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www.kau.edu.sa/Files/140/Files/32055_Blood%20Trans... Presentation Transcript

  • 1. بسم الله الرحمن الرحيم بسم الله الرحمن الرحيم
  • 2. Guidelines and Updates in Blood Transfusion for ß -Thalassemia Patients Salwa Hindawi MSc, MRCPath, CTM Medical Director of Blood Transfusion Services KAUH, Jeddah KSA
  • 3. Introduction
        • Transfusion is the mainstay of the care of individuals with thalassemia major.
        • The purpose of transfusion is to improve the anemia and to suppress the ineffective erythropoiesis.
        • Chronic transfusions prevent most of the serious growth, skeletal, and neurological complications of thalassemia major.
  • 4. Introduction
    • The decision to start transfusions is based on inability to compensate for the low hemoglobin
    • signs of increased cardiac effort, tachycardia, sweating, poor feeding, and poor growth due to increasing symptoms of ineffective erythropoiesis : bone changes, massive splenomegaly.
  • 5. Guidelines
      • Schedule transfusions at three to four week intervals to maintain hemoglobin level greater
      • than or equal to 9-9.5 gm/dl prior to the next transfusion.
      • Evaluate hemoglobin prior to each transfusion, If the pre-transfusion hemoglobin is less than 9.0 gm/dl, the patient may need more frequent (every two to three weeks) transfusions or increased volume of transfusion.
  • 6. Guidelines
    • Perform extended red cell phenotyping prior to initiating the transfusion regime.
    • The use of phenotypically matched blood products, from the beginning of chronic transfusion, can prevent most cases of alloimmunization.
  • 7.
    • DONE ON FIRST SAMPLE
    • DONE ON THE RETICS
  • 8. Guidelines
    • The decision to start regular transfusions depends on clinical and laboratory assessment:
    • worsening anemia, inability to tolerate anemia, massive splenomegaly worsening bone disease, increasing nucleated red blood cells and dropping hemoglobin.
    • Skeletal malformation can be severe in thalassemia intermedia and should be considered in the decision to start transfusion.
  • 9. Guidelines
    • Assess spleen size at each visit, splenomegaly could account for increased blood requirement.
    • Calculate all blood given to the patient (total cc’s) and divided by an average weight over the past 6 months (cc / kg / year).
    • If transfusion requirement is greater than 200 cc / kg / year, the cause for such a high transfusion requirement should be explored.
  • 10. splenectomy
    • In the face of marked splenomegaly or other evidence of significant hypersplenism
    • (leukopenia, thrombocytopenia).
    • splenic embolization, splenectomy or partial
    • splenectomy may be considered.
  • 11. Splenectomy
    • Splenectomy is generally not recommended for thalassemia patients because of the risk of the complications.
    • Splenectomy is associated with significant risk of serious short and long term complications Infectious, pulmonary, hepatic, and thrombotic.
  • 12. Splenectomy
    • Pre splenectomy:
    • Vaccination
    • Obtain pneumococcal IgG titers. If the titers are inadequate, immunize to maximize coverage of all serotypes (7-valent conjugate vaccine recommended in children under five years (Prevnar)
    • 23 valent (Pneumovax) as a booster at five years of age or later. Reimmunize patients with inadequate IgG responses.
  • 13. Splenectomy
    • Post splenectomy:
      • -Monitor the platelet count and treat with an anti-platelet aggregate (low dose Aspirin) if platelet count is 1 x 106 or greater.
      • -Consider chronic low dose anticoagulation (coumadin) or anti-platelet agent (aspirin) in older splenectomized patients reduce the risk of pulmonary thrombotic events and pulmonary hypertension.
      • -All post splenectomy thalassemia patients require treatment with prophylactic penicillin.
      • -Intensive family education should be provided.
  • 14. Iron Overload
    • Regular blood transfusion can lead to Iron overload.
    • Serum iron & ferritin, TIBC, and/or liver Iron.
    • Chelation should be considered after one to two years of transfusion therapy, when the serum ferritin is greater than 1000 ng/dL, or when the hepatic iron is approximately 7 mg /gram dry weight
  • 15.
    • Determination of liver iron by biopsy is recommended prior to initiation of desferrioxamine therapy as well as every 12 to 24 months (or as clinically indicated).
    • Though not routinely available, liver iron can be also determined by ferritometry (SQUID).
    • Evaluate ferritin level quarterly.
  • 16. CHRONIC IRON OVERLOAD
    • ):
    • TOXICITY HEPATIC IRON
    • Desferrioxamine Toxicity Hearing Loss < 3 mg/g dry wt
    • Blindness
    • Growth Failure
    • Optimal Hepatic Iron Level 4 to 7.5 mg/g dry wt
    • Risk of Endocrine
    • Complications Diabetes Mellitus 7.5 to 15mg/g dry wt
    • Hypogonadism
    • Hypoparathyroidism
    • Cirrhosis > 10 mg / g dry weight
    • Risk of Cardiovascular
    • Complications Cardiomyopathy
    • Dysrythmia > 15 mg/g dry wt.
    • Olivieri and Brittenham Blood 89:3,1997,739-761
  • 17. Alloimmunization
    • The factors which contributing to alloimmunization :
    • 1-The RBC antigenic difference between the blood donor and the recipient.
    • 2-the recipient's immune status.
    • 3-the immunomodulatory effect of the allogeneic blood transfusions on the recipient's immune system.
  • 18. ALLOANTIBODIES AND AUTOANTIBODIES
    • If an autoantibody or/and alloantibody is detected, the specific antibodies causing the
    • transfusion reaction should be determined by the blood bank or by a reference laboratory.
    • The use of blood matched by extended antigen is usually indicated. Other treatment modalities, as steroids or immunosuppressive agents may be considered as well.
  • 19. ALLOANTIBODIES AND AUTOANTIBODIES
    • Autoimmunization or alloimmunization should be considered if the hemoglobin is less
    • than 9.0-9.5 gm/dl or is significantly less than usual for the particular patient prior to the
    • transfusion on two occasions.
    • Hemoglobin level and direct and indirect Coombs test should be determined 24 to72 hours after the transfusion.
  • 20.
    • antibodies screening and identification
    • Perform antibodies screening test (all patients & donors ) Using 3 cells panel screening cells
    • If antibody screening negative -NAD
    • If antibodies screening positive do antibody identification and autocontrol
    • PANEL CELLS POS AUTOCONTROL NEG
    • PANEL CELLS POS AUTOCONTROL POS
  • 21.
    • REAGENT CELL PANEL POS
    • AUTO CONTROL NEG
    • SOME CELLS NEG
    • SOME CELLS POS ( SAME STRENGTH & PHASES )
    • Suspect Single Antibody
    • Test other selected cells to eliminate other specificities
    • Test the patient cell to confirm they lack antigen ( phenotyping )
  • 22. REAGENT CELL PANEL POS AUTO CONTROL NEG
    • SOME CELLS NEG
    • SOME CELLS POS ( DIFFERENT STRENGTH & OR PHASES )
    • Suspect Multiple Antibody
    • ALL CELLS POS ( DIFFERENT STRENGTH & OR PHASES )
    • Suspect Multiple Antibody
    •  
  • 23.
    • Multiple antibodies identification:
    • Test selected cells to confirm and eliminate other specificities
    • Extended panel ( 15 or 20 cells panel )
    • You may need another technique ( enzyme )
    • Test the patient cell to confirm they lack antigen ( phenotyping )
    • May need help from reference laboratory for identification and confirmation
  • 24. Mangement
    • Compatible blood
    • Frozen –deglycerated rbcs
    • Least incompatible blood
    • *balanced decision
    • *avoid the strong immunogenic Ags
    • *premeditations ,initial slow infusion
    • *close monitoring and follow haemolysis indices
  • 25. Other options:
    • IvIg
    • Corticosteroids
    • Splenectomy
  • 26.
    • - Use of hydroxyurea & Erthropiotin.
    • Blood May,2003.
    • -Use of 2units collection through Apheresis from specific volunteer Donor.
  • 27. Hydroxyurea
    • The use of hydroxyurea may eliminate the need for future blood transfusions in children with beta-thalassemia major
    • administration of hydroxyurea to patients with severe forms of beta-thalassemia would result in production of fetal hemoglobin.
    WASHINGTON, DC - Blood August 12, 2003
  • 28. Singer ST; Wu V; Mignacca R; Kuypers FA; Morel P; Vichinsky EP Department of Hematology/Oncology at the Children's Hospital Oakland, California, USA
    • 64 transfused thalassemia patients (75% Asian) were evaluated.
    • 14 (22%) of 64 patients became alloimmunized. K, c, S, and Fyb accounts for 38% of the alloantibodies among Asian patients.
    • Patients who had a splenectomy had a higher rate of alloimmunization than patients who did not have a splenectomy 36% vs 12.8%.
    • Erythrocyte autoantibodies developed in 25% or 16 of the 64 patients.
    • Transfusion of phenotypically matched blood for the Rh and Kell proved to be effective in preventing alloimmunization
    • Blood.  2000; 96(10):3369-73
  • 29. Frequency of irregular red cell alloantibodies in patients with thalassemia major: a bicenter study
    • study conducted at two centers from January to December 2001 a total of 97 patients were included in the study.
    • Alloantibodies were found in 9 (9.2%). Mean age of patients who developed red cell alloantibody was 11.9 years. Three (33.3%) patients developed anti-K while two (22.2%) had non-specific antibody.
    • One 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%) developed anti-E and anti-K.
    J Pak Med Assoc 2005 Dec;55(12):563-5
  • 30. CONCLUSION
    • 1-There is relatively high rate of
    • alloimmunization in their patients when compared to data from the region.
    • 2- Red cell alloimmunization should not be overlooked in patients receiving regular blood transfusions.
  • 31. BONE MARROW TRANSPLANTATION
    • Bone marrow transplantation is the only cure for thalassemia patients.
    • It should be considered in all patients who have an acceptable donor.
    • Patients are classified on the basis of
    • their risk factors which include:
    • inadequate chelation, presence of liver fibrosis and hepatomegaly.
  • 32. Recommendations
    • 1) Extended rbc phenotyping
    • Perform extended red cell phenotyping prior to initiating the transfusion regime.
  • 33.
    • 2) Red cell matching.
    •  
    • Select ABO matched red cell units, which are K negative and matched for the common Rh antigens (D, C, E, c, e).
    •  
    • If clinically significant red cell antibodies are present, select antigen negative units and issue blood compatible in the crossmatch by IAT.
  • 34.
    • 3) Leucodepletion.
    •  
    • i) Bedside filtered red cells is not used routinely nowadays.
    •  
    • ii) Pre-storage Leucodepletion
    • Leucodepleted red cells (WBC <5 x 106 per unit) following filtration at the blood transfusion services (at source) are recommended.
  • 35.
    • 4) Age of the red cell units
    • Ideally, the red cell units selected for transfusion dependent patients should be less than 2 weeks old to ensure maximum possible survival in the recipient’s circulation
  • 36.
    • 5)Encourage central blood bank
        • With regular phenotype donors
        • Frozen blood
        • Frozen rare panel cell
    • 6)Cooperation between Hospitals.
    • 7)The use of new oral iron chelator for the treatment of iron overload.
  • 37. THANKS