-Fetal Toxicity: Can cause fetal harm. MONITORING PARAMETERS Monitor neutrophil count weekly and discontinue treatment if neutropenia develops. Monitor plasma-zinc concentration.

Not Classi Pregnancy Limited available data with use in pregnant women are insufficient to inform a drug-associated risk of major birth defects and miscarriage Verify the pregnancy status of females of reproductive potential prior to initiating therapy Contraception Females: Advise females of reproductive potential to avoid pregnancy during treatment and 6 months of contraception is recommended after cessation of therapy; advise females to immediately report pregnancy Males: Advise males with female sexual partners of reproductive potential to use effective contraception during treatment and 3 months of contraception is recommended after cessation of therapy Lactation There is no information regarding the presence of deferiprone in human milk, the effects on the breastfed child, or the effects on milk production Because of the potential for serious adverse reactions, including the potential for tumorigenicity shown for deferiprone in animal studies, advise not to breastfeed during treatment and for 2 weeks after last dose

Avoid using deferiprone with aluminium-containing antacids as it can chelate trivalent metal ions. Drugs Associated with Neutropenia or Agranulocytosis: Avoid coadministration. If co-administration is unavoidable, closely monitor the absolute neutrophil count. UGT1A6 Inhibitors: Avoid co-administration. Polyvalent Cations: Allow at least a 4-hour interval between administration of Deferiprone and drugs or supplements containing polyvalent cations (e.g., iron, aluminum, or zinc).

>10% Chromaturia (14.6%),Nausea (12.6%),Abdominal pain/discomfort (10.4%) 1-10% Arthralgia (9.8%),Vomiting (9.8%),Increased ALT (7.5%),Decreased neutrophil count (7.3%),Neutropenia (6.2%),Increased appetite (4%),Diarrhea (3%),Headache (2.5%),Dyspepsia (2%),Back pain (2%),Extremity pain (1.9%),Increased weight (1.9%),Agranulocytosis (1.7%),Arthropathy (1.4%),Increased AST (1.2%),Decreased appetite (1.1%)

Deferiprone is an orally effective iron-chelating agent. It is being used when desferrioxamine is unsuitable or contraindicated.

Defera 500mg Tablet manufactured by Jenphar Bangladesh Ltd.. Its generic name is Deferiprone.