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  • 1. Salwa Hindawi
  • 2. Salwa HindawiSalwa Hindawi Guidelines and Updates in BloodGuidelines and Updates in Blood Transfusion forTransfusion for ßß -Thalassemia-Thalassemia PatientsPatients Salwa HindawiSalwa Hindawi MSc, MRCPath, CTMMSc, MRCPath, CTM Medical Director of Blood Transfusion ServicesMedical Director of Blood Transfusion Services KAUH, JeddahKAUH, Jeddah KSAKSA
  • 3. Salwa Hindawi IntroductionIntroduction Transfusion is the mainstay of the care ofTransfusion is the mainstay of the care of individuals with thalassemia majorindividuals with thalassemia major.. The purpose of transfusion is to improve theThe purpose of transfusion is to improve the anemia and to suppress the ineffectiveanemia and to suppress the ineffective erythropoiesiserythropoiesis.. Chronic transfusions prevent most of theChronic transfusions prevent most of the serious growth, skeletal, and neurologicalserious growth, skeletal, and neurological complications of thalassemia majorcomplications of thalassemia major..
  • 4. Salwa Hindawi IntroductionIntroduction The decision to start transfusions is based on inability toThe decision to start transfusions is based on inability to compensate for the low hemoglobincompensate for the low hemoglobin signs of increased cardiac effort, tachycardia, sweating,signs of increased cardiac effort, tachycardia, sweating, poor feeding, and poor growth due to increasingpoor feeding, and poor growth due to increasing symptoms of ineffective erythropoiesis : bone changes,symptoms of ineffective erythropoiesis : bone changes, massive splenomegalymassive splenomegaly..
  • 5. Salwa Hindawi GuidelinesGuidelines Schedule transfusions at three to four weekSchedule transfusions at three to four week intervals to maintain hemoglobin level greaterintervals to maintain hemoglobin level greater than or equal to 9-9.5 gm/dl prior to the nextthan or equal to 9-9.5 gm/dl prior to the next transfusiontransfusion.. Evaluate hemoglobin prior to each transfusion, IfEvaluate hemoglobin prior to each transfusion, If the pre-transfusion hemoglobin is less than 9.0the pre-transfusion hemoglobin is less than 9.0 gm/dl, the patient may need more frequent (everygm/dl, the patient may need more frequent (every two to three weeks) transfusions or increasedtwo to three weeks) transfusions or increased volume of transfusionvolume of transfusion..
  • 6. Salwa Hindawi GuidelinesGuidelines Perform extended red cell phenotyping prior toPerform extended red cell phenotyping prior to initiating the transfusion regimeinitiating the transfusion regime.. The use of phenotypically matched blood products,The use of phenotypically matched blood products, from the beginning of chronic transfusion, canfrom the beginning of chronic transfusion, can prevent most cases of alloimmunizationprevent most cases of alloimmunization..
  • 7. Salwa Hindawi • DONE ON FIRST SAMPLE • DONE ON THE RETICS
  • 8. Salwa Hindawi GuidelinesGuidelines The decision to start regular transfusions depends onThe decision to start regular transfusions depends on clinical and laboratory assessmentclinical and laboratory assessment:: worsening anemia, inability to tolerate anemia, massiveworsening anemia, inability to tolerate anemia, massive splenomegaly worsening bone disease, increasingsplenomegaly worsening bone disease, increasing nucleated red blood cells and dropping hemoglobinnucleated red blood cells and dropping hemoglobin.. Skeletal malformation can be severe in thalassemiaSkeletal malformation can be severe in thalassemia intermedia and should be considered in the decision tointermedia and should be considered in the decision to start transfusionstart transfusion..
  • 9. Salwa Hindawi GuidelinesGuidelines Assess spleen size at each visit, splenomegaly couldAssess spleen size at each visit, splenomegaly could account for increased blood requirementaccount for increased blood requirement.. Calculate all blood given to the patient (total cc’s) andCalculate all blood given to the patient (total cc’s) and divided by an average weight over the past 6 monthsdivided by an average weight over the past 6 months (cc / kg / year(cc / kg / year).). If transfusion requirement is greater than 200 cc / kg /If transfusion requirement is greater than 200 cc / kg / year, the cause for such a high transfusion requirementyear, the cause for such a high transfusion requirement should be exploredshould be explored..
  • 10. Salwa Hindawi splenectomysplenectomy In the face of marked splenomegaly or other evidenceIn the face of marked splenomegaly or other evidence of significant hypersplenismof significant hypersplenism ((leukopenia, thrombocytopenialeukopenia, thrombocytopenia).). splenic embolization, splenectomy or partialsplenic embolization, splenectomy or partial splenectomy may be consideredsplenectomy may be considered..
  • 11. Salwa Hindawi SplenectomySplenectomy Splenectomy is generally not recommended forSplenectomy is generally not recommended for thalassemia patients because of the risk of thethalassemia patients because of the risk of the complicationscomplications.. Splenectomy is associated with significant risk ofSplenectomy is associated with significant risk of serious short and long term complications Infectious,serious short and long term complications Infectious, pulmonary, hepatic, and thromboticpulmonary, hepatic, and thrombotic..
  • 12. Salwa Hindawi SplenectomySplenectomy Pre splenectomyPre splenectomy:: VaccinationVaccination Obtain pneumococcal IgG titers. If the titers areObtain pneumococcal IgG titers. If the titers are inadequate, immunize to maximize coverage of allinadequate, immunize to maximize coverage of all serotypes (7-valent conjugate vaccine recommendedserotypes (7-valent conjugate vaccine recommended in children under five years (Prevnarin children under five years (Prevnar(( 2323valent (Pneumovax( as a booster at five years of agevalent (Pneumovax( as a booster at five years of age or later. Reimmunize patients with inadequate IgGor later. Reimmunize patients with inadequate IgG responsesresponses..
  • 13. Salwa Hindawi SplenectomySplenectomy Post splenectomyPost splenectomy:: --Monitor the platelet count and treat with an anti-Monitor the platelet count and treat with an anti- platelet aggregate (low dose Aspirin( if plateletplatelet aggregate (low dose Aspirin( if platelet count is 1 x 106 or greatercount is 1 x 106 or greater.. --Consider chronic low dose anticoagulationConsider chronic low dose anticoagulation (coumadin( or anti-platelet agent (aspirin( in older(coumadin( or anti-platelet agent (aspirin( in older splenectomized patients reduce the risk ofsplenectomized patients reduce the risk of pulmonary thrombotic events and pulmonarypulmonary thrombotic events and pulmonary hypertensionhypertension.. --All post splenectomy thalassemia patients requireAll post splenectomy thalassemia patients require treatment with prophylactic penicillintreatment with prophylactic penicillin.. --Intensive family education should be providedIntensive family education should be provided..
  • 14. Salwa Hindawi Iron OverloadIron Overload Regular blood transfusion can lead to Iron overloadRegular blood transfusion can lead to Iron overload.. Serum iron & ferritin, TIBC, and/or liver IronSerum iron & ferritin, TIBC, and/or liver Iron.. Chelation should be considered after one to two yearsChelation should be considered after one to two years of transfusion therapy, when the serum ferritin isof transfusion therapy, when the serum ferritin is greater than 1000 ng/dL, or when the hepatic iron isgreater than 1000 ng/dL, or when the hepatic iron is approximately 7 mg /gram dry weightapproximately 7 mg /gram dry weight
  • 15. Salwa Hindawi Determination of liver iron by biopsy is recommendedDetermination of liver iron by biopsy is recommended prior to initiation of desferrioxamine therapy as wellprior to initiation of desferrioxamine therapy as well as every 12 to 24 months (or as clinically indicatedas every 12 to 24 months (or as clinically indicated(.(. Though not routinely available, liver iron can be alsoThough not routinely available, liver iron can be also determined by ferritometry (SQUIDdetermined by ferritometry (SQUID(.(. Evaluate ferritin level quarterlyEvaluate ferritin level quarterly..
  • 16. Salwa Hindawi CHRONIC IRON OVERLOADCHRONIC IRON OVERLOAD :(:( TOXICITY HEPATIC IRONTOXICITY HEPATIC IRON Desferrioxamine Toxicity Hearing Loss < 3 mg/g dry wtDesferrioxamine Toxicity Hearing Loss < 3 mg/g dry wt BlindnessBlindness Growth FailureGrowth Failure Optimal Hepatic Iron Level 4 to 7.5 mg/g dry wtOptimal Hepatic Iron Level 4 to 7.5 mg/g dry wt Risk of EndocrineRisk of Endocrine Complications Diabetes Mellitus 7.5 to 15mg/g dry wtComplications Diabetes Mellitus 7.5 to 15mg/g dry wt HypogonadismHypogonadism HypoparathyroidismHypoparathyroidism Cirrhosis > 10 mg / g dry weightCirrhosis > 10 mg / g dry weight Risk of CardiovascularRisk of Cardiovascular Complications CardiomyopathyComplications Cardiomyopathy Dysrythmia > 15 mg/g dry wtDysrythmia > 15 mg/g dry wt.. Olivieri and Brittenham Blood 89:3,1997,739-761Olivieri and Brittenham Blood 89:3,1997,739-761
  • 17. Salwa Hindawi AlloimmunizationAlloimmunization The factors which contributing toThe factors which contributing to alloimmunizationalloimmunization:: 11--The RBC antigenic difference between the bloodThe RBC antigenic difference between the blood donor and the recipientdonor and the recipient.. 22--the recipient's immune statusthe recipient's immune status.. 33--the immunomodulatory effect of the allogeneic bloodthe immunomodulatory effect of the allogeneic blood transfusions on the recipient's immune systemtransfusions on the recipient's immune system..
  • 18. Salwa Hindawi ALLOANTIBODIES ANDALLOANTIBODIES AND AUTOANTIBODIESAUTOANTIBODIES If an autoantibody or/and alloantibody is detected, theIf an autoantibody or/and alloantibody is detected, the specific antibodies causing thespecific antibodies causing the transfusion reaction should be determined by the bloodtransfusion reaction should be determined by the blood bank or by a reference laboratorybank or by a reference laboratory.. The use of blood matched by extended antigen is usuallyThe use of blood matched by extended antigen is usually indicated. Other treatment modalities, as steroids orindicated. Other treatment modalities, as steroids or immunosuppressive agents may be considered as wellimmunosuppressive agents may be considered as well..
  • 19. Salwa Hindawi ALLOANTIBODIES ANDALLOANTIBODIES AND AUTOANTIBODIESAUTOANTIBODIES Autoimmunization or alloimmunization should beAutoimmunization or alloimmunization should be considered if the hemoglobin is lessconsidered if the hemoglobin is less than 9.0-9.5 gm/dl or is significantly less than usual forthan 9.0-9.5 gm/dl or is significantly less than usual for the particular patient prior to thethe particular patient prior to the transfusion on two occasionstransfusion on two occasions.. Hemoglobin level and direct and indirect Coombs testHemoglobin level and direct and indirect Coombs test should be determined 24 to72 hours after theshould be determined 24 to72 hours after the transfusiontransfusion..
  • 20. Salwa Hindawi antibodies screening and identificationantibodies screening and identification Perform antibodies screening test (all patients &Perform antibodies screening test (all patients & donors ( Using 3 cells panel screening cellsdonors ( Using 3 cells panel screening cells If antibody screening negative -NADIf antibody screening negative -NAD If antibodies screening positive do antibodyIf antibodies screening positive do antibody identification and autocontrolidentification and autocontrol PANEL CELLS POS AUTOCONTROL NEGPANEL CELLS POS AUTOCONTROL NEG PANEL CELLS POS AUTOCONTROL POSPANEL CELLS POS AUTOCONTROL POS
  • 21. Salwa Hindawi REAGENT CELL PANEL POSREAGENT CELL PANEL POS AUTO CONTROL NEGAUTO CONTROL NEG SOME CELLS NEGSOME CELLS NEG SOME CELLS POS ( SAME STRENGTH & PHASESSOME CELLS POS ( SAME STRENGTH & PHASES(( Suspect Single AntibodySuspect Single Antibody Test other selected cells to eliminate other specificitiesTest other selected cells to eliminate other specificities Test the patient cell to confirm they lack antigen ( phenotypingTest the patient cell to confirm they lack antigen ( phenotyping((
  • 22. Salwa Hindawi REAGENT CELL PANEL POSREAGENT CELL PANEL POS AUTO CONTROL NEGAUTO CONTROL NEG  SOME CELLS NEGSOME CELLS NEG SOME CELLS POS ( DIFFERENT STRENGTH & OR PHASESSOME CELLS POS ( DIFFERENT STRENGTH & OR PHASES(( Suspect Multiple AntibodySuspect Multiple Antibody  ALL CELLS POS ( DIFFERENT STRENGTH & OR PHASESALL CELLS POS ( DIFFERENT STRENGTH & OR PHASES(( Suspect Multiple AntibodySuspect Multiple Antibody  
  • 23. Salwa Hindawi Multiple antibodies identificationMultiple antibodies identification:: Test selected cells to confirm and eliminate otherTest selected cells to confirm and eliminate other specificitiesspecificities Extended panel ( 15 or 20 cells panelExtended panel ( 15 or 20 cells panel(( You may need another technique ( enzymeYou may need another technique ( enzyme(( Test the patient cell to confirm they lack antigenTest the patient cell to confirm they lack antigen ( phenotyping( phenotyping(( May need help from reference laboratory forMay need help from reference laboratory for identification and confirmationidentification and confirmation
  • 24. Salwa Hindawi MangementMangement Compatible bloodCompatible blood Frozen –deglycerated rbcsFrozen –deglycerated rbcs Least incompatible bloodLeast incompatible blood **balanced decisionbalanced decision **avoid the strong immunogenic Agsavoid the strong immunogenic Ags **premeditations ,initial slow infusionpremeditations ,initial slow infusion **close monitoring and follow haemolysis indicesclose monitoring and follow haemolysis indices
  • 25. Salwa Hindawi Other optionsOther options:: IvIgIvIg CorticosteroidsCorticosteroids SplenectomySplenectomy
  • 26. Salwa Hindawi --Use of hydroxyurea & ErthropiotinUse of hydroxyurea & Erthropiotin.. Blood May,2003Blood May,2003.. --Use of 2units collection through Apheresis fromUse of 2units collection through Apheresis from specific volunteer Donorspecific volunteer Donor..
  • 27. Salwa Hindawi HydroxyureaHydroxyurea The use of hydroxyurea may eliminate the need forThe use of hydroxyurea may eliminate the need for future blood transfusions in children with beta-future blood transfusions in children with beta- thalassemia majorthalassemia major administration of hydroxyurea to patients with severeadministration of hydroxyurea to patients with severe forms of beta-thalassemia would result in productionforms of beta-thalassemia would result in production of fetal hemoglobinof fetal hemoglobin.. WASHINGTON, DC - Blood August 12, 2003
  • 28. Salwa Hindawi Singer ST; Wu V; Mignacca R; Kuypers FA; Morel P;Singer ST; Wu V; Mignacca R; Kuypers FA; Morel P; Vichinsky EPVichinsky EP Department of Hematology/Oncology at the Children's Hospital Oakland,Department of Hematology/Oncology at the Children's Hospital Oakland, California, USACalifornia, USA 6464transfused thalassemia patients (75% Asian( were evaluatedtransfused thalassemia patients (75% Asian( were evaluated.. 1414((22%22%((of 64 patients became alloimmunized. K, c, S, and Fybof 64 patients became alloimmunized. K, c, S, and Fyb accounts for 38% of the alloantibodies among Asian patientsaccounts for 38% of the alloantibodies among Asian patients.. Patients who had a splenectomy had a higher rate ofPatients who had a splenectomy had a higher rate of alloimmunization than patients who did not have aalloimmunization than patients who did not have a splenectomy 36% vs 12.8%splenectomy 36% vs 12.8%.. Erythrocyte autoantibodies developed in 25% or 16 of the 64Erythrocyte autoantibodies developed in 25% or 16 of the 64 patientspatients.. Transfusion of phenotypically matched blood for the Rh and KellTransfusion of phenotypically matched blood for the Rh and Kell proved to be effective in preventing alloimmunizationproved to be effective in preventing alloimmunization Blood. 2000; 96(10(:3369-73Blood. 2000; 96(10(:3369-73
  • 29. Salwa Hindawi Frequency of irregular red cell alloantibodies inFrequency of irregular red cell alloantibodies in patients with thalassemia major: a bicenterpatients with thalassemia major: a bicenter studystudy study conducted at two centers from January to December 2001 astudy conducted at two centers from January to December 2001 a total of 97 patients were included in the studytotal of 97 patients were included in the study.. Alloantibodies were found in 9 (9.2%(. Mean age of patients whoAlloantibodies were found in 9 (9.2%(. Mean age of patients who developed red cell alloantibody was 11.9 years. Three (33.3%(developed red cell alloantibody was 11.9 years. Three (33.3%( patients developed anti-K while two (22.2%( had non-specificpatients developed anti-K while two (22.2%( had non-specific antibodyantibody.. One patient developed anti-D (11.1%( and anti-E (11.1%(. Two hadOne patient developed anti-D (11.1%( and anti-E (11.1%(. Two had anti-D (11.1%( and anti-C while the other one (11.1%( developedanti-D (11.1%( and anti-C while the other one (11.1%( developed anti-E and anti-Kanti-E and anti-K.. J Pak Med Assoc 2005 Dec;55(12(:563-5
  • 30. Salwa Hindawi CONCLUSIONCONCLUSION 11--There is relatively high rate ofThere is relatively high rate of alloimmunization in their patients whenalloimmunization in their patients when compared to data from the regioncompared to data from the region.. 22--Red cell alloimmunization should not beRed cell alloimmunization should not be overlooked in patients receiving regularoverlooked in patients receiving regular blood transfusionsblood transfusions..
  • 31. Salwa Hindawi BONE MARROWBONE MARROW TRANSPLANTATIONTRANSPLANTATION Bone marrow transplantation is the only cure forBone marrow transplantation is the only cure for thalassemia patientsthalassemia patients.. It should be considered in all patients who have anIt should be considered in all patients who have an acceptable donoracceptable donor.. Patients are classified on the basis ofPatients are classified on the basis of their risk factors which includetheir risk factors which include:: inadequate chelation, presence of liver fibrosis andinadequate chelation, presence of liver fibrosis and hepatomegalyhepatomegaly..
  • 32. Salwa Hindawi RecommendationsRecommendations 11((Extended rbc phenotypingExtended rbc phenotyping Perform extended red cell phenotyping priorPerform extended red cell phenotyping prior to initiating the transfusion regimeto initiating the transfusion regime..
  • 33. Salwa Hindawi 22((Red cell matchingRed cell matching.. Select ABO matched red cell units, which are KSelect ABO matched red cell units, which are K negative and matched for the common Rhnegative and matched for the common Rh antigens (D, C, E, c, eantigens (D, C, E, c, e(.(. If clinically significant red cell antibodies areIf clinically significant red cell antibodies are present, select antigen negative units andpresent, select antigen negative units and issue blood compatible in the crossmatch byissue blood compatible in the crossmatch by IATIAT..
  • 34. Salwa Hindawi 33((LeucodepletionLeucodepletion.. i( Bedside filtered red cells is not used routinelyi( Bedside filtered red cells is not used routinely nowadaysnowadays.. ii( Pre-storage Leucodepletionii( Pre-storage Leucodepletion Leucodepleted red cells (WBC <5 x 106 per unit(Leucodepleted red cells (WBC <5 x 106 per unit( following filtration at the blood transfusion servicesfollowing filtration at the blood transfusion services (at source( are recommended(at source( are recommended..
  • 35. Salwa Hindawi 44((Age of the red cell unitsAge of the red cell units Ideally, the red cell units selected forIdeally, the red cell units selected for transfusion dependent patients should betransfusion dependent patients should be less than 2 weeks old to ensure maximumless than 2 weeks old to ensure maximum possible survival in the recipient’spossible survival in the recipient’s circulationcirculation
  • 36. Salwa Hindawi 55((Encourage central blood bankEncourage central blood bank With regular phenotype donorsWith regular phenotype donors Frozen bloodFrozen blood Frozen rare panel cellFrozen rare panel cell 66((Cooperation between HospitalsCooperation between Hospitals.. 77((The use of new oral iron chelator for theThe use of new oral iron chelator for the treatment of iron overloadtreatment of iron overload..
  • 37. Salwa Hindawi