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P-42 EXPERIENCE OF LIPID APHERESIS PROCEDURE OF MERSIN UNIVERSITY APHERESIS UNIT
Poster presentations / Transfusion and Apheresis Science 47 (2012) S21–S57
Hyperbilirubinemia is a common clinical manifestation, and may be life threatening. Many diseases result in hyperbilirubinemias, some are refractory, and cannot be cured by medication or surgery. How to reduce the high concentration of bilirubin in the blood of patients has always been a focus of study for many researchers. We treated severe hyperbilirubinemia by performing plasma exchange. Plasma exchange not only removes bilirubin, endotoxin and complement activator, but also replenishes albumin, coagulation factor and hepatic regenerative stimulating substance, which can correct metabolic disorder. It is not easy to decide when to start plasma exchange, the recovery liver function is difficult to asses. The problems with plasma exchange are the difficulty in obtaining large amounts of fresh frozen plasma and its high cost. The survival rate of patients with hyperbilirubinemia might be improved with plasma exchange and timely management of complications. The implementation of plasma exchange should not be delayed when it is indicated, it can be a therapeutic choice in these patients. P-42 EXPERIENCE OF LIPID APHERESIS PROCEDURE OF MERSIN UNIVERSITY APHERESIS UNIT N. Tiftik1 , F. Koksal2 , T. Ozcan2 , A. Tombak1 , M.A. Ucar1 , S. Kizilalp1 , O. Hallioglu3 , A. Kiykim4 . 1 Mersin University Medical Faculty Internal Medicine Hematology Department, 2 Mersin University Medical Faculty Cardiology Department, 3 Mersin University Medical Faculty Pediatric Cardiology Department, 4 Mersin University Medical Faculty Nephrology Department, Turkey The patients with severe hyperlipidemia are sometimes resistant to diet and lipid-lowering drugs. Lipid apheresis is the therapeutic option for such patients. Here, we report our experience of lipid apheresis procedure of our apheresis unit. One pediatric patient with familial hypercholesterolemia was treated by direct adsorption of lipoproteins (DALI). Same pediatric patient and 7 adult patients with hypercholesterolemia and hypertriglyceridemia underwent cascade filtration. Twenty procedures with DALI and 20 procedures with cascade filtration were performed. In DALI, the mean of total cholesterol values before and after therapy were 475 and 154 mg/dL. The mean of low-density lipoprotein (LDL) cholesterol values before and after therapy were 422 and 114 mg/dL. In cascade filtration, the mean of total cholesterol values before and after therapy were 467 and 129 mg/dL. The mean of LDL cholesterol before and after therapy 353 and 75 mg/dL. Four patients with hypertriglyceridemia underwent cascade filtration and mean triglyceride values before and after therapy were 1314 mg/dL and 336 mg/dL, respectively. Allergic reaction was occured in 2 patients applied DALI, and heart block was occured in one patient applied cascade filtration. According to the experince of our apheresis unit, lipid apheresis is a therapeutic procedure in patients with high risk hypercholesterolemia and hypertriglyceridemia despite efficient medical therapy.
S41
P-43 THE CONTROL OF ACUTE LYMPHOBLASTIC LEUKAEMIA CAUSED HYPERLEUKOCYTOSIS WITH LEUKOPHERESIS ON AN ADULT ATAXIA-TELANGIECTASIA PATIENT M. Keklik1 , S. Sivgin1 , B.S. Kalin1 , G. Akyol1 , C. Pala1 , M. Solmaz1 , L. Kaynar1 , B. Eser1 , M. Cetin1 , A. Unal1 . 1 Erciyes University Division of Hematology, Kayseri, Turkey Introduction: Ataxia-telangiectasia (AT) is a hereditary disorder characterized by progressive neurological dysfunction, oculocutaneous telangiectasia, immunodeficiency, cancer susceptibility, and radiation sensitivity. The prognosis of the malignancies is impaired by the immunodeficiency and the susceptibility to ionising radiation and chemotherapeutics. Pediatric patients may develop lymphomas and acute lymphoblastic leukaemia (ALL), especially of the T-lineage. However the AT and ALL on adults are less common so we presented a case of hyperleukocytosis leukostasys to which we performed leukapheresis. Patient: 25 years old male patient, while followed for AT and mental retardation diagnoses, was admitted to the Emergency unit due to fatigue, nausea and headaches. Patient had a moderate general condition and cooperation was established. Fever was 37.5°C, Pulse: 120 bpm and blood presure: 100/70 mmHg. No lymphadenopathy, splenomegaly or hepatomegaly was observed to the patient. Hb: 13.6 g/dl. WBC: 466×103 /mL. Plt: 6×103 7 mL. Peripheral blood smear showed dominance of blasts. Peripheral blood results of flow cytometry showed T-ALL and BUN: 28 mg/dl. Creatinin: 0.7 mg/dl. AST: 58 u/L. ALT: 71 u/L. Results: 2 sessions of large volume leukapheresis was performed to the patient by the insertion of a central venous catheter at the Apheresis Unit. Fresh frozen plasma (FFP) was used as replacement fluid. After the procedure WBC value decreased to 170×103 /mL. There were no procedure-related adverse events. Symptoms due to hyperleukocytosis markedly improved after leukapheresis. In addition the chemotherapy protocol including parenteral steroid, vincristine, doxorubicin, methotrexate, folinic acid and L. asparaginase was performed to the patient. Patients also was treated intrathecally with methotrexate, cytosine arabinoside and dexamethasone and afterword his general status improved. Conclusion: Ataxia telangiectasia (AT) is a rare multisystem, neurodegenerative genetic disorder. Due to its wide clinical heterogeneity, it often leads physicians to an incorrect or missed diagnosis, and insight into this rare disease is important. We present an adult patient with acute lymphoblastic leukemia and ataxia-telangiectasia. Risk of severe complications or death during the initial period of acute leukemia was markedly decreased. The incidence of leukostasis and tumor lysis syndrome depends on absolute initial white blood cell counts and the underlying type of leukemia. Leukapheresis may improve the prognosis of high risk patients. Leukapheresis or exchange transfusion together with conservative management and specific oncological therapy may contribute to rapid leukocyte reduction with acceptable risk.
Hyperbilirubinemia is a common clinical manifestation, and may be life threatening. Many diseases result in hyperbilirubinemias, some are refractory, and cannot be cured by medication or surgery. How to reduce the high concentration of bilirubin in the blood of patients has always been a focus of study for many researchers. We treated severe hyperbilirubinemia by performing plasma exchange. Plasma exchange not only removes bilirubin, endotoxin and complement activator, but also replenishes albumin, coagulation factor and hepatic regenerative stimulating substance, which can correct metabolic disorder. It is not easy to decide when to start plasma exchange, the recovery liver function is difficult to asses. The problems with plasma exchange are the difficulty in obtaining large amounts of fresh frozen plasma and its high cost. The survival rate of patients with hyperbilirubinemia might be improved with plasma exchange and timely management of complications. The implementation of plasma exchange should not be delayed when it is indicated, it can be a therapeutic choice in these patients. P-42 EXPERIENCE OF LIPID APHERESIS PROCEDURE OF MERSIN UNIVERSITY APHERESIS UNIT N. Tiftik1 , F. Koksal2 , T. Ozcan2 , A. Tombak1 , M.A. Ucar1 , S. Kizilalp1 , O. Hallioglu3 , A. Kiykim4 . 1 Mersin University Medical Faculty Internal Medicine Hematology Department, 2 Mersin University Medical Faculty Cardiology Department, 3 Mersin University Medical Faculty Pediatric Cardiology Department, 4 Mersin University Medical Faculty Nephrology Department, Turkey The patients with severe hyperlipidemia are sometimes resistant to diet and lipid-lowering drugs. Lipid apheresis is the therapeutic option for such patients. Here, we report our experience of lipid apheresis procedure of our apheresis unit. One pediatric patient with familial hypercholesterolemia was treated by direct adsorption of lipoproteins (DALI). Same pediatric patient and 7 adult patients with hypercholesterolemia and hypertriglyceridemia underwent cascade filtration. Twenty procedures with DALI and 20 procedures with cascade filtration were performed. In DALI, the mean of total cholesterol values before and after therapy were 475 and 154 mg/dL. The mean of low-density lipoprotein (LDL) cholesterol values before and after therapy were 422 and 114 mg/dL. In cascade filtration, the mean of total cholesterol values before and after therapy were 467 and 129 mg/dL. The mean of LDL cholesterol before and after therapy 353 and 75 mg/dL. Four patients with hypertriglyceridemia underwent cascade filtration and mean triglyceride values before and after therapy were 1314 mg/dL and 336 mg/dL, respectively. Allergic reaction was occured in 2 patients applied DALI, and heart block was occured in one patient applied cascade filtration. According to the experince of our apheresis unit, lipid apheresis is a therapeutic procedure in patients with high risk hypercholesterolemia and hypertriglyceridemia despite efficient medical therapy.
S41
P-43 THE CONTROL OF ACUTE LYMPHOBLASTIC LEUKAEMIA CAUSED HYPERLEUKOCYTOSIS WITH LEUKOPHERESIS ON AN ADULT ATAXIA-TELANGIECTASIA PATIENT M. Keklik1 , S. Sivgin1 , B.S. Kalin1 , G. Akyol1 , C. Pala1 , M. Solmaz1 , L. Kaynar1 , B. Eser1 , M. Cetin1 , A. Unal1 . 1 Erciyes University Division of Hematology, Kayseri, Turkey Introduction: Ataxia-telangiectasia (AT) is a hereditary disorder characterized by progressive neurological dysfunction, oculocutaneous telangiectasia, immunodeficiency, cancer susceptibility, and radiation sensitivity. The prognosis of the malignancies is impaired by the immunodeficiency and the susceptibility to ionising radiation and chemotherapeutics. Pediatric patients may develop lymphomas and acute lymphoblastic leukaemia (ALL), especially of the T-lineage. However the AT and ALL on adults are less common so we presented a case of hyperleukocytosis leukostasys to which we performed leukapheresis. Patient: 25 years old male patient, while followed for AT and mental retardation diagnoses, was admitted to the Emergency unit due to fatigue, nausea and headaches. Patient had a moderate general condition and cooperation was established. Fever was 37.5°C, Pulse: 120 bpm and blood presure: 100/70 mmHg. No lymphadenopathy, splenomegaly or hepatomegaly was observed to the patient. Hb: 13.6 g/dl. WBC: 466×103 /mL. Plt: 6×103 7 mL. Peripheral blood smear showed dominance of blasts. Peripheral blood results of flow cytometry showed T-ALL and BUN: 28 mg/dl. Creatinin: 0.7 mg/dl. AST: 58 u/L. ALT: 71 u/L. Results: 2 sessions of large volume leukapheresis was performed to the patient by the insertion of a central venous catheter at the Apheresis Unit. Fresh frozen plasma (FFP) was used as replacement fluid. After the procedure WBC value decreased to 170×103 /mL. There were no procedure-related adverse events. Symptoms due to hyperleukocytosis markedly improved after leukapheresis. In addition the chemotherapy protocol including parenteral steroid, vincristine, doxorubicin, methotrexate, folinic acid and L. asparaginase was performed to the patient. Patients also was treated intrathecally with methotrexate, cytosine arabinoside and dexamethasone and afterword his general status improved. Conclusion: Ataxia telangiectasia (AT) is a rare multisystem, neurodegenerative genetic disorder. Due to its wide clinical heterogeneity, it often leads physicians to an incorrect or missed diagnosis, and insight into this rare disease is important. We present an adult patient with acute lymphoblastic leukemia and ataxia-telangiectasia. Risk of severe complications or death during the initial period of acute leukemia was markedly decreased. The incidence of leukostasis and tumor lysis syndrome depends on absolute initial white blood cell counts and the underlying type of leukemia. Leukapheresis may improve the prognosis of high risk patients. Leukapheresis or exchange transfusion together with conservative management and specific oncological therapy may contribute to rapid leukocyte reduction with acceptable risk.