ALBERT

Beschreibung: Purpose The study is conducted in 29 sickle cell anemia (SCA) patients, who are in ischemic attacks (in vasoocclusive crises period) and in steady state; the level of T helper cells, cytotoxic T cells and natural killer cells were determined and the effect of ischemic attack on clinical prognosis and the immune functions. Materials and Methods In the study, 29 patients with sickle cell anemia in ischemic attacks with painful crises and in steady state and 24 healthy children were chosen in the same age group for control group. These groups were examined with complete blood cells count, Hb electrophoreses and the blood biochemistry. The study was performed by flowcytometric method to find the level of CD3 monoclonal antibody for total T lymphocyte, CD4 monoclonal antibody for T helper cells, CD8 monoclonal antibody for cytotoxic T cells and CD16+56 monoclonal antibodies for natural killer cells in the mentioned groups to compare the statistical data. Findings The average of HbS in ischemic periods in SCA patients are to be found 83±6,6 %. There were decrease in hemoglobin and hematocrit levels in SCA versus control group (p0,05). Results As a result of the study, in the patients with sickle cell anemia whom associated with chronic hemolysis and tissue hypoxia during the ischemic attack, the levels of total T lymphocyte (CD3) was found significantly lower than the control groupp

Beschreibung: Introduction Mutations in JAK2 have been implicated in polycythemia vera (PV), essential thrombocythemia (ES), primary myelofibrosis (PMF) as wll as other myeloproliferative disorders. Aim In this study we aimed to investigate the frequency of JAK2V617F mutation on 216 patients with hematologic malignancies in childhood and 176 patients with myeloproliferative disorders in adulthood. Materials and method Patient group consist of 164 ALL and 52 AML patients in childhood and 79 PV, 51 ES, 22 chronic myeloid leukemia patients (CML) and 24 PMF patients in adulthood. These patients followed by Cukurova University, Departments of Pediatric and Adult Hematology, are included in this study. Blood samples were collected in these patients group and DNA was isolated using high pure template preparation kit (Roche) and stored -80oC. Gene mutations were studied using TMB (TıbMolBiol) LightMix Kit JAK2V617F genomic and analyzed by Light Cycler 2.0 Roche Diagnostic, GmBh, Germany in both groups. Findings JAK2V617F mutation was found 1 of 164 ALL patients (0,6%), 0 of 52 AML patients (0%) in childhood. Nevertheless, JAK2V617F mutation was also found 71 of 79 PV patients (89,8%), 22 of 51 ET patients (43,1%), 1 of 22 CML patients (4,5%) and 15 of 24 PMF patients (62,5%) in adulthood. Result As a result we found high frequency of JAK2V617F mutation in PV patients than the other myeloproliferative disorders. JAK2V617F mutation was significantly high in myeloproliferative disorders in adulthood comparing with childhood acute leukemias (p

Beschreibung: Introduction Sickle cell anemia is a disease which is characterized with hemolytic anemia, hypercoagulopathy and painful crisis. Microparticles are 0,1-1 µm sized little membrane particles which are derived during activation or apoptotic phase of cell cycle. It is reported that microparticles are increased in many systemic disease including sickle cell anemia. Aim In this study we aimed to investigate the role of microparticles on during crisis and non-crisis periods in sickle cell anemia patients. Materials and method Twenty nine patients, following by Cukurova University, Department of Pediatric Hematology, are included in this study. Blood samples were collected in 26 of these patients in non-crisis period. Control group formed with 18 healthy children without any systemic disease. Complete blood count, hemoglobin electrophoresis and biochemical parameters were studied in both groups. Also patients’ total microparticle levels, erythrocyte (CD235a), endothelial (CD106), monocyte (CD14) particle levels and tissue factor expressing (CD142) microparticle levels were studied by flow cytometry and whole data was statistically analyzed. Findings Hemoglobin and hematocrit levels were significantly low in sickle cell anemia patients (p

Beschreibung: Abstract 4628 Aim In this study we evaluated to pulmonary functions and determined relations of these findings with clinical parameters in children with sickle cell anemia (SCA) who were at follow up in our pediatric heamatology clinic. Materials and Methods 24 children with sickle cell anemia and 9 healthy children as control group include to the study. Complete blood count, hemoglobin electrophoresis and biochemical values were eveluated for both groups. At pulmonology department, the carbonmonoxide diffusion test performing for both groups. At the same day spirometric respiratory function evaluation and exercise test performed both groups at department of sports physiology. The data recieved are compared statistics. Results HbS, HbF, SGPT, ferritin, total bilirubine, direkt bilirubine and Fe++ values were high at patient group (p

Beschreibung: Abstract 4824 Micro-RNAs are functional, non-protein coding RNA molecules and their transcriptions provided by intron or exon regions of the genome and non-protein coding regions of RNA genes. The role of micro-RNAs in acute leukemia has become the subject of research increasingly. In this study we aimed to identify micro-RNA profiles in the childhood acute leukemia that one of hematologic malignancies. Fourty nine patients who were diagnosed with acute leukemia and admitted to Cukurova University Faculty of Medicine Department of Pediatric Hematology between December 2010 and September 2011. Blood samples were taken twice in patient groups at diagnosis and during remission and plasma samples were stored. Blood samples were taken once in the healthy group and plasma were separated. The plasma samples were investigated by PCR analysis of micro-RNA. Acute leukemia was diagnosed by cytomorphological, immuno histochemical and flow cytometric studies. Thirtyone patients who were diagnosed with ALL and fortyseven healthy children as a control group were included to study. miR20a, miR25, miR92a, miR30c, miR106b, miR203, miR150, miR192, miR302c, miR184, miR218, miR320, miR342-3p, miR223, miR328, miR483-5p, miR376a, miR381, miR451, miR576-3p, miR548a levels were increased in newly diagnosed ALL patients when compared to healthy controls (p

Beschreibung: Abstract 4191 PURPOSE: Erythrocyte apheresis is an effective therapy in the acute and chronic treatment of sickle cell anemia (SCA). The main goal of erythrocyte apheresis is to improve blood perfusion through reducing Hemoglobin S (HbS) to 〈 %30 and keeping hemoglobin (Hb) at desired level. The aim of this study was to evaluate the effect of the erythrocyte apheresis on immunological parameters in SCA patients with crisis. METHODS Between February 2009 and June 2009, 37 patients (20 female / 17 male) received 44 apheresis treatments at Hemapheresis Unit of Cukurova University School of Medicine, Adana, Turkey. The median age was 22 (Range, 4-56) years and the mean body weight was 51.9±18.6 kilograms. The apheresis procedures were carried out using a Cobe Spectra Cell Separator. A mean of 1.77 body volume calculated red blood cell volumes were exchanged with a mean duration of 125.9±34.9 minutes (Table 1). Sickling negative and leukoreduced packed red cells were used for apheresis treatments. Hemoglobin electrophoreses, complete blood counts, immunoglobins, specific surface markers for T, B and natural killer (NK) cells were performed before and after each procedure. While three patients had a SCA-induced functional asplenia, none of the patients was HIV-seropositive. RESULTS The erythrocyte apheresis resulted in a decrease on white blood cell (WBC) counts as expected. Average pre and post apheresis WBC counts were 12883±5775/mm3 and 8506±3331/mm3 (p

Beschreibung: Objective: Coagulopathy resulting from liver pathologies and the hemorrhages secondary to this are the complications observed in chronic liver diseases, fulminant hepatitis, hepatocellular cancer, liver transplantation and other similar conditions. The literature on the use of rFVIIa which was initially used in hemophiliac patients with inhibitors for hemorrhages that can not be managed with conventional methods or operations that can not be performed safely, is increasingly growing. Here we will present a patient that was successfully operated by using rFVIIa for recurrent intracranial hemorrhage secondary to liver disease. Case: The 7 months old female infant was brought to our clinic due to abdominal distention and bleeding from anus. Her medical history included a treatment by hospitalization in the Regional Hospital when two months old due to high fever, forceful vomiting, foaming at mouth and yellow coloring of skin (convulsion?), operation due to hemorrhage detected at cerebral imaging, two subsequent operations at the same hospital due to hemorrhage upon sudden purple coloring of skin observed 1.5 months later and finally, dismission from hospital following a 25-day monitoring under conditions of intensive care. Following the yellow coloring of eyes, hematemesis, abdominal distention, echhymoses in the back and chest observed in the patient, she was referred to our clinic and her cerebral CT findings revealed pressure effect and subdural hematoma extending from temporal localization to frontoparietal cranium at left and to tentorium superior section and anterior interhemispheric fissure in cranium at posterior, with the widest part reaching 1.5 cm. The patient, for whom an operation was planned, was twice supplemented with platelet suspension at a dose of 10 cc/kg (53000/mm3) for her thrombocytopenia. Despite the administration of Frozen Plasma supplement, PT, PTT and Fibrinogen was detected as 18, 42.8 and 117 mg/dl, respectively and 1.2 mg of Novoseven was administered to the patient once preoperatively and twice postoperatively at a two-hour interval due to her history of postoperative hemorrhage. Upon the preoperative detection of PT, PTT, Fibrinogen as 12.1, 43.1 and 145 mg/dl and KZ and PZ as 6 and 5 minutes respectively, the patient was operated. No new postoperative hemorrhages or other complications were observed. The diagnosis of Gaucher disease was made at the bone marrow examination and the relevant treatment was planned. Conclusion: rFVIIa can be safely used in high-risk patients with a history of recurrent hemorrhage, for whom no improvement can be achieved in the hemostasis tests.

Beschreibung: Objective: Platelet transfusion is used for the treatment of acute hemorrhages in diseases other than immunological events, which lead to severe thrombocytopenia. Besides the diseases in which production of platelets is completely terminated in the bone marrow and the platelet requirement is increased, immune thrombocytopenia and alloimmunization may also result in not being able to achieve the desired platelet levels with platelet transfusion, and life threatening hemorrhages. rFVIIa which is used for the management of life threatening hemorrhages in hemophiliac patients with inhibitors and in cases of acquired coagulopathies has also been used successfully in patients with thrombocytopenia. Here we present a case with a diagnosis of kala-azar, where nasal hemorrhage couldn’t be managed with conventional methods and required continuous replacement, and finally treated successfully by using rFVIIa. Case: At admittance to hospital, hepatosplenomegaly and pancytopenia were detected in the fifteen year old male patient. He was diagnosed with kala-azar upon the detection of Leishmania donovani amastigote form in the examination for bone marrow aspiration. Treatment with sodium stibogluconate was initiated (60 mg/kg). Replacements were performed in the patient due to thrombocytopenia and anemia, who developed nasal hemorrhage during follow-up and had anterior nasal cushion placed. The patient was supplemented with fresh frozen plasma, vitamin K and fibrinogen for his acquired coagulopathy. Since coagulopathy wasn’t corrected and nasal hemorrhage continued actively, 5-minute infusions of rFVIIa were administered to the patient at a dose of 4.8 mg (120 microgram/dl) three times every two hours. His hemorrhage was stopped by rFVIIa treatment. Conclusion: Our case demonstrated that use of rFVIIa for the management of hemorrhage in thrombocytopenic patients is also feasible in cases other than coagulopathy and that it may be an option for patients with hemorrhages that can not be managed with intensive platelet and plasma transfusions. This result shows that rFVIIa can be used effectively in other diseases other than hematological disorders that cause hemorrhage due to coagulopathy and thrombocytopenia.

Beschreibung: Objective: Prothrombin complex concentrates (PCC), active prothrombin complex concentrates (APCC;FEIBA) or recombinant Factor VIIa (rFVIIa; Novoseven) are safely used for the treatment of hemorrhages in hemophiliac patients with inhibitors or during the interventions performed in these patients. A hemophilia-A patient with inhibitors in whom bilateral cataract operation and circumcision were successfully performed by rFVIIa administration was presented. Case: The 13 year old male patient had been diagnosed with severe Hemophilia-A by the detection of a FVIII level below 1% in the factor assay performed due to echhymoses in his body when he was 6 months old. The inhibitor level of the patient who developed bilateral cataract during the follow-up period was determined as 2.95 BU; however the cataract operation had to be delayed due to the absence of an adequate increase in his factor VIII level despite the 2000 U factor replacement administered in July 2002. The patient was readmitted to the hospital for operation in October 2002. However, the operation was delayed again since a safe level of factor VIII wasn’t achieved with Factor VIII replacement. The patient was excluded from follow-up for approximately 2 years. In August 2004, the patient was hospitalized due to echhymosis in the scrotum, and his factor VIII level was measured as 1% and the VIII inhibitor level as 88 BU. IV steroid treatment was initiated in the patient at a dose of 2 mg/kg, with the factor VIII replacement intended at a level of 100 U/kg. 2 doses of 90 microgram/kg of rFVIIa were administered at a two-hour interval due to catheterization for plasmapheresis and subsequently, plasmapheresis program was carried out. The inhibitor level was measured as 25.6 BU after the first plasmapheresis. Factor VIII level of the patient whose FVIII inhibitor level declined to 15.4 BU with these treatments, didn’t exceed 3%. 120 microgram/kg rFVIIa was administered to the patient prior to operation and maintained during the 1st and 2nd days of the operation every 2–3 hours at the same dose. At this operation, right eye small incision cataract extraction and posterior chamber intraocular lens implantation were performed under general anesthesia. A month later, cataract operation was performed in the other eye together with the circumcision operation. A 3-grade increase was achieved in visual acuity of both eyes. Conclusion: Here, the cataract operation together with the circumcision operation performed in a hemophiliac patient with inhibitors was presented, demonstrating that rFVIIa which has been started to be used also in other fields other than hemophilia patients, can be used safely in operations.

Beschreibung: Abstract 4724 Aim Abnormalities and alterations in apoptosis mechanism may lead to cancer development. Cystean proteases enzymes, called caspases, appear to be involved in both the initial signaling events. There are many proteins that trigger intrinsic and extrinsic pathway and induce apoptosis signals. Fas and its specific ligand that known as Fas Ligand are the best defined dead receptors and have functions in apoptosis regulation with many tumor types. Fas binds the ligand on the cytotoxic T cells and start apoptosis. Objectives of this study were to determine serum levels of Fas and Fas Ligand at the time of diagnosis in childhood acute leukemias that may be play important role in apoptosis mechanism. Patients and Methods In this study, we investigated serum Fas and Fas Ligand levels by using ELISA method in childhood acute leukemias. Twenty-nine cases with acute lymphoblastic leukemia and twenty-three cases with acute myeloblastic leukemia at the ages of 1-18 years are included this study. The age distrubition of the control group varied 1-15 years consisted of twenty-seven children. We investigated serum Fas and Fas Ligand levels at the time of diagnosis from peripheral blood samples. Results The comparison of the mean values of Fas and Fas Ligand levels in acute leukemia patients groups and control group have shown important difference as statistically (p0,05). The comparison of the Fas levels in ALL patients according to immunophenotypes; CALLA(+) B-ALL have higher mean level than T-ALL and shown important difference as statistically (p