# Thalassaemia Major & Trait Referral - Category: chronic - Review status: reviewed (every claim checked against a document named on this page) - Sources: Thalassaemia International Federation (TIF) Guidelines 2021 · Egyptian National Drug Formulary - Blood Disorder Medications 2025 (folic acid monograph) - Verified date: 2026-08 ## Verified against - Thalassaemia International Federation (TIF) Guidelines 2021 - Egyptian National Drug Formulary - Blood Disorder Medications 2025 (folic acid monograph) ## Treatment metadata - Deferasirox — 360 mg — oral.solid - Folic acid — 5 mg — oral.solid ## Complete treatment card ```text THALASSAEMIA MAJOR & TRAIT REFERRAL Sources: Thalassaemia International Federation (TIF) Guidelines 2021 · Egyptian National Drug Formulary - Blood Disorder Medications 2025 (folic acid monograph) Review status: REVIEWED against 2 sources listed above (2026-08) IS IT THIS? - reference only, to read alongside your own examination SYMPTOMS - what the patient reports (8) - The trait is usually picked up by chance on a routine blood count - Carriers have at most mild anaemia symptoms and little to find on examination [anaemia] - Major declares itself between 6 and 24 months, as fetal haemoglobin gives way to adult - The infant presents with feeding problems, irritability, faltering growth, pallor, diarrhoea, repeated fevers and a swollen abdomen [abdominal distension · diarrhoea · failure to thrive · irritability · pallor] - Intermedia is milder by definition and does not need regular transfusion - Intermedia can appear in a child as young as two, with growth and developmental delay [developmental delay] - The mildest intermedia may not surface until adulthood, as tiredness and pallor [fatigue · pallor] - Long-standing red cell breakdown brings gallstones and gallbladder symptoms [gallstones · haemolysis] SIGNS - what you find (5) - Untreated or undertreated children show stunted growth, jaundice, brown skin pigmentation and poor musculature [jaundice · stunted growth] - Also knock knees, a large liver and spleen, leg ulcers and masses of blood-forming tissue outside the marrow [foot deformity] - Marrow expansion deforms bone: a bossed forehead, an overgrown upper jaw and bent long bones - Intermedia can show the same marrow-expansion findings, yet make enough red cells to avoid transfusion - Under 2 years old with small-cell anaemia, mild jaundice and a big liver and spleen, the diagnosis is major [anaemia · jaundice] TESTS (12) - In major the count shows small pale cells, Hb less than 7g/dl, MCV 50 to 70 fl and MCH 12 to 20pg - Intermedia sits at Hb 7 to 10 g/dl, an MCV of 50 to 80 fl, and MCH between 16 and 24pg - In the trait the red cell count is often high, with a low MCV and MCH and only a slight RDW rise - A normal or barely raised RDW separates it from iron deficiency and sideroblastic anaemia, where RDW is high - The film shows small pale cells with target cells, teardrop cells and coarse basophilic stippling - In severe disease the film is bizarre, with wildly varying shapes and many nucleated red cells - Count and film alone cannot confirm it; confirmation needs haemoglobin electrophoresis or HPLC - The usual pattern is a fall in HbA with a mild rise in HbA2, and HbF variably raised - An HbA2 above 10% means a variant haemoglobin rather than beta-thalassaemia - The trait characteristically shows an HbA2 of 4-8% with HbF normal to slightly up - Major typically shows HbF markedly raised, from 30% to over 95%, with HbA2 normal or slightly up - Coexisting iron deficiency normalises the HbA2 and hides the trait, so the electrophoresis reads normal IF NOT THIS - what else fits (7) - Alpha thalassaemia is the main alternative small pale-cell anaemia - Anaemia of chronic disease, iron deficiency, sideroblastic anaemia and lead nephropathy complete the list - Sideroblastic anaemia is told apart by ring sideroblasts in marrow and raised red cell protoporphyrin, both absent here - Iron deficiency is excluded by normal iron studies: serum iron, iron-binding capacity and transferrin saturation - Other haemoglobin disorders need electrophoresis or HPLC to sort out - Major versus intermedia is decided clinically; no laboratory finding separates the two - A raised HbA2 also occurs with antiretroviral drugs, B12 or folate deficiency and an overactive thyroid Source StatPearls "Beta Thalassemia" - disease-level clinical article Status traced to the source above 1. DEFERASIROX [1st line] Adult 14 mg/kg once daily (film-coated tablet) or 20 mg/kg once daily (dispersible tablet) - long-term under haematology oversight Peds 14-28 mg/kg/day [child max 1400 mg] (Children >=2 years under pediatric haematologist: film-coated 14 mg/kg once daily, increased if needed to 28 mg/kg/day; dispersible 20 mg/kg once daily. The weight figures above are the film-coated range.) 3kg -> 42-84 mg/day 4kg -> 56-112 mg/day 5kg -> 70-140 mg/day 6kg -> 84-168 mg/day 7kg -> 98-196 mg/day 8kg -> 112-224 mg/day 9kg -> 126-252 mg/day 10kg -> 140-280 mg/day 11kg -> 154-308 mg/day 12kg -> 168-336 mg/day 13kg -> 182-364 mg/day 14kg -> 196-392 mg/day 15kg -> 210-420 mg/day 16kg -> 224-448 mg/day 17kg -> 238-476 mg/day 18kg -> 252-504 mg/day 19kg -> 266-532 mg/day 20kg -> 280-560 mg/day 21kg -> 294-588 mg/day 22kg -> 308-616 mg/day 23kg -> 322-644 mg/day 24kg -> 336-672 mg/day 25kg -> 350-700 mg/day 26kg -> 364-728 mg/day 27kg -> 378-756 mg/day 28kg -> 392-784 mg/day 29kg -> 406-812 mg/day 30kg -> 420-840 mg/day 31kg -> 434-868 mg/day 32kg -> 448-896 mg/day 33kg -> 462-924 mg/day 34kg -> 476-952 mg/day 35kg -> 490-980 mg/day 36kg -> 504-1008 mg/day 37kg -> 518-1036 mg/day 38kg -> 532-1064 mg/day 39kg -> 546-1092 mg/day 40kg -> 560-1120 mg/day 41kg -> 574-1148 mg/day 42kg -> 588-1176 mg/day 43kg -> 602-1204 mg/day 44kg -> 616-1232 mg/day 45kg -> 630-1260 mg/day 46kg -> 644-1288 mg/day 47kg -> 658-1316 mg/day 48kg -> 672-1344 mg/day 49kg -> 686-1372 mg/day 50kg -> 700-1400 mg/day Source Thalassaemia International Federation (TIF) Guidelines 2021 Why First-line oral iron chelator registered in Egypt for transfusional iron overload Caution IRON OVERLOAD ALERT: Deferasirox is prescribed strictly for transfusional thalassaemia major under specialist care. NEVER GIVE IRON SUPPLEMENTS: Iron overload causes fatal cardiac, hepatic, and endocrine toxicity. Monitor renal function (creatinine) and serum ferritin monthly. Thalassaemia trait (minor) requires reassurance and genetic counseling, NOT iron therapy unless co-existing iron deficiency proven. Egypt DEFEROXINOVA 125MG 10 DISP. TABS. DEBEIKY > INNOVA PHARMA 208.75 EGP -> ? strength differs DEFEROXINOVA 250MG 10 DISP. TABS. DEBEIKY > INNOVA PHARMA 392.50 EGP -> ? strength differs RASIROXPINE 125MG 28 DISP. TABS. HIKMA SPECIAL... 750.00 EGP [? strength differs] RONISIROX 500MG 10 DISPERSIBLE TABS. AVERROES PHARMA-EGYPT 825.00 EGP -> ? strength differs RASIROXPINE 250MG 28 DISP. TABS. HIKMA SPECIAL... 1099.00 EGP [? strength differs] EXJADE 125MG 28 DISP. TABS. NOVARTIS 1350.00 EGP [? strength differs] EXJADE 250MG 28 DISP. TABS. NOVARTIS 2550.00 EGP [? strength differs] EXJADE 500MG 28 DISP. TABS. NOVARTIS 4950.00 EGP [? strength differs] ANDORASIROX 500MG 20TABS. FOR ORAL SUSP. ANDALOUS PHARMA 1530.00 EGP (76.50/unit) -> ? strength differs, ? different route - not oral solid 2. FOLIC ACID [add-on - not a substitute] Adult 5 mg once daily - long-term Peds Children: 2.5-5 mg once daily Source Egyptian National Drug Formulary - Blood Disorder Medications 2025 (folic acid monograph) Why Adjunct vitamin supplement added to the referral pathway for thalassaemia major to meet the increased folate demand from chronic ineffective erythropoiesis; supports red cell production but does not replace transfusion therapy or specialist management. Caution Supports ineffective erythropoiesis. Do not combine with iron supplements. Egypt FOLIC ACID (EL NILE) 5 MG 50 TAB. EL NILE. 26.25 EGP (0.53/unit) FOLIC ACID (MEMPHIS) 5 MG 30 TAB. MEMPHIS 15.75 EGP (0.53/unit) FOLICINAD 5 MG 50 TABS. INAD PHARMA 50.00 EGP (1.00/unit) FOLIC ACID (EIPICO) 5 MG 20 TAB. EIPICO 24.00 EGP (1.20/unit) FOLIC ACID (EL NILE) 5 MG 30 TAB. EL NILE. 36.00 EGP (1.20/unit) FOLIRAQ 5000 MCG 30 CAPSULES HP PHARMA 45.00 EGP (1.50/unit) FOLIDDOX 400 MCG / 5 ML LIQUID 150 ML MADDOX PHARMA 150.00 EGP -> ? strength differs, ? different route - not oral solid Prices are indicative (dataset snapshot 2026-06); verify with the pharmacy. ``` --- Dawaa Reference is a reference for prescribers, not a medical device, and does not replace clinical judgement. [Privacy policy](/privacy)