E-ISSN: 1308-5263
Turk J Hematol: 16 (4)
Volume: 16  Issue: 4 - 1999
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1.Bone Marrow Transplantation in Thalassemia
Buket Erer, Guido Lucarelli
Pages 147 - 159
Abstract | Full Text PDF

2.The Role of Interleukin-1 Inhibitors on Acute Myeloblastic Leukemia Blast Proliferation; Future Potential for Biotherapy
Cavit Çehreli, G. Hayri Özsan, Fatih Demirkan, Halil Ateş, Bülent Ündar, Faize Akyol, İlhan Öztop, Uğur Yılmaz
Pages 161 - 166
The effect of interleukin-1 (IL-1) as an autocrine growth factor on the proliferation of the acute myeloblastic leukemia (AML) blasts was studied. Bone marrow specimens were obtained from nine patients with different subgroups of AML. IL-1 receptor antagonist (IL-1RA) and IL-1 ß neutralizing antibody (IL-1ß NA) alone or in combination were added to the culture mediums of the AML blast cultures for the detection of their inhibitory effect on AML blast cell proliferation and colony formation. Average colony numbers in the IL-RA, IL-ßNA, and IL-IRA plus IL-IßNA included culture flasks, were 63.7 ± 21.5 %, 69.5 ± 19 %, 53.4 ± 23.7 %, respectively, as compared to those of the control (p < 0.01). Inhibition of colony formation by IL-IRA plus IL-IßNA was more prominent than by IL-IßNA alone (p < 0.01). No correlation between the inhibition of AML blast colony ormation and FAB AML subgroups was seen. RESULT: Both IL-1RA or IL-IßNA or in combination induced varying degrees of inhibition on blast colony formation. IL-I inhibitory molecules could be considered as an alternative therapy for AML in patients whose blast cells are sensitive to IL-1 inhibition.

3.Increased Serum Soluble CD23 and Soluble IL-2R Levels in Haematologic Malignancies
Ayşen Timurağaoğlu, İhsan Karadoğan, C. Erdoğan, Levent Ündar
Pages 167 - 169
Serum soluble CD23 (sCD23) and soluble IL-2 receptor (sIL-2R) levels increase not only in disorders with immune system activation, but also in hematological malignancies. They have been used as markers of disease progression and/or the response to therapy in lymphoproliferative disorders (LPD). In this study, we investigated the serum sCD23 and sIL-2R levels of 21 patients with different hematological malignancies [10 LPD, 6 multiple myeloma (MM), and 5 myelodysplastic syndrome (MDS)] before treatment, and compared them with 19 age- and sex- matched healthy subjects. Median sIL-2R levels were found to be significantly elevated in both the overall patient group and each of the subgroups. Median sCD23 levels were significantly higher in the overall patient group and in patients with LPD and MM. A positive correlation was found between sIL-2R and sCD23 levels in LPD. Our preliminary findings suggest that elevated serum levels of these soluble factors are not only markers of LPD but might be also used for other hematologic malignancies, except for MDS. Further studies should be designed to find out if it might be the result of an overactive immune system or not.

4.Congenital Agranulocytosis (Kostmann’s Syndrome) and G-CSF Therapy in an Infant
Hanifi Soylu, Ayşegül Ünüvar, Nuran Üstün, Fatih M. Mete, Onur Kutlu, Soner Sazak, Ünsal Özgen
Pages 171 - 175
Congenital agranulocytosis (Kostmann’s Syndrome) is a rare autosomal recessive inherited disorder characterised by severe neutropenia, recurrent infections, and death in early life, with the bone marrow showing a maturation arrenst of myeloid cells at the myelocyte stage. The treatment of Kostmann’s Syndrome with G-CSF results in rapid improvement. However, a few unexpected results with the use of G-CSF, were reported. Here, we describe a 7-month-old female with Kostmann’s Syndrome who had recurrent skin infections and a large pyogenic infection in the supravulvar region. The patient was treated with G-CSF successfully at low doses. The infectious process and the quality of lite of the patient improved. There was no adverse effect due to the dosage and the duration. Currently bone marrow transplantation is the best way to treat Kostmann’s Syndrome. Nevertheless, our experience showed that G-CSF treatment in Kostmann’s syndrome was highly effective and successfull on a short term basis.

5.Massive Intraabdominal Relapse in a Patient with Acute Lymphocytic Leukemia
Simten Dağdaş, Meltem Aylı, Gülsüm Özet
Pages 177 - 179
Abdominal involvement in an isolated extramedullary relapse region in patients with acute lymphocytic leukemia (ALL) is a rare event, especially in patients in hematological remission. The literature related to this subject is very limited. In this study, a 23-year-old male patient, is presented who had been in remission for 4 years, was admitted because of an enlarged testis, and was found to have an abdominal bulky mass during physical examination. An early diagnosis of abdominal involvement is extremely important and must be kept in mind for ALL patients who are admitted for testicular relapse.

6.Schistosoma mansoni Infection Following Allogeneic Bone Marrow Transplantation
Atilla Yalçın, Ferit Avcu, Ali Uğur Ural, Cengiz Beyan, Serdar Bedii Omay, Mehmet Tanyüksel, Levent Doğancı
Pages 181 - 184
A case of Schistosoma mansoni infection in a 28 year old male after allogeneic bone marrow transplantation presenting with portal hypertension and gross hematuria is described. Schistosomiasis was confirmed by the discovery of parasites in the feces, together with the failure the patient to respond to multiple antimicrobial and antifungal treatment. After praziquantel administration, toxic or septic shock syndrome evolved and the patients died of acute renal failure on day 39 post-transplant. In this report, we would like to emphasize the importance of pre-transplant stool and urine cultures, and appropriate serologic tests in patients coming from endemic areas. Patients diagnosed with schistosomiasis must be treated at least 3 to 7 weeks before transplantation.

7.p53 Gene Polymorphisms in the Turkish Population
Nejat Akar, Ece Akar, Şükrü Cin
Pages 187 - 189
There different RFLP’s on the p53 gene were studied in a healthy Turkish population. These RFLP’s were located on the exon 4 (CD 47), exon 6 (CD 213) and intron 6 (A-G). CD 47 (C-T) was not present. CD 213 (G) occurred very rarely with a frequency of 0.0114. The frequency of the Int 6 A/G a alteration was found to be 0.70 for “G”. The heterozygosity rate was 32.72%.


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