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CP Case Presentation: Blood Bank

CP Case Presentation: Blood Bank. Christina Day, MD July 14, 2004. This is the case of MC. 49 year old male with history of TTP in April 2003, HIV (on Kaletra and Combivir with undetectable viral load), and Glucose 6-Phosphate deficiency.

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CP Case Presentation: Blood Bank

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  1. CP Case Presentation: Blood Bank Christina Day, MD July 14, 2004

  2. This is the case of MC • 49 year old male with history of TTP in April 2003, HIV (on Kaletra and Combivir with undetectable viral load), and Glucose 6-Phosphate deficiency. • First episode of TTP was treated at Lincoln, during which he was disoriented, suffered renal complications and citrate intoxication, but had no allergic reactions.

  3. Presentation • Patient presented in ED with dizziness, weakness, jaundice, dark urine, DOE, HA and nausea x 4 days. • On exam: AA male, 5’11”, 177lb, alert and oriented, scleral icterus, clear chest, heart with RR @108/min, 2/6 SEM >@LLSB no radiation, guaiac negative. • Peripheral smear with schistocytes • Labs: VL <400, CD4 437

  4. Schistocyte

  5. Impression and Plan • Impression was relapse of his TTP. • Hematology and Transfusion services consulted. • Plan to initiate daily plasmapheresis as soon as a shiley catheter could be placed.

  6. Day #1 • Pt was pre-medicated with 50mg Benadryl. • Infusion of 14u FFP followed by therapeutic plasma exchange. • During the procedure the pt developed rigors that responded to Demerol. • Post-procedure he developed acute SOB, O2 saturation to 65% and ABG showed respiratory alkalosis.

  7. Hospital Day #1 continued • Chest X-ray at this time showed new diffuse lung infiltrates. • Initial assessment was fluid overload, Lasix given without improvement but responded well to 100% O2 on venturi mask and alb/atrovent nebs. • When transfusion service made aware, a diagnosis of transfusion-related acute lung injury, or TRALI, was made. Serum taken for HLA and Leukocyte antibody testing. • 7 hours later pt comfortable on 5L 02, @24hr RA

  8. Hospital Days # 2-5 • Pt continued to deny any SOB, fever, chills or discomfort, aside from pain at the femoral catheter site. • Daily plasmapheresis and PRBC transfusions were continued without complication. • The patient’s platelet count quickly improved and his jaundice decreased as the bilirubin normalized

  9. The Numbers

  10. Hospital Days # 6-9 • On day # 6 the pt became febrile to 103°F and continued to have pain and developed discharge from femoral catheter site. Line was removed • Blood cultures and wound swabs taken, positive for S. aureus on days #5 and 6. Vanco was started. • CXR remained negative. • Pt received TPE procedures 6-9 without incident. • Over the next three days lab values continue to worsen with pt remaining clinically stable.

  11. The Next Numbers

  12. What’s Going ON? • The question is whether this is a recurrence of the TTP or an isolated sepsis reaction. • The fact that the numbers have continued to drop in the labs while pt is on appropriate antibiotics may indicate a recurrence.

  13. Thrombotic Thrombocytopenic Purpura • Clinical syndrome defined by a classic pentad of symptoms: • Profound thrombocytopenia (number varies). • Microangiopathic hemolytic anemia (schistocytes on peripheral smear). • Fever. • Renal dysfunction (usually mild with Cr > 3.5). • Neurologic dysfunction (fluctuating status and generalized seizures).

  14. TTP • Usual clinical picture also shows: • Acute fulminant event • Plasma is a “coca cola” color • LDH is elevated to between 600 and 2500 IU/L.

  15. TTP: Incidence • 1991 was 1:1,000,000, now 3.7 per million. • All ages • Ratio of women to men is approximately 10:1

  16. TTP: Pathophysiology • Primary inciting event appears to be endothelial damage, especially small vessels • Entities known to cause endothelial damage are HIV and other viral and bacterial infections, pregnancy and drugs (cyclosporin, plavix, simvistatin) • This causes release of large vWF multimers and endothelin. • These large vWF molecules have greater platelet attraction and aggregation properties and adhere to damaged subendothelium

  17. TTP: Pathophysiology • Normally these multimers are cleaved by vWF specific metalloprotease (ADAMTS-13) • Recent research shows a deficiency of ADAMTS-13 or presence of an inhibitor to ADAMTS-13 is highly specific for cases of TTP in patient without an underlying medical illness.

  18. Thrombocytopenia in HIV • Anywhere from 3-30% of HIV infected pt. • Possible etiologies include immune-mediated destruction, TTP, impaired hematopoiesis, and toxic effects of medications…. Has been suggested that direct HIV infection of megakaryocytes impairs platelet production. • Most often an isolated hematological abnormality with increased or normal megakaryocytes in the bone marrow. • This is a syndrome commonly referred to as immune thrombocytopenic purpura (ITP).

  19. TTP and HIV • Increased incidence since 1991 is attributed in part to HIV and drug-induced reactions. • Majority of TTP pt with HIV have not developed AIDS and have relatively high CD4/CD8 ratios. • A good response to plasmapheresis is expected

  20. Transfusion-Related Acute Lung Injury • Uncommon, potentially fatal complication of allogenic transfusion (RBCs, Plt, FFP, Cryo and Granulocytes) first described in 1952. • Consists of acute pulmonary insufficiency manifested by severe dyspnea, hypoxemia, and CXR suggestive of pulmonary edema in the presence of normal cardiac function.

  21. TRALI • USDA: 3rd leading cause of transfusion related deaths in the USA. • 2nd only to ABO incompatibility in Britain for transfusion related morbidity and mortality. • Initial risk stated to be 1:5000 units transfused…more recent studies report a prevalence of 1:1120 units(1) (1) Silliman, C. et al, Transfusion-related acute lung injury: epidemiology and a prospective analysis of etiologic factors. Blood. 2003;101(2):454-462.

  22. TRALI: Clinical Presentation • Constellation of signs and symptoms including dyspnea, hypoxia, hypotension, fever and bilateral pulmonary edema. • Usually presents within 2-6 hours of transfusion. • Can be triggered by extremely small volumes of blood component.

  23. TRALI: Etiology is Controversial • Immunologic Mechanism • Non-immunologic Mechanism • Biologically Active Lipids Mechanism

  24. TRALI: Immunologic Mechanism 1. Immunologic Mechanism (65-85% ?): -Infusion of donor antileukocyte antibodies (HLA Class I and II and anti-granulocyte). -Ab complexes with recipient leukocyte antigens. -Complexes may activate neutrophils and production of inflammatory mediators. -Inflammatory mediators cause increased vascular permeability leading to capillary leaks and pulmonary edema.

  25. TRALI: Non-Immunologic • Predisposing patient conditions shown to place the patient at greater risk of developing TRALI: • Surgery (especially cardiothoracic). • Trauma • Severe infection • Thrombotic Thrombocytopenic Purpura Webert and Blajchman. Transfusion-related lung injury. Transfusion medicine reviews, 2003;17(4):252-262.

  26. TRALI: Biologically Active Lipids • In >15% of cases there is no Ab detected (Silliman found this to be true in approx 75%) • This means that either there were Ab there that were undetectable by current methods and assays, or some other factor is involved. • The “other factor” proposed is the infusion of biologic response modifiers, specifically biologically active lipids, IL-6 and IL-8, that have PMN-priming activity. • These lipids have been shown to accumulate in cellular blood products with prolonged storage. Silliman, C. et al. Plasma and lipids from stored platelets cause acute lung injury in an animal model. Transfusion, 2003;43:633-640.

  27. TRALI: BAL • Silliman goes on to propose there is a two-event process. • First, pt has a predisposing condition that causes cytokines, inflammatory or infectious mediators to prime PMNs and activate pulmonary endothelial cells leading to sequestration of PMNs. • Second, infusion of BRMs (antileukocyte Abs or lipids from stored blood) that activate the adherent PMNs resulting in endothelial damage, capillary leak and acute lung injury.

  28. TRALI: Diagnosis • No rapid or conclusive test. • Must have high index of suspicion if clinical picture develops within 6 hours of transfusion with exclusion of other causes of pulmonary edema. • Serum samples from patient and donor, if possible, for HLA and granulocyte Ab testing.

  29. TRALI: Treatment • Ventilatory support, which may include oxygenation, intubation and not infrequently, mechanical ventilation. • Vasopressors may be necessary to support hypotension (these pt are unresponsive to fluid infusion). • Treatment with steroids, PGE, NSAIDS, anti-TNF Ab and surfactant have been suggested but no established direct benefit. Webert and Blajchman. Transfusion-related lung injury. Transfusion medicine reviews, 2003;17(4):252-262.

  30. TRALI: Prognosis • Unlike ARDS, patients with TRALI have a good prognosis. • Generally improve within 48-96 hours. • Resolution of pulmonary CXR finding within 1-4 days • 20% have protracted hypoxemia and pulmonary infiltrates for >7 days. • 6-14% mortality... SERIOUS CONDITION!

  31. TRALI: Consensus? • A recent conference produced a report on TRALI in April 2004 stating: “The TRALI mechanism probably involved white cell antibody in donor blood, although other factors, such as a patient’s predisposed condition, seem to contribute. Data demonstrate increased TRALI when older units are transfused, possibly caused by a proliferation of [BRM] during storage.” The only thing that agreed on was that more research needs to be done!

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