E-ISSN: 1308-5263
Turk J Hematol: 18 (2)
Volume: 18  Issue: 2 - 2001
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1.Screening of Hemoglobinopathies in Kahramanmaraş, TURKEY
Güneş T. Yüregir, Metin Kılınç, Hasan Ekerbiçer, Neşet Bilaloğlu, Nermin Tekin
Pages 79 - 83
Thalassemia and sickle cell anemia are prevalent in southern Turkey. Being in close proximity to Çukurova we screened Kahramanmaraş to assess the prevalence and foci of the diseases. The sample sizes were calculated by EpiInfo 6.0 computer program at 95% confidence level. 1491 subjects aged 2-69 were studied. Hematological parameters were analyzed by an electronic cell counter. Electrophoresis were performed and Hemoglobin A2 and hemoglobin F levels were determined on samples with MCV< 80 fL. The results of Canatan et al. on Elbistan were included in the final results. Thus, the prevalence of ß-thalassemia, hemoglobin D, Hemoglobin O Arab carriers were 0.68%, 0.28% and 0.013%, respectively. No hemoglobin S was detected. In conclusion, Kahramanmaraş seems not to be a high risk area but the public must be informed about these diseases. Every community in close proximity to high prevalence areas must be enlightened.

2.Prevalence of Beta Thalassemia Trait in Denizli
Zahit Bolaman, Yaşar Enli, Mehmet Köseoğlu, Hasan Koyuncu, Diler Aslan
Pages 85 - 88
Beta thalassemia, characterized by the deficiency or the absence of beta globulin production, is the most widespread inherited disorder in the world and is also common in Turkey. To determine the prevalence of carriers for beta thalassemia, we screened the couples before their marriage. For this aim, from 1994 to 1999, a total of 14.200 people were screened. The complete blood count and red blood cell indices (hemoglobin: Hb, hematocrite: Hct, median corpusculer volume: MCV, median corpusculer hemoglobin: MCHb, median corpusculer hemoglobin concentration: MHbC, concentration were measured by automated cell counter on the same day of collection. Then for the samples with MCV values of 78 fL or below, hemoglobin electrophoresis were employed. Testing for beta thalassemia was carried out by the conventional cellulose asetate electropheresis
at pH 8.4. People who have elevated HbA2 (≥ 3.5%) were accepted as beta-thalassemia carrier or patient. We detected 3300 people with MCV levels of 78 fL or below and 311 cases of beta thalassemia carrier and 11 cases of beta thalassemia. The prevalence of carriers for beta thalassemia in Denizli was 2.2%. This result indicated that the people with anemia in our region should be investigated for the existence of hemoglobinopathy.

3.Rapid Identification of Family-Specific Mutations in the Factor VIII Gene by One-Step DGGE
Ayşe Anıl Timur, Davut Albayrak, S. Hande Çağlayan
Pages 89 - 93
A one-step denaturing gradient gel electrophoresis (DGGE) strategy for the rapid detection of mutations in the factor VIII gene of haemophilia A patients is described. All coding (except the middle part of exon 14) and flanking intronic regions of the gene corresponding to approximately 6.6 kb were amplified in 27 fragments using four PCR programs. Heteroduplex formation was performed for each fragment. A common denaturant gradient gel (35-65%) was chosen that allowed the simultaneous analysis of all PCR amplified regions on a single gel and run for 3.5 h at 160 V. This method was implemented for a patient whose family was seeking carrier determinations. An abnormal pattern was detected in exon 23 and the family-specific mutation was found by subsequent DNA sequencing. One-step DGGE is a promising rapid method for the carrier detection and prenatal diagnosis in haemophilia A families when immediate results are required and when polymorphic markers fail to give information.

4.The Hemostatic Changes in Active Pulmonary Tuberculosis
Muzaffer Sezer, Ahmet Öztürk, Ahmet İlvan, Mehmet Özkan, Necdet Üskent
Pages 95 - 100
Severe pulmonary tuberculosis (PTB) is sometimes complicated with deep venous thrombosis (DVT). We have examined the role of possible hemostat›c disturbance, which are predisposing factors for venous thrombosis in patients with PTB. Coagulation and platelet function tests have been studied in 40 patients with severe PTB and 40 healthy control volunteers before therapy and they were compared with 30th day results and
controls. Analysis in patients with active PTB showed anemia, leucocytosis, thrombocytosis, elevation in plasma fibrinogen, factor VIII, plasminogen activator inhibitor 1 (PAI-1) with depressed antithrombin III (ATIII) and protein C (PC) levels. On the 30th day of treatment, anemia, leucocytosis and thrombocytsis were improved. Fibrinogen and factor VIII levels decreased to normal levels, PC and AT III levels increased to normal levels whereas there was no difference in PAI-1 levels. Platelet aggregation studies demonstrated increased platelet activation. Activated protein C resistance was not determined. DVT was not detected in patients during the follow up period. Decreased AT III, PC and elevated plasma fibrinogen levels and increased platelet aggregation appear to induce hypercoagilable state seen in PTB and improves with tretament.

5.Long Term Low Dose Maintenance Chemotherapy in the Treatment of Acute Myeloid Leukemia
Murat Tombuloğlu, Seçkin Çağırgan
Pages 101 - 105
In this study the effect of low dose long-term maintenance chemotherapy in patients with acute myeloid leukemia (AML) was evaluated. Following a complete remission two consolidation courses were given with the same drugs. Thereafter patients received low dose maintenance chemotherapy in every four weeks until disease relapsed or for up to two years. A total of 68 patients were evaluated. The median duration of remission of 22.5 months in patients who received maintenance chemotherapy while it was only 7 months in those without maintenance chemotherapy after a median follow-up time of 71 months, which was significant. Overall survival (OS) was also significantly longer in patients with maintenance therapy. Similar results were also obtained in comparison of patients over 60. Thus, it was concluded that maintenance therapy might be beneficial
for older AML patients with limited therapy choice.

6.Clinical, Immunophenotypic and Cytogenetic Features of Megakaryocytic Blast Crisis of Chronic Myeloid Leukemia: A Single Institution Study
Vladimir Lazarevıc, Gradimir Jankovıc, Nada Kraguljac, Vesna Djordjevıc, Predrag Mıljıc, Marina Bogunovıc, Zoran Rajıc, Vesna Cemerıkıc-martınovıc, Nada Suvajdzıc, Milica Colovıc
Pages 107 - 115
We present 15 patients with megakaryocytic (Mk) blast crisis (BC) of a Philadelphia (Ph) chromosome positive CML confirmed by immunophenotype analysis between 1989-2000. The primary aim of this study is to define clinical, immunological, cytogenetic and laboratory characteristics of Mk BC in Ph positive CML. We have done retrospective analysis regarding basic clinical findings, immunologic phenotype, cytogenetic studies
and platelet functions. All patients had significant expression of CD61 (14/14) and CD34 (13/13) antigens, and a high frequency of expression of CD13 (9/12), CD33 (10/12) and CD11b (9/11). The BC in 6/15 patients was presented with thrombocytosis, 7/15 had a normal platelet count and two patients had thrombocytopenia. A grade IV myelofibrosis was present in 8/10 patients. Six patients evolved additional karyotypic abnormalities.
Two patients had extramedullary BC. The serum activity of LDH (med. 1095.6) was elevated in all patients. A platelet dysfunction was documented in 4/5 patient tested. There are no clinical and hematological characteristics specific for Mk BC of CML. Normal or elevated platelet count (med. 427.4 x 109/L) in BC of CML with prominent expression of CD34 and CD61 antigens, and significant myelofibrosis (grade IV) are the most consistent clinical findings.

7.Hodgkin’s Disease: Results of a Single Center in Central Anatolia
H. Şenol Coşkun, Bülent Eser, Mustafa Çetin, Özlem Er, Ali Ünal, Mustafa Altınbaş, Eray Karahacıoğlu, Bünyamin Kaplan
Pages 117 - 122
Hodgkin’s Disease (HD) accounts for about 1% of newly diagnosed malignant diseases. In this study 119 HD cases followed in Erciyes University Hospital were evaluated. 67.2% of the patients was male, 32.8% female. The patients’ age ranged from 15 to 72 years with a median of 41.5 years. Of the patients 10.1% was stage I, 29.4% stage II, 39.5% stage III, and 21.0% stage IV. According to Rye classification frequency of histologic
subtypes was as follows; 21.0% lymphocyte predominant, 25.2% nodular sclerosis, 43.7% mixed cellularity, and 10.1% lymphocyte depletion. Combination chemotherapy consisting cyclophosphamide, vincristine, procarbazine and prednisolone (COPP) was used as first line treatment in 59.7% of patients. Complete remission was achieved in 84.9% of patients and partial remission in 5.0% of patients; response could not be obtained in remaining 10.1% of patients. Disease progression or recurrence was observed in 30.2% of patients. Five year survival rate was found as 70.8% of all patients, 90.1% for stage I-II, 55.3% for stage III-IV patients (p= 0.03).

8.Epstein-Barr Virus Latent Membrane Protein 1 (LMP-1) in Hodgkin’s Lymphoma Patients in Turkey
Ömür Ataoğlu, Turgay Fen, Özlem Süer, Mehmet Dağlı, Zeynep Akı, Kadri Yamaç
Pages 123 - 126
Epstein-Barr virus (EBV) has been implicated as a contributing factor in the development of Hodgkin’s lymphoma. The aim of this study was to elucidate the association of Hodgkin’s lymphoma with EBV in a Turkish population using immunohistochemical detection of LMP-1. We studied a total of 21 consecutive cases of Hodgkin’s lymphoma from Turkey. LMP-1 protein was detected in 9 of 21 (42.8%) cases. LMP-1 was positive
in 4 of 7 (57%) mixed cellularity and 5 of 13 (38.4%) nodular sclerosis subtype. The results of the current study suggests a strong association of Epstein-Barr virus with Hodgkin’s lymphoma in Turkey and, together with those reported previously showed that Epstein-Barr virus correlated with mixed cellular type, with a slight male predominancy while there was no correlation with age.

9.Myelotoxicity of Cyclophosphamide, Methotrexate and 5-fluorouracil Regimen in the Early Stage Breast Cancer Patients with Diabetes Mellitus
Hasan Üstün, Sabri Barutca, Esin Şanlı, Mine Çabuk, Mehmet Alakavuklar
Pages 127 - 130
CMF (cyclophosphamide, methotrexate and 5-fluorouracil) is one of the most commonly used chemotherapy (CT) regimens in breast cancer. To the best of our knowledge there are no published studies on the toxicity of this regimen in the existence of diabetes mellitus (DM), in the literature. We retrospectively analyzed the myelotoxicity of CMF CT after 40 adjuvant cycles of 18 diabetics, according to WHO toxicity scala. Leucopenia/
granulocytopenia was the most prominent toxicity (observed in overall 30% of the cycles), but it was relatively mild (5% grade III and 2.5% grade IV granulocytopenia). Anemia was only grade I (10% of the cycles), and there was no trombocytopenia. Two of the cases with grade III and IV granulocytopenia, had grade I and II urinary tract infections respectively, following the CT. The case with grade IV granulocytopenia and infection had received G-CSF. We conclude that CMF regimen is tolerable in DM as regard to its myelotoxicity. However, the patients should be closely monitored as infections may easily arise in parallel to deepening leucopenia in DM. Further extended studies would be appropriate on the toxicity of CMF as well as the other common CT regimens in DM.

10.The Frequency of Pneumocystis carinii in Patients with Haematologic Malignancies and Pneumonia
Ayşen Timurağaoğlu İrfanoğlu, Dilara Öğünç, Gözde Öngüt, Candan Öğüş, Evren Tercan, Tümer Vural
Pages 131 - 136
Pneumocystis carinii (P. carinii) is an organism which was previously considered as a protozoan but recently it was shown to be more related to fungi. P. carinii increasingly causes opportunistic infections in immunocompromised patients. In this study, we detected P. carinii oocysts by indirect immunofluorescence test in 33 specimens obtained from 31 patients with haematological malignancies who had symptoms of pneumonia
and investigated probable risk factors (corticosteroid usage, neutropenia duration, severe or mild neutropenia and type of haematological malignancy) for P. carinii pneumonia in P. carinii (+) patients. Although not statistically significant, PCP incidence was higher in relapsed acute leukemia (AL) patients (62.5%), patients with prolonged neutropenia (57.1%), and who received high dose ARA-C therapy (62.5%). P. carinii (+) patients
were treated with trimethoprim-sulfamethoxazole. Six patients with PCP did not respond to therapy and died (50%). In conclusion PCP is not infrequent in AL (especially relapsed AL) and, indirectly we can suggest that chemoprophylaxis may be considered for these patients when they were in severe and prolonged neutropenia after high dose ARA-C therapy.

11.Homozygous ß-Thalassemia (FCS8-AA) and Hereditary Spherocytosis in the Same Patient
Zümrüt L. Uysal, Nejat Akar, Şükrü Cin, Filiz Ekici, Nazlı Başak
Pages 137 - 141
A three-year old Turkish girl having both homozygous ß-thalassemia and hereditary spherocytosis and her family have been studied. The molecular defect causing thalassemia in the family was of the frame shift codon 8 (-AA) mutation type. The diagnosis of hereditary spherocytosis is based on osmotic fragility test in the patient and the family. However, the examination of erythrocyte membrane proteins has not been possible. ßthalassemia is in the heterozygous form in the mother, the father, and in two sisters. The mother, the father, and one of the sisters also have hereditary spherocytosis in addition to thalassemia. All those family members are asymptomatic. However, the patient who has frame shift codon 8 homozygosity along with hereditary spherocytosis presented with a severe form of hemolytic anemia.

12.Successful Surgical Management of Typhlitis in a Patient with Acute Myeloblastic Leukemia
İhsan Karadoğan, Levent Ündar
Pages 143 - 146
Typhlitis (neutropenic enterocolitis) is a potentially life-threatening complication associated with neutropenia and combination chemotherapy. The incidence of this disease is increasing in both patients with hematologic malignancies and solid tumors with the advent of more aggressive chemotherapy. Here, we describe a patient with acute myeloblastic leukemia in whom typhlitis developed during induction chemotherapy and managed successfully with both medical and surgical intervention during neutropenic period. Our experience reinforces prior reports that intense medical treatment, close observation and emergent surgical intervention has been shown to be life saving.

13.Images in Haematology
Naci Tiftik, Kaan Ünlü, Orhan Ayyıldız, Ekrem Müftüoğlu
Page 147
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Page 149
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15.Abbrevations and symbols to be used in manuscripts for Turkish Journal of Haematology

Page 151
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