İşletme

Serum selenium concentration was investigated in 40 children with iron deficiency anemia and in 40 control subjects matched for age, sex and geographical origin. A spectrofluorometric method was used for determination of the selenium level. It was found to be significantly lower (p < 0.001) in the patient group, which consisted of both normally developed and malnourished children. Patients also having pica had higher levels of selenium compared to patients without pica. There was no relation between the serum selenium concentration and hematological parameters such as hemoglobin, serum iron, serum iron binding capacity and unsaturated iron binding capacity. However the results of 15 patients followed during iron therapy indicated that the duration of the anemic period may affect the selenium concentration. This study also suggests the effectiveness of iron and selenium administration.

In children with iron deficiency anemia, bactericidal capacity of polymorphonuclear leukocytes (PMN) and serum opsonic activity were studied. Nitroblue tetrazolium test (NBT), hexose monophosphate (HMP) shunt activation, and myeloperoxidase (MPO) activity of PMN of these cases were also examined. Bactericidal capacity and HMP shunt activation were found to be decreased in iron deficiency anemia ( p less than 0.001). MPO activity, NBT test, and serum opsonic activity were found to be within normal limits. After 1 1/2 months of iron therapy there was an improvement in bactericidal capacity and it returned to a normal level after 3 months of therapy.

Killing capacities of polymorphonuclear neutrophils (PMN) and the serum opsonic activity were investigated in 15 children with measles. Seven of them had bacterial infection secondary to the measles. The mean values for killing capacities of the patients were significantly impaired when they were compared with those of the control group which consisted of 15 healthy age-matched children (p less than 0.001). These defects were found to be present during the early stages of the measles. The serum opsonic activity was found to be normal.

In order to explain susceptibility to bacterial infection in patients with nephrotic syndrome, bactericidal capacity of polymorphonuclear neutrophils (PMN) and serum opsonic activity were studied. The groups consisted of 29 patients and 29 controls. Bactericidal capacity was found to be statistically significant defective in the patient group when compared with controls for Staphylococcus aureus and Excherichia coli. Serum opsinic activity was found to be defective in up to 50% of the patients, but it was not statistically significant for either bacteria. There is no correlation between the impairment of bactericidal capacity and serum total protein, albumin, globulin, total lipid, cholesterol, age of patients and the duration of the illness. When bactericidal capacity was examined in five patients in remission it had returned to the normal level. The results of this study suggest that bactericidal capacity and possibly serum opsonic activity are influenced by the nephrotic syndrome.

The presented case is a boy with T-cell acute lymphoblastic leukemia (ALL) with hairy cell (HC) features and monoclonal gammopathy. The disease process had an acute onset and followed a rapid, progressive course. The patient had minimal splenomegaly and bicytopenia, but the bone marrow displayed increased numbers of reticulin fibers. The blasts were positive for tartrate-resistant acid phosphatase (TRAP) and CD11c. Molecular analysis revealed rearrangement of immunoglobulin heavy chain genes and a rearranged T-cell receptor (TcRbeta) beta gene. The patient responded to conventional ALL therapy. Acute T-cell ALL with HC features in childhood has not been reported previously, either alone or in association with monoclonal gammopathy. We propose "T-ALL with hairy cell features" to describe this case.

We have attempted to investigate the dysplastic changes in the hematopoietic system associated with juvenile rheumatoid arthritis (JRA) and its relation to disease activity. The peripheral blood smear and bone marrow aspiration samples of 17 JRA patients were investigated and correlations with laboratory parameters of disease activity sought. The age range was 6-16 years and the duration of disease 1.5-108 months. Abnormal finding of the peripheral smear and bone marrow were scored separately. The score of pathological peripheral blood findings correlated significantly with CRP and ferritin (both P <0.05). In the bone marrow specimens marked changes were noted in the myeloid, erythropoietic, and megakaryopoietic series; however, the score of pathological findings did not correlate with laboratory parameters of disease activity (P > 0.05). We suggest that JRA is associated with marked myelodysplastic changes, also manifested in the peripheral blood smear; these changes may well be the consequence of the inflammatory milieu, including cytokines, during active disease.

The function of the immune surveillance system has been studied extensively in patients with neoplastic disease. Defective monocyte chemotaxis has been demonstrated in a variety of neoplastic conditions. We have assayed chemotaxis in monocytic and polymorphonuclear leukocytes taken from patients with epidermoid tumors of the head and neck. Multiwell chemotaxis chambers and the chemoattractant, formyl-methionyl-leucyl-phenylalanine, were used. Polymorphonuclear leukocytes responded normally, but monocytes from all tumor patients studied displayed marked reductions in random migration (86% inhibition) and in chemotaxis (77% inhibition) toward formylpeptide.

Nineteen children with hemophagocytic lymphohistiocytosis (HLH) were studied in the Department of Pediatric Hematology, Hacettepe University. Patients were divided into two groups. Group 1: Thirteen patients were classified as having a genetic etiology (7 familial, 6 presumed familial) on the basis of an affected sibling and consanguinity. There was a history of consanguineous marriage in 13 of the families. Seven of them had a history of a sibling with HLH. Group 2: Six patients were diagnosed with sporadic HLH. The age at presentation for familial patients was 0.7-84 months (mean 21.9 +/- 24.9 months), and for sporadic cases it was 2.5-48 months (mean 22.7 +/- 19.8 months). The clinical and laboratory data of these two groups were similar at diagnosis. Thirteen cases were diagnosed premortem by bone marrow aspiration. Splenic biopsy was performed in 2 patients. Four patients were diagnosed by postmortem examination. Elevated LDH levels were found in all patients tested. No significant differences for clinical and laboratory data were found between the two groups.