The role of methylenetetrahydrofolate reductase (MTHFR C677T), glutathione Stransferases (GSTM1 and GSTT1 null, GSTP1 Ile105Val), and cytochromes p450 (CYP1A1*2A) genotypes in the etiology of childhood leukemia was simultaneously investigated. 144 Turkish children with acute lymphoblastic leukemia (ALL) and 33 with acute nonlymphoblastic leukemia (ANLL) were studied and compared with 185 healthy pediatric controls. The frequency of MTHFR genotype was insignificantly higher in ALL (7.7%) and ANLL (6.3%) than in controls (4.4%). Equal distribution of the GSTM1 null genotype was detected between ALL patients and controls (55%), while its incidence was slightly higher in ANLL patients (61.3%). Although GSTT1 null genotype was insignificantly lower in ALL patients (20.9%) than controls (22.7%), it was significantly underrepresented in ANLL patients (6.5%) (P = 0.05, OR 0.24, 95% CI 0.05-1.03). The homozygous frequency of GSTP1 genotype did not differ significantly between groups of ALL (3.7%), ANLL patients (9.1%) and controls (4.9%). Homozygous CYP1A1*2A genotype was underrepresented in ALL patients (1%) as compared to control (4.8%) but the differences did not reach to statistical significance (OR 0.21; 95% CI 0.03-1.72). Homozygosity for this genotype was not detected in ANLL patients. No particular association was noted between different combinations of combined genotypes and risk of development of childhood ALL and ANLL. These results suggested that there are no significant associations between the studied genotypes and the risk of developing either form of acute leukemia except GSTT1 null and homozygosity for CYP1A1 genotypes that may play protective roles in the development of ANLL in Turkish children. Am. J. Hematol. 73:154-160, 2003.

The serum zinc concentration in 101 pregnant women and 40 control subjects were determined by using atomic absorption spectrophotometer. The zinc levels were significantly low in 30 poorly nourished pregnant women compared to controls (P less than 0.01), whereas there was no statistical difference between the normal mean zinc values and that of well-nourished pregnant women (P greater than 0.05). This study revealed that nutrition is an important factor during pregnancy among Turkish women. In view of severe teratogenic effects of maternal zinc depletion in experimental animals as well as epidemiological evidence that maternal zinc deficiency could be a cause of severe congenital malformations of the central nervous system in humans, more extensive studies are definitely needed in Turkey.

Eighty-one Turkish children with Burkitt's lymphoma (BL) were observed during a period of 24 years (1968-1992). The diagnosis was established histologically according to WHO criteria. BL represented 48.5% of NHL in this series. The median age of patients was 5 years with a sex (M/F) ratio of 2.3/1. The most common primary site of tumor involvement at initial presentation was the abdomen (70.4%), which was followed by facial tumors, in particular the jaw and orbit (45.7%). The majority of the patients (84.0%) were in advanced stages (C and D) at initial diagnosis. Facial tumors observed in Turkish children with BL were more similar to African Burkitt's lymphoma than American or European cases. High titers of antibodies against VCA and EA of EBV were also observed in 32 recent cases of BL. Preliminary molecular and immunologic studies revealed EBV-DNA (type I) and T cell deficiency. The clinical presentation, median age, and association with EBV revealed that BL appears to be inbetween African and non-African types in Turkish children. This will be further elucidated in the future by direct examination of tumor cells for EBV and investigation of the molecular characteristics in these cases.

A high serum sCD44 level and/or tumor tissue expression at diagnosis is associated with poor prognostic criteria and/or unfavorable outcome in childhood leukemias and lymphomas.

Thirty-three patients presenting with orbito-ocular granulocytic sarcoma (OOGS) and acute myelomonocytic leukemia (AMML) were diagnosed in Turkish children from 1963 to 1983. OOGS, characterized by exophthalmos, chemosis and orbital masses, was observed in 33 (27.2%) of 121 AML patients compared with 41 children of AMML without ophthalmic tumors during the same period. Eye tumor and bone marrow aspirates were also studied under light and electron microscopies. The comparison of the hematological parameters did not indicate any statistical difference between the groups. Despite similar chemotherapy regimens administered to all patients, the mean survival time was 8.7 months in the OOGS group, which is significantly shorter compared to those without OOGS (28.6 months) (p < 0.01). These cases may be classified as a ‘high risk’ subgroup of childhood AMML.