Biomedical subjects
Publications and source records attributed to S Erdem.
Two patients with CML who developed endometrial cancer following 2 years of busulfan treatment are reported. The possibility of a causal relationship between the cytotoxic drug and the development of this malignancy is discussed.
7 cases of multiple myeloma with a history of exposure to benzene, radioactive iodine, chemotherapy for Hodgkin's disease and of repeated injections of autovaccine to Staphylococcus albus hemolyticus are described. The relationship between the development of multiple myeloma and possible etiologic factors is discussed.
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135 Turks living in the vicinity of Antalya, a Turkish city on the Mediterranean coast, were studied for haemoglobin variants, beta-thalassaemia G-6-PD deficiency and haptoglobin types. The incidence of Hb-S was 2.3%. 8 beta-thalassaemic individuals with increased Hb-A2 and patient with 1 sickle cell-beta2-thalassaemia disease were found. The incidence of beta-thalassaemia with increased Hb-A2 was 6.7% and that of G-6-PD deficiency was 5.4%. The distribution of haptoglobin types in these people was very similar to that found in Turkish people in general; the only exception was the presence of Hp O in 2 individuals without haemolytic disorder. Gene frequencies of Hp1 and Hp2 were 0.26 and 0.7p4, respectively.
Clinical and haematological findings of 164 Turkish beta-thalassaemic heterozygotes with increased Hb-A2 are presented. The series comprised of 19 children, 81 females and 64 males. The majority of these thalassaemic individuals were asymptomatic and only 4 of them had slight or moderate splenomegaly. The mean values of haemoglobin concentration, PCV, MCH and MCHC were significantly lower than those of the corresponding normal controls. Erythrocytosis was present in 33% of them. Hb-A2 values ranged between 3.8 and 6.1%, and between 3.8 and 6.5% as assessed by the methods of DEAE- or DE-cellulose chromatography, respectively. Hb-F was found to be above the normal range in 15.1%. It is established that beta-thalassaemia with increased Hb-A2 is the most frequent variety of thalassaemia in Turkey (80%) and both beta+- and beta0-thalassaemia genes were present in this country. The regional distribution of beta-thalassaemia in Turkey is also presented and the origin of beta-thalassaemia genes in Turkish people is discussed.
The unstable hemoglobins Istanbul and Saint-Etienne have the same amino acid substitution (alpha 2 beta 2 92F8 His leads to Gln). Despite this, there are some clinical and hematological differences between the individual with Hb Istanbul and the one with Hb Saint-Etienne. These are: (1) the clinical course of the patient with Hb Istanbul before splenectomy was more severe when compared with that of the individual with Hb Saint-Etienne; (2) although Hb F was found within normal range in the individual with Hb Istanbul, a high level of Hb F (19%) was observed in the patient with Hb Saint-Etienne. The alkali-resistant Hb found in the former was of adult type and in the latter case it was of fetal type. The possible explanations for these differences are discussed.
20 patients with beta-thalassemia intermedia classified according to the results of genetic studies are presented. (1) 9 patients with beta-thalassemia intermedia homozygous for beta-thalassemia with increased Hb-A2 are reported. (2) 8 patients with beta-thalassemia intermedia, 3 homozygous for beta-thalassemia with normal levels of Hbs.A2 and F, 5 heterozygous for both this and beta-thalassemia with increased Hb-A2 are presented. (3) 2 families with beta-thalassemia intermedia heterozygous for both beta-thalassemia with increased HbA2 and 'silent" beta-thalassemia are reported. Two different varieties are presented.
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The distribution of various types of leukemia due to chronic exposure to benzene is described in a series comprising 34 cases. The incidence of leukemia among 31 show-workers was 13.5/100,000. Acute myeloblastic leukemia was the most frequent type, followed by preleukemia, acute erythroleukemia and acute lymphoblastic leukemia. The extreme rarity of chronic myeloid leukemia was a noteworthy finding. The differences and similarities between the distribution of various types of leukemia in different series of patients with chronic exposure to benzene and ionizing radiation are discussed.
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Two patients, one with myeloid metaplasia and the other one with PNH, both due to chronic exposure to benzene are presented. The patient with PNH exhibited also marked monocytosis as a rare hematologic finding of chronic benzene exposure. The etiological relationship between myeloid metaplasia, PNH and chronic benzene exposure is discussed.
The investigation of serum samples of 200 unrelated adult Turks revealed the following gene frequencies: Hp-1 = 0.265; Hp-2 = 0.735. The frequencies are in the range of most of the Asiatic population.
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