Results of IDA-FLAG programme in the treatment of recurrent acute myeloblastic leukaemia--preliminary report.

BACKGROUND: Unsatisfactory treatment results of acute myeloblastic leukaemia inspire the search for new drugs, characterised by higher efficiency and lower toxicity. The aim of the study was the assessment of the efficiency and side effects associated with the implementation of IDA-FLAG protocol. MATERIAL AND METHODS: The assessment of treatment results and undesirable effects was based on the material of 4 children with the relapse of acute myeloblastic leukaemia, after a total of 6 IDA-FLAG protocols. RESULTS: Complete remission was obtained in 2 (50%) children, with mean remission time of 13.5 months. One of the children underwent the transplantation of haematopoietic cells from unrelated donor. The main side effect observed was bone marrow aplasia. Leucopenia, granulocytopenia, thrombocytopenia and anaemia persisted for approx. 20 days. Apart from haematological symptoms, the following were also observed: the symptoms of intolerance after ARA-C, aspergillosis of paranasal sinuses and lungs and severe coagulation disorders. CONCLUSION: IDA-FLAG protocol may be recommended for use in children with AML relapse and the undesirable effects observed are acceptable.

TNFRs and IL-6R transmembrane receptors expression and release of their soluble forms by neutrophils and mononuclear cells from cancer patients.

TNF-alpha and IL-6 are multipotential mediators involved in the control of many host's reactions to tumour. Their biological effects are mediated through the membrane-bound receptors (TNFRp55, TNFRp75 and IL-16R respectively) which can exist in soluble forms. In the present study we compared release of soluble sTNFRp55, sTNFRp75 and sIL-6R with expression of their membrane-bound on PMN and PBMC. Cells were isolated from patients with cancer diseases with a different location and histological classification. We have found that alterations of membrane-bound TNFRp75 expression and in the secretion of soluble TNFRp75 form, as opposed to other receptors examined, are characteristic features of neutrophils and mononuclear cells isolated from cancer patients. The similar changes observed in the expression of TNFRp75 by PMN and PBMC appear to confirm a significant role of PMN in the tumour response mediated by TNF-alpha. Furthermore, the altered membrane-bound TNFRp75 expression and sTNFRp75 secretion appear to depend on the tumour type.

[Flow cytometry: diagnostic, therapeutic and prognostic usefulness in solid tumors]. / Cytometria przeplywowa--przydatnosc diagnostyczna, lecznicza i rokownicza w litych guzach.

Flow cytometry is a new technique measuring several optical parameters of particular cells in cell suspension. These parameters are: optical density, light scatter and fluorescence emission. Optical assay conjugated with digital data processing permit the precise characterisation of chosen cellular subpopulations. The optical signs transformed by photomultipliers as analogue signals are presented as descriptive cytograms. Digitally converted signals analysed by multichannel analyser are stored as histigrams. Flow cytometry is widely used in the diagnostics of blood neoplastic diseases and, in a less routine manner, in the diagnostics of solid tumours. The latter requires accurate disaggregation of tumour tissue to obtain homogenous cell suspension. Different, more or less efficient, mechanical or enzymatic disaggregation techniques are used to prepare appropriate cell suspensions. Flow cytometry DNA analysis with simultaneous determination of several protein proliferation factors are heavily used in research and clinical laboratories to follow tumour cell proliferation, efficacy of cancer therapy and finally as prognostic factors. Although these assays yield promising results in some specific tumours, their value and accuracy seems still equivocal.

[The evaluation of lymphocyte subpopulation by flow cytometry in healthy children]. / Ocena subpopulacji limfocytów oznaczanych metoda cytometrii przeplywowej u zdrowych dzieci.

Seeing that amount of experiences concerning the cytometry of lymphocyte subpopulation in particular age ranges is very low we considered that undertaking this subject is useful. By the method of flow cytometry 49 children at the age from 2 months to 15 years were studied. According to the results of our studies we found, starting from the third year of life decrease of mean percentage values of cells CD19+, progressive in relation to the age, increase of mean percentage values of cells CD2+, increase of percentage values of cells CD8+ and cells CD56++ and the decrease of mean values of the indicator Th/Ts. Remaining CD3, CD4 did not show any statistically significant differences in investigated age ranges. Determined by the method of flow cytometry the percentage and complete values of lymphocyte subpopulations in peripheral blood in investigated age ranges can make the reference values in the assessment of immunological state.

[Anomalies of the cervical spine in children]. / Anomalie w zakresie kregoslupa szyjnego u dzieci.

Two children are presented with congenital anomalies of the cervical spine and associated malformations of the nervous and urinary systems. The attention is paid to the usefulness of the cervical spine assessment in children in cases of recurrent cerebellar manifestations of unclear aetiology or combined malformations of the urinary system.

TAR syndrome with orofacial clefting.

A case of TAR syndrome with bilateral cleft lip and palate is presented. Bilateral symmetric focomelia, normal thumbs among five fingers of hands, synostosis of IVth and Vth metacarpal bones and some defects of lower limbs with associated thrombocytopenia were noted. Dysmorphic facial features included hypertelorism, epicanthus, blue sclerae, broad nasal root, micrognathia, low-set ears, sparse blond hair. To our knowledge this patient represents an unusual association of TAR syndrome with orofacial clefting. A common background of TAR and Roberts/SC syndrome is suggested.

[TAR syndrome (congenital thrombocytopenia and aplasia of the radial bones) in a 5-months-old boy]. / Zespól TAR (wrodzonej trombocytopenii i braku kosci promieniowych) u 5-miesiecznego chlopca.

A boy aged 5 months had the TAR syndrome (thrombocytopenia and aplasia of radial bones) of autosomal recessive inheritance. Bilateral reduction of the length of the upper extremities included both forearm bones and humerus, so that the shoulder girdle was connected directly to the hand, with all fingers and thumb normally shaped. These skeletal anomalies were associated with thrombocytopenia. Various other abnormalities were present, with the most controversial anomaly being bilateral cleft of the lip and secondary palate. It may be an accidental coexistence of abnormalities or may suggest a common mechanism of the development of the TAR syndrome, Roberts syndrome and SC-phocomelia belonging to hereditary reduction of the extremities with identical mode of inheritance.

[A case od rare location of Ewing's sarcoma in a 6-year-old boy]. / Przypadek rzadkiej lokalizacji miesaka Ewinga u 6-letniego chlopca.

A 6 boy is described treated for two months for pneumonia. In view of absent improvement the diagnosis was verified and malignant tumour was recognized. Radiological examination and clinical observation showed presence of a tumour in the 4th rib with pulmonary metastases. Histological examination of a biopsy specimen from the rib demonstrated primary microcellular Ewing's sarcoma. Of importance are: the age of the patient, rarely described location and diagnostic difficulties in the initial stage of the disease.

[A particular role of free fatty acids in the fetus and newborn and during pregnancy and labor]. / Szczególna rola wolnych kwasów tluszczowych u plodu i noworodka oraz w okresie ciazy i porodu.

Free fatty acids account for a small fraction of plasma lipids. The author describes their significant role in the energy-yielding processes in the organism, and their participation in many important metabolic functions in the tissues during intrauterine life, neonatal period, pregnancy and labour.

Concentration of free fatty acids (FFA) in amniotic fluid and maternal and cord serum in cases of intrauterine growth retardation.

There were no efficacious methods of diagnosing the syndrome of intrauterine growth retardation (IUGR) before birth. Only about 50% of cases with this syndrome can be recognized by clinical examinations. The aim of our investigations was an attempt to determine free fatty acid level (FFA) in amniotic fluid as a biochemical test in prenatal diagnosis. The investigations were carried out in 49 pregnant women and their newborns in whom IUGR was diagnosed clinically. The control group consisted of 30 pregnants who had an uneventful pregnancy and normal labor. Samples of amniotic fluid were taken in the first stage of parturition (dilatation of cervical os +/- 4-5 cm) by vaginal amniocentesis or amniotomy during cesarean section. The concentration of FFA in samples of amniotic fluid and serum were determined by Ducombe's method. It was found that during pregnancy complicated by IUGR, the free fatty acid concentration in amniotic fluid was almost three times higher than in normal pregnancy (0.355 mmol/l and 0.125 mmol/l) respectively. These differences are statistically significant (t = 12.58 p less than 0.001). Thus, determination of FFA can be used as a biochemical test in prenatal diagnosis of IUGR.