Thallasemia

TREATMENT OF THALASEMIA DR. ARGHYA KUSUM PAL

CARDIO CLINICAL PSY ENDOCRIN ORTHO OPTHAL NEURO

Conventional Treatment Curative Treatment Alternative GENE THERAPY HbF MODULATION

TRANSFUSION THERAPY ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

WHOM TO TRANSFUSE ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

EVALUATION PRIOR TO TRANSFUSION ,[object Object],[object Object],[object Object],[object Object]

TRANSFUSION REGIMEN TYPE PRETRAN.Hb MEAN Hb RECOMMD PALLIATIVE <7 <8.5 NO MODERATE 9-10.5 >12 YES HYPER >10 >12 NO SUPER >12 >14 NO

What to transfuse? ,[object Object],[object Object],[object Object],[object Object],[object Object]

Blood products for special situations ,[object Object],[object Object],[object Object],[object Object],[object Object]

Frequency of transfusion SOURCE: MANAGEMENT PROTOCOL FOR TRT OF THAL. TIF,NICOSIA,CYPRUS ,[object Object],WEEKS BETWEEN TRANSFUSION Hb LEVEL gm/dl PRETRANFUSION POST-TRANSF MEAN 2 11.0 13.0 12 3 10.5 13.5 12 4 10.0 14.0 12 5 9.5 14.5 12 6 9.0 15.0 12

HOW MUCH TO TRANSFUSE ,[object Object],[object Object],TARGET Hb  Hct of donor cell 50% 60% 75% 80% 1 gm/dl 4.2 ml/kg 3.5 2.8 2.6 2 8.4 7.0 5.6 5.2 3 12.6 10.5 8.4 7.8 4 16.8 14.0 11.2 10.4

EFFICACY OF TRANSFUSION REGIMEN ,[object Object],[object Object]

ADVERSE REACTIONS OF BT IgA def. pt. Hypersensitivity reaction REACTION CAUSE TREATMENT NHFTR Leukocytes Anti-pyretic ALLERGIC RXN Plasma Prot Washed red cells Ac. Hemolytic rxn Faulty typing & CM 1.Stop . 2.IVF with diuretic. 3.Maintain vitals Auto immune Hemolytic rxn Alloimmunisation 1.Steroid. 2.Immunosuppresives 3.IV Ig Delayed transf.rxn Alloantibody -do- TRALI Anti-neutophil or Anti HLA. ,[object Object],[object Object],[object Object],GVHD INFECTIONS Treat as per cause

IRON OVERLOAD IN THALASSEMIA ,[object Object],[object Object],[object Object],160 – 200 ml/kg 120 -160 ml/kg 80 – 120 ml/kg Annual Blood Requirement (Haematocrit 75%) Annual Blood Requirement (Haematocrit 60%) Volume of Pure RBCs/kg (Haematocrit 100%) Daily IronLoading 100 – 150 ml/kg 60 -90 ml/kg 0.18 -0.27 mg/kg 150 – 200 ml/kg 90 – 120 ml/kg 0.27 – 0.36 mg/kg 200 -250 ml/kg 120 – 150 ml/kg 0.36 – 0.44 mg/kg

IRON OVERLOAD REDUCTION STRATEGY

CHELATION THERAPY ,[object Object],[object Object],[object Object],[object Object]

When to start ? ,[object Object],[object Object],[object Object]

DESFERIOXAMINE ,[object Object],THERAPEUTIC INDEX(PORTER’S INDEX ) = MEAN DAILY DOSE(mg/kg) / FERRITIN LEVEL(µg/L ) KEEP INDEX <0.025 AT ALL TIMES MEAN DAILY DOSE = (ACTUAL DOSE ON EACH OCCAS. X DOSES PER WEEK) /7 ,[object Object],FERRITIN LEVEL µg/L DOSE mg/kg/day < 2000 25 2000-3000 35 >3000 55 Not to exceed 100 mg/kg/day

Mode of administration ,[object Object],[object Object],[object Object],[object Object],[object Object]

Use of Vitamin C with DFO ,[object Object],[object Object],[object Object],[object Object],[object Object]

Continuous IV admin. Of DFO ,[object Object],[object Object],[object Object],[object Object]

MONITORING IRON OVERLOAD ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

DEFERIPRONE(L1) ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

COMBINATION THERAPY (DFO+L1) ,[object Object],[object Object],[object Object],[object Object]

DEFERASIROX(ICL 670) ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

SPLENECTOMY ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Complications ,[object Object],[object Object],[object Object],[object Object]

PREVENTIVE MEASURES ,[object Object],[object Object],[object Object]

Other compl. Of Splenec. ,[object Object],[object Object],[object Object],[object Object]

Post- transplant care ,[object Object],[object Object],[object Object],[object Object],[object Object]

PBSCT ,[object Object],[object Object],[object Object],[object Object]

CBSCT ,[object Object],[object Object],[object Object],[object Object]

ALTERNATIVE APPROACH ,[object Object],[object Object]

WHEAT GRASS THERAPY ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

MONITORING IN THALASSEMIA ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

CONTD. ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

CONTD… ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

DIET IN THALASSEMIA ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

STRATEGIES FOR CONTROL OF β -THAL. IN INDIA

Thallasemia

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Thallasemia