ISSN: 1300-7777 E-ISSN: 1308-5263
Turk J Hematol: 18 (3)
Volume: 18  Issue: 3 - 2001
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1.Activated Protein C Resistance in Polycythemia Vera
Alp Günay, Ahmet Öztürk, Tülin Budak, Uğur Özbek, Necdet Üskent
Pages 157 - 164
Activated protein C resistance is a result of a point mutation in factor V gene (Leiden mutation) and can be identified in approximately 50% of patients with thrombosis, making it an important risk factor for thrombosis. The aim of this study is to evaluate the role activated protein C resistance in the hypercoagulable state seen in polycythemia vera. We compared patients with polycythemia vera (n: 24) for increased risk of thromboembolism and activated protein C resistance, with the results of patients with chronic myelogenous leukemia (n: 27) and healthy control group (n: 52). Activated protein C resistance test and factor VIII activity was determined by an aPTT based test. Anticardiolipin antibodies IgG and IgM were also determined by ELISA. Leiden mutation was studied with polymerase chain reaction. Venous thromboses were observed in 12.5% and arterial thromboses in 41.6% of patients with polycythemia vera. Arterial thromboses were recognized in 7.4% of patients with chronic myelogenous leukemia. Activated protein C resistance was identified in 20.8% of patients with polycythemia vera and 14.8% with chronic myelogenous leukemia (versus 1.8% of healthy control subjects). The risk of thrombosis in patients with polycythemia vera was independent from the presence of activated protein C resistance. Leiden mutation was observed in only 1 patient out of 4 patients with chronic myelogenous leukemia who had activated protein C resistance, but not thrombosis. Factor VIII levels of patients with chronic myelogenous leukemia (158% ± 14) were higher than healthy control subjects (99% ± 15) (p< 0.05). Patients with activated protein C resistance in both groups had no seropositivity for anticardiolipin antibodies IgG and IgM. Activated protein C resistance and in some cases its association with Leiden mutation in polycythemia vera may not have a major role in the pathogenesis of thromboembolic complications of polycythemia vera.

2.Thrombolysis of Acute Arterial Occlusion with rt-PA
Mehmet Kurtoğlu, Volkan Granit, Ahmet Necefli, Metin Kurtoğlu, Recep Güloğlu
Pages 165 - 172
The use of thrombolytic agents to treat peripheral arterial occlusions is a new method. Despite its advantages, information about complications caused by the use of rt-PA and about its place in treatment is still incomplete. The aim of this study was to establish a dose range for rt-PA and to follow the patients with a protocol during and after thrombolysis. Between May 1999 to January 2000, 14 patients with symptoms of peripheral arterial occlusion came to Istanbul Medical Faculty Emergency Surgery Unit. The duration of ischaemia before their hospitalization took an average of 44 hours. (Range 3 hours-7 days). A pulse-spray catheter was directed to the thrombus under angiographic control. Bolus injection of 5 mg of rt-PA was followed by 15 minutes of interval. The extent of thrombolysis was checked by angiography and then bolus injection of 5 mg of rt- PA was repeated. After angiographic control, patients having insufficient thrombolysis, received 0.05 mg/kg/hour of infusion for 12 hours. At the end of 12 hours, thrombolytic treatment ended with a control angiography. A thromboembolectomy operation was made to patients still having an occlusion after thrombolysis. To avoid re-occlusions, all of the patients received 1.5 mg/kg/day low molecular weight heparin (enoxaparin) for 1 week. At the end of thrombolysis, 9 patients had complete lysis. A patient, having an occlusion in superior mesenteric artery had 60% recanalisation. 2 patients (14%) having 90% stenosis, needed a balloon angioplasty besides thrombolysis, and both of them had complete reperfusion. 2 patients (14%) needed a thromboembolectomy operation due to insufficient thrombolysis. 2 patients (14%) had a minor bleeding after thrombolytic treatment. After thrombolysis, 2 patients (14%) had a stroke. There were no amputations. 1 of the patients having a stroke, died 2 days after thrombolytic treatment 1 patient died due to myocardial infarction during thrombolysis. 1 patient (7%) died due to diabetic coma on the 20th day. Acute myocardial infarction was the cause of death in 1 patient on the 25th day. In conclusion pulse spray thrombolysis with rt-PA is safe and efficient. Moreover there is a reduction in complications and need for surgical procedure. The recent problem is to find the optimum dosages for the best thrombolysis and for least complications.

3.Defective Function of Peripheral Blood Monocytes from Patients with Non-Hodgkin’s Lymphoma
G. Abdul Hamid, H. Wolf, S. Albrecht
Pages 173 - 178
The aim of the study was to analyze the function of peripheral blood monocytes from patients with Non-Hodgkin’s lymphoma before and after incubation with zymosan and indomethacin. Peripheral blood samples were collected from 28 patients with malignant lymphoma (13 males and 15 females with age range 20-65) years. Their clinical record and pathologic material were reviewed. The control group consisted of 17 normal subjects, (9 men and 8 women) of age range of 20-45 years. The following investigations were carried out in all patients: Bactericidal activity against Escherichia coli. Level of superoxide anion and Chemiluminescence’s Technique for analysis of oxygen metabolite Results. The mean bactericidal indices of E. coli by peripheral blood monocytes without indomethacin were 56.75 (SD ± 10.5) in control group at 60 minutes and it was 36.88% (SD ± 11.3) in Non-Hodgkin’s lymphoma patients. The level of INT was greater in healthy control than NHL-patients. The improvement after addition of zymosan was significant in all groups. The peak generation of chemiluminescence in Non-Hodgkin lymphoma patients was 11256 x 10-3 CPM at 20 minutes and in healthy controls 16575 x 10-3 CPM at 5 minutes and after incubation with zymosan and indomethacin were 13843 x 10-3 CPM at 5 minutes in NHL patients and 16312 x 10-3 CPM in healthy controls. The time of appearance of CL peak improved in Non-Hodgkin’s lymphoma patients (p< 0.01) but there is no difference in the time of CL peak of the healthy controls.

4.Oral Health Status in Children with Acute Lymphoblastic Leukemia and Lymphoma
Cem Doğan, Cenk Haytaç, Bülent Antmen, İlgen Şaşmaz, Atila Tanyeli
Pages 179 - 183
We evaluated the oral health status of 85 acute lymphoblastic leukemia (ALL)/lymphoma pediatric patients who received remission-induction and maintenance chemotherapy and 85 age and sex-matched healthy children with the criteria of World Health Organization (WHO) and to determine the prevalence and distribution of dental problems in order to constitute preventive dentistry precautions in this study. The gingival tissues were scored with Community Index of Periodontal Treatment Necessity (CPITN) and dmf-t and DMF-T indices were used for caries evaluation. In the study group, malocclusion was found in 24 patients (28.2%). CPITN was scored as follows in the study group; 11% of the patients had healthy gingiva (Grade 0), the presence of plaque (Grade I) 79% of the patients, the presence of calculus (Grade II) 10% of patients were observed. Nevertheless, mucositis was found with various grades in 9 patients who received chemotherapy. Decayed teeth were found in the 76 patients and in 45 healthy children. 91.7% of patients and 52.9% of children needed dental treatment were determined. The DMF-T and dmf-t scores showed that ALL/lymphoma patients had more decayed and needed more dental treatment, missing or filled teeth both in their deciduous (p< 0.001) and permanent (p< 0.05) dentition when compared to systemically healthy children.

5.Relationship Between Leptin Levels and Body Indexes in Patients with Haematologic Malignancy
Rafet Mete, İmdat Dilek, Ekrem Algün, Reha Erkoç, Hayati Ayakta, Cevat Topal, Haluk Dülger, Halis Aksoy
Pages 185 - 189
Leptin is a recently found hormone regulating body weight. In human obesity, this weight-regulating hormone level is in a positive correlation with FMI (fat mass index) and BMI (body mass index). In this study, we aimed to investigate the relation between serum leptin levels and BMI, PF (percentage fat), LMI (lean mass ındex), FMI and some other parameters of patients with haematologic malignant diseases. Fourty-four patients with haematologic malignant diseases and 25 healthy control group were taken into the study. In the comparison, there were no significant difference between the PF and FMI values of both groups, while the mean BMI and LMI values of the control group were significantly higher than that of the patient group. There was a positive correlation between leptin levels and BMI and FMI among parameters studied in our control group, whereas we couldn’t demostrate any such correlation in patient group. We estimate that the alteration may be due to disturbances in the feed back mechanism developing in patient with haematologic malignancy.

6.Bernard-Soulier Syndrome Like Platelet Defect in a Patient with Noonan Syndrome; A Case Report
Ahmet Koç, Mustafa Kösecik, M. Mansur Tatlı, Ali Atas, H. Haldun Emiroğlu
Pages 191 - 193
Noonan’s Syndrome (NS) is characterized by dismorphic facial features, short stature, short or webbed neck, congenital heart defects and testicular abnormalities. Various bleeding disorders in Noonan Syndrome have been reported. Bernard-Soulier Syndrome (BSS) is a rare congenital bleeding disorder characterized by thrombocytopenia and giant platelets. There is not any reported case of Noonan syndrome associated with BSS in literature. We report here a four-year-old male patient with Noonan Syndrome and BSS like platelet defect.

7.Homozygous ß-Thalassemia Associated with Familial Mediterranean Fever in a Turkish Patient
Duran Canatan, Rüya Coşan, Hakkı Taştan, Onur Bilenoğlu, A. Nazlı Başak
Pages 195 - 197
We report here a ß- thalassemia major case (homozygous IVS-1-110 G-A) associated with Familial Mediterranean Fever (FMF) (homozygous 694 Met-Val). Our patient’s clinical course revealed a possible synergistic effect between colchicine and desferrioxamine (DFO) However, this could be a only a coincidence, as under colchicine therapy, fever attacks may appear, this may be the topic of a further investigation.

8.Hemoglobin LeporeBoston in a Turkish Family
Münci Yağcı, Zübeyde Nur Özkurt, Gülsan Türköz Sucak, Ece Akar, Nejat Akar, Rauf Haznedar
Pages 199 - 201
An abnormal hemoglobin was detected in a Balkan immigrant Turkish family. Erythrocyte morphology was similar to ß-thalassemia trait. Molecular analysis showed that the abnormal hemoglobin was Hemoglobin LeporeBoston. All affected family members were in heterozygote state and asymptomatic.

9.A Case with Micromegakaryocytes
Tiraje Celkan, Hilmi Apak, Alp Özkan, Hilal Akı, Mustafa Taşyüreklı, Lebriz Yüksel, İnci Yıldız
Pages 203 - 206
A boy with no previous history of bleeding presented with ecchymoses and splenomegaly. He was followed up for thrombocytopenia and micromegakaryocytes for 20 months till clinically malignancy was diagnosed. Micromegakaryocytes must always be treated with suspicion, as they may provide an important clue for dyshematopoesis. Key Words: Micromegakaryocytes, Leukemia, Dismegakaryopoesis.

10.Consecutive IL-2 and IFN-a2a Maintenance in a Patient with Acute Non-lymphoblastic Leukemia and Renal Cell Cancer
Akın Uysal, Muhit Özcan, Celalettin Üstün, Semin Fenkçi
Pages 207 - 210
Over the last few years, the use of ınterleukin-2 (IL-2) and lymphokine-activated killer (LAK) cells have been found to be effective in the treatment of some solid tumours and acute myeloid leukemia. Our patient was initially diagnosed as having bilateral synchronous renal cell cancer (RCC) and underwent nephrectomy. Approximately two years after the operation he developed leukopenia without any sign of residual renal cell cancer. Bone marrow examination revealed acute myeloblastic leukemia (AML). IL-2 following IFN-a2a was used as a maintenance therapy after a standard remission induction and a consolidation therapy. Our patient has been still disease free for 58 months after the diagnosis of AML and 71 months after the diagnosis renal cell cancer. Review of the literature showed that this is the first case who has both RCC and AML and was treated successfully with IL-2 and IFN-a2a.

11.Images in Haematology
Bülent Eser, Ali Ünal, Işın Soyuer, Özlem Er
Page 211
Abstract | Full Text PDF

12.Chronic Myeloid Leukemia Associated with Ebstein’s Anomaly
K. Pavithran, G. Girija, M. Thomas
Page 215
Abstract | Full Text PDF

13.Idiopathic Hypereosinophilic Syndrome with Hepatic Tumoral Lesions
Carlos R. De Medeiros, Christiana G. Moreira
Page 217
Abstract | Full Text PDF


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