1 / 34

BLOOD COMPONENT THERAPY IN PEDIATRICS

ERYILMAZ E M.D Assistant Professor ACIBADEM UNIVERSITY ACIBADEM SCHOOL OF MEDICINE. BLOOD COMPONENT THERAPY IN PEDIATRICS. LEARNING OBJECTIVES. Recognize what blood components are available, what they contain, and how to use them

freemanm
Download Presentation

BLOOD COMPONENT THERAPY IN PEDIATRICS

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. ERYILMAZ E M.D Assistant Professor ACIBADEM UNIVERSITY ACIBADEM SCHOOL OF MEDICINE BLOOD COMPONENT THERAPY IN PEDIATRICS

  2. LEARNING OBJECTIVES Recognize what blood components are available, what they contain, and how to use them Answer patients questions about infection and other risks of transfused blood components. Recognize the correct orders for blood components Know how to deal with ``unexpected antibodies`` Administer components safely avoiding the pitfalls Recognize the various adverse transfusion reactions, and know what to do when you suspect there has been an occurrence

  3. YOU ALREADY KNOW Basic hematopathology / Pathophysiology Basic immunology and genetics The infectious diseases that blood may carry The ABO system, with its naturally occurring isoagglutinins The Rh typing system and basic genetics The direct and indirect coombs test Diffuse alveolar damage (ARDS)‏

  4. WHAT IS AVAILABLE Whole Blood Packed red cells / Pediatric form Irradiated / Leuko-reduced Red cells (filtered and /or washed)‏ Granulocyte concentrates Platelet concentrates Fresh Frozen Plasma (FFP) Cryoprecipitate CMV negative blood products

  5. TESTING OF DONOR ABO Rh-include test for weak D , if negative (Allo) Antibody screen Hepatitis B (HbsAg , Anti-HBc)‏ Anti HCV and Nucleic acid Tech (NAT)‏ Anti HIV 1 –HIV2 and NAT West Nile Virus RNA Anti- HTLV I-II Serologic test for syphilis Malaria? Donor screening. No convenient test

  6. Component Preparation 450 ML whole blood + 63 ML (AP) solution Soft spin RBC (4 C – 21/ 42 days)‏ Hard spin PC 50 ml (21C - 5 days)‏ (platelet concentrate) Remaining Plasma 250 ml FFP (-18 C - 1 year)‏

  7. Apheresis Traditionally this has been used for platelet plasma and granulocyte collection, newer methods support RBC collection PC 3 x 10 (11)‏ RBC loss is minimal during platelet apheresis, donation can be performed more often than with WBC collection

  8. BLOOD COMPONENT CHARACTERISTICS storage volume expiry dose RBC (CPDA-1) 1-6 C 250ml 35 d 10-20ml/kg (AP) 1-6 C 300-350ml42 d10-20 ml/kg PC 20-24 C 50-75 ml 5 d 1-2 U/10kg* Apheresis 0-24C 200-400ml5 d 1-2U/10kg* FFP <-18C 200-500ml 1 year 10-20ml/kg (Thawed) 1-6 C200-500ml24 h 10-20ml/kg Cryoprecipitate < -18 C 10 -15 ml 1 year 1 U/5kg Granulocyte Con 20-24 C 200-300 ml 24 h 1-2 x 109 PMNs /kg/d

  9. LEUKOCYTE REDUCTION FNHTRs / Febrile non hemolytic transfusion reactions caused by donor WBC and cytokines, reduced by leukocte reduction Leukoreduced label = < 5x10 (6) WBC `s provided by filters even five times less with some newer filters. Leukocyte reduction is also used to reduce transmission of CMV in high risk patient populations.

  10. POST TRANSFUSION CMV RELATED MORBIDITY RISK FACTORS Prematurity, sero-negative neonates less than 1250g Hemapoetic stem cell and solid organ transplants Fetuses who receive intrauterine transfusion Severely immunocompromised individuals Leukocyte reduction <5x10(10) is effective in preventing CMV infection CMV sero-negative products use remains debate full 1,4% (sero-negative) versus %2,4 (LR)

  11. GAMMA IRRADIATION OF BLOOD COMPONENTS Gamma irradiation of blood comp at 2500cGy Transfusion associated graft versus host disease TA-GVHD occurs when an immunocompromised / deficient patient receives cellular blood products that possess immunologically compotent lymphocytes In the event where a donor is homozygous and a recipient is heterozygous for a particular HLA antigen the donor lymphoctes engraft in immuno compotent host resulting in TA-GVHD

  12. TA-GVHD CLINICAL SYMPTOMSand LABORATORY In 3-30 days Fever Erythemateous rash that may progress Anorexia Diarrhea Additionally *Severe cytopenia *Mild hepatitis- Fulminant liver failure *Mortality high (90% in pediatric population)‏

  13. TA-GVHD risk group Congenital immunodefficiencies of cellular immunity Intrauterine transfusion followed by neonatal exchange transfusion Bone marrow transplant recipients Recipients of HLA-Matched cellular components or blood components from blood related donors Patients who are undergoing intense chemotherapy or immunomodulatory therapy Neonates, prematurity ?

  14. ALLOIMMUNIZATION 18 % - 47% Sickle Cell Anemia (SCD)‏ 5%-11% Chronically transfused thalassemia 0,2 % - 2,6 % General population Methods to reduce the risk for alloimmunization in high risk populations vary: however phenotypically matching for Rh (D,C,E c and e) and Kell (K and k) decreases the incidence of alloantibodies per unit transfused and the incidence of hemolytic reactions in chronically transfused SCD patients.

  15. NEONATES Severe pulmonary or cyanotic heart disease/ congestive heart failure <15 gr/dl CPAP / MV with airway pressure > 6-8 cm H2O FiO2 >35% via oxygen hood < 12 gr /dl CPAP / MV with airway pressure < 6 cm H2O FiO2 < 35% via oxygen hood < 10 gr /dl

  16. NEONATES (2)‏ Low reticulocyte count and symptoms of anemia < 7 gr /dl Anemia within 24 hours of life <12 gr /dl Neonate receiving intensive care < 12 gr / dl Chronic oxygen dependency < 11 gr /dl Late anemia stable patient < 7 gr /dl

  17. PLATELETS Random donor platelet 1 U 5,5-7,5 X 10 (10) in 50-70 ml Plasma Apheresis = single donor platelet 4-8 U 3 x 10(11) in 250 ml Must be stored at 20-24 C Shelf life 5 days Risk of bacterial contamination highest Platelets express intrinsic ABO antigens but not Rh antigens Rh negative patients especially women should be transfused Rh (-) platelets because RBCs are present in small amounts or

  18. PLATELETS (2)‏ In cases when Rh negative platelets are unavailable Rh immune globuline may be administered within 72 hours of transfusion at a dose of 120 IU/mL of RBCs intramuscularly. Clinical factors considered when assessing the need for a platelet transfusion include the primary diagnosis…presence of fever, sepsis or splenomegaly. Also presence of uremia and medications may alter platelet function.

  19. PLATELETS (3)‏ Platelets should be maintained greater than 10 000 for adults and children who do not have an additional risk factors. > 20 000 for invasive procedures > 50 000 for major surgeries > 100 000 for CNS bleeding or planned CNS surgery > 30 000 in neonatal ICUs without other risk factors or previous intraventricular hemorragia. (Murray and colleagues ) A calculated platelet dose of 5 to 10 mL/kg for neonates and 0.1 to 0.2 U/kg for children over 10 kg should result in a platelet increment of 50 000 to 100 000 if no predisposing risk factors for refractoriness

  20. FFP Multiple coagulation factor deficiencies (eg, liver failure, vitamin K deficiency from malabsorbation or biliary disease or DIC)‏ Reversal of warfarin emergency Dilutional coagulopathy from massive transfusion Replacement of rare congenital factor deficiencies when spesific concentrates are not available (eg protein C or factor II, V, X, XI or XIII deficiency FFP should not be used to prevent bleeding except for a non- bleeding pt whose PT PTT is x1,5 times for intra vascular volume expansion correction/prevention of protein malnutrition When specific factor concentrates are available

  21. CRYOPRECIPITATE Cryoprecipitated antihemophilic factor, or cryoprecipitate, is preparedby thawing FFP at 1C to 6C, removing the supernatant, and refreezingat 18C for up to 1 year. The resulting small volume of precipitate containsconcentrated levels of factor VIII, factor XIII, factor VIII: von willebrand’sfactor (VWF), fibrinogen, and fibronectin Each cryoprecipitate unit (sometimesreferred to as a ‘‘bag’’ of cryoprecipitate, 10 to 15 mL) contains a minimumof 80 units of factor VIII activity and 150 mg of fibrinogen.

  22. CRYOPRECIPITATE (2)‏ Cryoprecipitate shouldnot be considered first-line treatment for hemophilia A and B or von Willebranddisease (VWD). In emergent cases of bleeding, however may be used for replacement Cryoprecipitate is indicated for treatment for activebleeding in patients who have dysfibrinogenemia, hypofibrinogenemia(<150 mg/dL), or afibrinogenemia with active bleeding. For complex coagulationfactor deficiency states (ie, DIC and dilutional coagulopathy), Cryoprecipitatemay be needed along with FFP to normalize fibrinogen levels. For correcting states of hypofibrinogenemia, the same replacement formulacan be applied, but in general, 1 U/5 kg should increase a small child’sfibrinogen by approximately 100 mg/dL

  23. PLASMA DERIVATIVES Plasma derivatives are concentrates of plasma proteins prepared from large donor pools (10,000 to 60,000) of plasma or cryoprecipitate. The specific protein of interest is purified and concentrated and cell fragments,cytokines, and viruses are inactivated or removed

  24. PLASMA DERIVATIVES (2)‏ Factor concentrates can be humanplasma derived or produced in vitro using genetically engineered celllines (recombinant). Human-derived and recombinant factor VIII and IXpreparations are available for short-term and prophylactic treatment ofbleeding in patients who have hemophilia A or B. Because the newer recombinantproducts (Recombinate, Kogenate, Advate, Benefix, and Novoseven)have limited or no albumin as a human protein, they are consideredextremely safe for transmitting human infectious organisms and, therefore,are the preferred products when available for specific single factor replacement.

  25. PLASMA DERIVATIVES (3)‏ • Certain selected human-derived factor VIII preparations (Humate Pand Koate-HP) contain significant amounts of VWF and are used for treatment of significant bleeding in VWD rather than cryoprecipitate when available. The VWF activity and dosing are expressed as ristocetin cofactor units. Recombinant factor VIIa (Novoseven) is indicated for the use of acute bleeding and prophylaxis for patients with hemophilia A or B, who have inhibitors to FVIII and FIX, respectively, and congenital factor VII deficiency.

  26. PLASMA DERIVATIVES (4)‏ Activated prothrombin complex (FEIBA) is a humanplasma–derived factor IX complex that in addition to factor IX containsvarious amounts of activated factors II, VII, and X and trace amounts offactor VIII. Although traditionally used for patients who have hemophiliaA and who have inhibitors, FEIBA has been replaced by recombinant factorVIIa because of a higher thrombotic risk at higher doses and because thesmall amounts of factor VIII present within the product can stimulate anamnesis,thereby increasing inhibitor titers in patients who have hemophiliaand who are considered for immune tolerance therapy .

  27. GRANULOCYTES Granulocytes are the least used blood component for many reasons Granulocyte transfusion should be used as an adjunct to other medicaltherapies, including the use of granulocyte-stimulating factors, antibiotics,and antifungals. Granulocyte transfusions frequently are accompanied by fevers, chills,band allergic reactions. More severe reactions, such as hypotension, respiratorydistress, and lung injury, occur in 1% to 5% of transfusions. Because granulocyte concentrates contain lymphocytes,all components should be gamma irradiated and CMV negative ifappropriate to recipients, because leukoreduction is contraindicated.

  28. ACUTE TRANSFUSION REACTIONS Fever is a common symptom of transfusion reactions. When a fever develops, serious transfusion reactions, such as bacterial contamination, acute hemolysis of ABO incompatible red cells, or transfusion-related acute lung injury (TRALI), should be considered; the transfusion should be stopped and the transfusion service notified so as to initiate the appropriate evaluation and quarantine any further products from the suspected donor unit. A common less severe etiology includes FNHTRs, which can result from the transfusion of RBCs, platelets, or plasma . In the past, FNHTRs occurred in up to 30% of transfusions; however, since the advent of leukoreduction, the incidence is only 0.1% to 3% for all products Although FNHTRs are relatively harmless, they may be uncomfortablefor recipients. A fever (1C increase in temperature) often is accompanied by chills, rigors, and overall discomfort making it difficult to discern thisentity from other more serious etiologies.

  29. ACUTE TRANSFUSION REACTIONS (2)‏ Allergic transfusion reactions (ATRs) are the most common of all acutetransfusion reactions. The severity of the allergic reaction can range from mild localized urticaria, pruritis, and flushing to bronchospasm and anaphylaxis. Unlike other acute transfusion reactions, fever usually is absent, and ifthe symptoms are mild and resolve with stopping the transfusion and administeringantihistamines, the transfusion may be restarted Most patientswho have ATRs respond to antihistamines and pretreatment may help toprevent recurrence. These reactions are caused by an antibody response inrecipients to soluble plasma proteins within the blood product. Leukoreductionis not shown to decrease the incidence of ATRs as it has for FNHTRs Severe ATRs leading to anaphylaxis often are the result of the developmentof anti-IgA antibodies in recipients who are IgA deficient.

  30. ACUTE TRANSFUSION REACTIONS (3)‏ Pretransfusion medication with antihistamines and steroids is recommendedand washed RBCs and platelets should be used because they remove most ofthe plasma responsible for the ATR. Epinephrine should be readily availableduring subsequent transfusions. In patients who are IgA deficientand have documented anti-IgA antibodies, IgA-deficient plasma productsmay be obtained

  31. ACUTE TRANSFUSION REACTIONS (4)‏ An acute hemolytic transfusion reaction (AHTR) occurs when RBCs aretransfused to a recipient who has preformedantibodies to antigens on thetransfused RBCs. Signs and symptoms of AHTRs include fever, chills, nausea, vomiting,shortness of breath, chest pain, hypotension, vasoconstriction, and hemoglobinuria,with potential progression to DIC and acute renal failure. When AHTR is suspected, the transfusion should be stopped immediately and a full transfusion evaluation initiated, which includes obtaining bloodcultures from the units, comparing the direct antibody test from the patient’spretransfusion crossmatch to the post-transfusion direct antibodytest, and a clerical check to verify if the correct unit was given to the correctpatient. Aggressive intravenous fluid therapy is required to maintain intravascularvolume and to prevent acute renal failure. The severity of the reactionand mortality rate are correlated directly to the rate and amount ofincompatible blood transfused. Mortality reaches 44%

  32. ACUTE TRANSFUSION REACTIONS (5)‏ TRALI is an uncommon yet potentially fatal acute immune-relatedtransfusion reaction that recently has become the leading cause of deathfrom transfusion. It typically occurs during or within4 hours of transfusion and presents with respiratory distress resultingfrom noncardiogenic pulmonary edema (normal central venous pressureand pulmonary capillary wedge pressure), hypotension, fever, and severehypoxemia (O2 saturation < 90% in room air)‏ TRALI usually improves after 48 to 96hours from onset of symptoms. TRALI is reported as a consequence of all blood product transfusions;however, plasma products (FFP and FP) account for the majority (50%–63%) of TRALI fatalities There are no definitive cases of TRALI documented in the neonatal population.

  33. Take Home Treat the patient not the lab values Most people < 7 gr /dl Hb do fine in short run 1 ml /kg FFP increases the concentration factor of each clotting factor by 1 % A unit of WB or RBC will raise the Hct by 3% and the Hb by 1 gr/dl 1 unit Cryoprecipitate per 10 kg child raises fibrinogen by 60-100 mg/dl

  34. HISTORICAL PERSPECTIVE ``…the physician must make every effort to avoid the transfusion of whole blood`` ``…despite its advantages, packed red blood cells should not be used when long-term platelet and granulocyte transfusions are anticipated.`` ``… leukocyte –poor red blood cell preparations… are far superior in respect to red blood cells than packed blood cells or whole blood`` ``…It must be remembered that there are times when a platelet transfusion is not indicated in spite of a very low platelet count `` ``…the use of plasma as an expander is somewhat questionable.``` Blood component therapy for cancer Benjamin Lichtiger CA Cancer J.Clin 1977 ; 27;194-200

More Related