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P Kobylka

Publications and source records attributed to P Kobylka.

32 records · Page 2Linked to original sources
PubMed1996

[Successful transplantation of fetal blood in a boy with leukocyte integrin deficiency syndrome].

The authors describe the first successful case of transplantation of haematopoietic stem cells in a patient with severe congenital immunodeficiency-the syndrome of deficiency of leukocytary integrins (LAD), leukocyte adhesion deficiency) in the Czech Republic and, at the same time, the first successful transplantation of umbilical blood in this disease in the world. The six-year boy was completely cured by the transplantation of haematopoietic stem cells contained in umbilical blood sampled during delivery of his healthy brother with identical HLA system. The pretransplantation myeloablative preparation was performed by a combination of busulfan, cyclophosphamide and etoposide. The relatively uncomplicated posttransplantation course was secured by preventive administration of antibiotics and immunoglobulins. The reattachment of the stem cells, estimated from the peripheral blood picture, occurred 25 days after the transplantation, the success of the intervention was confirmed by reaching physiological values of originally null expression of integrins on leukocytes of the patient 30 days after the transplantation. The transplantation of umbilical blood is a very promising therapeutic method especially in children with leukemia, congenital severe immunodeficiencies o inborn errors of metabolism.

Child↗
PubMed1996

[The first case of leukocyte integrin deficiency syndrome in the Czech Republic and successful prenatal diagnosis in the affected family].

The syndrome of leukocyte adhesion deficiency (LAD) is a rare congenital immunodeficiency which is usually manifested from birth by serious infections of the skin and mucosal membranes. The molecular basis of the disease is heterogeneous: quantitative or qualitative disorders of the beta 2 integrin sub-unit are involved which lead to the absence or substantially reduced expression of adhesive molecules of the CD11/CD18 complex on leukocytes. The authors describe the case of a boy who suffered from this syndrome. The diagnosis was established at the age of four years, based on the typical clinical picture and confirmed by examination of integrins on lymphocytes and granulocytes which were zero. During the mother's subsequent pregnancy prenatal diagnosis was made by puncture of the umbilical cord during the 22nd week of gestation. Affection of the foetus by this syndrome was ruled out by examination of integrin expression on foetal leukocytes, a normal finding was confirmed also after delivery. During delivery umbilical blood was collected which was frozen and later used for therapeutic transplantation to the sibling suffering from LAD. This is the first case of this syndrome in the Czech Republic and first prenatal diagnosis which led to aimed collection of umbilical blood used for treatment of this rare immunodeficiency.

Child, Preschool↗
PubMed1996

Successful HLA-identical sibling cord blood transplantation in a 6-year-old boy with leukocyte adhesion deficiency syndrome.

A 6-year-old boy with the severe form of the leukocyte adhesion deficiency syndrome (LAD) received a transplant of cord blood (CBT) from his HLA-identical brother. The donor was proved healthy by successful prenatal diagnosis. CBT was performed after conditioning with etoposide, busulfan and cyclophosphamide. After hematopoietic recovery complete chimerism was proved as well as the normal expression of CD11x/CD18 complex on circulating leukocytes. The only post-transplant complication was a mild pneumonitis resolving on the corticosteroid therapy. Thirteen months after CBT the boy is in good health and shows no signs of immunodeficiency. As far as we know this is the first report of successful CBT in a patient with LAD syndrome.

Child↗
PubMed1995

[Allogenic transplantation of bone marrow in childhood].

BACKGROUND: Bone marrow transplantation has become the therapeutic method in some forms of malignant haemotopoietic diseases, malignant tumours, inborn errors of metabolism and immunodeficiency states. The objective of the presented work is the analysis of 40 allogenic bone marrow transplantations in children made in 1989-1994. METHODS AND RESULTS: Bone marrow transplantation was made in 40 children (26 boys and 14 girls), mean age 10.5 years (range 1.5-17.5 years). Indications were acute lymphoblastic leukaemia in 11, acute myeloid leukaemia in 10, chronic myeloid leukaemia in 6, myelodysplastic syndrome in 2, aplastic anaemia and Fanconi's anaemia in 7, non-Hodgkin lymphoma in 2 and inborn errors in 2 children. The donor was in 33 patients in HLA identical sibling and in seven instances a monozygotic twin, HLA non-identical sibling or relative or unrelated matched donor. Bone marrow engraftment was achieved in 35 (87.5%) patients, in one instance the bone marrow was rejected (2.5%) and in four patients (10%) early death occurred before the bone marrow engraftment. On Aug. 15, 1995 20 patients (50%) survived, a relapse developed in 7 (17.5%) and 13 patients died in conjunction with the transplantation (32.5%). The most frequent cause of death were infectious complications (9 children) either in conjunction with the development of graft versus host reaction (6x) or without signs of this reaction (3x). As a prophylaxis of graft versus host disease 24x Cyclosporine A with corticosteroids was used, 16x with methotrexate. A chronic graft versus host disease developed in 6 of 28 children surviving 100 days after transplantation. The greatest problem are infectious (bacterial and mycotic) complications in the phase of bone marrow aplasia before engraftment of the transplanted bone marrow or in conjunction with a graft versus host reaction which cannot be completely avoided by preventive measures. CONCLUSIONS: Bone marrow transplantation is also in children an effective therapeutic method of some forms of malignant haematopoietic diseases, malignant tumours and immunodeficiency states. The correct indication, suitable donor, preventive measures against the graft versus host reaction and protection against infectious complications are essential for the success of this pretentious treatment.

Adolescent↗
PubMed1992

[Results of bone marrow transplantation at the Institute of Hematology and Blood Transfusion in Prague].

The first allogenic bone marrow transplantation (TKD), when for the preparation whole body irradiation was used, was implemented in the Institute of Haematology and Blood Transfusion (UHKT) in Prague in 1986. Before June 1992 36 TKD were performed incl. 28 allogenic, 2 syngenic and 6 autologous. For the first time bone marrow from a non-related donor was transplanted. Of 30 allogenic and syngenic TKD to the present time 17 patients survive, i.e. 56.6% of the whole group. According to individual diagnoses 8 patients with the diagnosis of chronic myeloid leukaemia (CML) survive, 5 of 10 patients with the diagnosis of acute leukaemia (AL) and 3 of 4 patients with the diagnosis of severe aplastic anaemia (SAA) or with Fancon's anaemia (FA) resp. The survival period of the whole group is from 1-62 months since the transplantation. The main cause of death of 8 from 13 patients who died were infections associated with acute or chronic disease of the graft against the host (GVHD). In autologous TKD the bone marrow was treated with etoposide. Of the six transplanted patients with AL five survive 1.5-30 months after transplantation. The authors present some general information of pretransplantation preparation, prevention of GVHD, its incidence and results of TKD.

Adolescent↗
PubMed1991

Blood count changes due to mononuclear cell apheresis.

Complete blood count and CFU-GM count of 41 healthy donors was determined immediately before and immediately after mononuclear cell (MNC) apheresis. Red blood cell count dropped from a mean of 5.2 to 4.9 x 10(12)/l, the difference being significant (p less than 0.001). Platelet count was reduced from a mean of 219 to 187 x 10(9)/l, the difference was once again significant (p less than 0.005). Polymorphonuclear cell count did not change significantly, while MNC count was reduced substantially (from mean 3.1 to 2.4 x 10(9)/l, p less than 0.001). The difference between pre- and postapheresis CFU-GM count (median 11.3 versus 8.8 x 10(3)/l respectively) was on the borderline of statistical significance (p = 0.05).

Adult↗
PubMed1991

Intermittent-flow versus continuous-flow mononuclear cell apheresis.

An intermittent-flow (Haemonetics V-50) and a continuous-flow (Fenwall CS-3000) blood cell separator were compared regarding mononuclear cell (MNC) collection. 17 evaluable V-50 and 24 CS-3000 aphereses were performed on healthy donors. No significant difference was found in MNC yield (median 6.4 x 10(9) on V-50, 6.3 x 10(9) on CS-3000) or in CFU-GM yield (median 41 x 10(3) versus 43 x 10(3) respectively). Nevertheless, red blood cell as well as polymorphonuclear cell contamination was substantially greater on V-50 than on CS-3000, while platelet contamination was slightly lower on V-50 than on CS-3000. The continuous-flow apheresis system CS-3000 is concluded to be more suitable for MNC harvesting than the intermittent-flow system V-50.

Adult↗
PubMed1989

[Comparison of the Haemonetics V50, Fenwal CS 3000 and IBM 2997 blood separators].

Haemonetics V 50 (HV-50), Fenwal CS 3000 (FCS-3000) and IBM 2997 separators are compared according to the results of donor plateletaphereses. The mean platelet yield of HV-50, FCS-3000 and IBM-2997 was 464 X 10(9), 551 X 10(9) and 468 X 10(9) respectively (the difference both between FCS-3000 and HV-50 and between FCS-3000 and IBM-2997 being significant - p less than 0.01). The median leukocyte contamination of platelet concentrates was 0.3 X 10(9), 0.2 X 10(9) and 5.3 X 10(9) respectively (the difference between FCS-3000 and HV-50 being not significant). The separators are further compared on the basis of platelet concentrate volume, erythrocyte contamination of thromboconcentrates, processing time, donor and staff convenience, our experience concerning product reliability and service quality, and finally the price of both the separators and the disposables.

Adult↗
PubMed1987

Cytogenetic study of circulating blasts in leukemias.

A special cultivation technique of separated blasts of peripheral blood in suspension culture has been used for cytogenetic diagnosis of patients suffering from acute nonlymphocytic leukemia, chronic myeloid leukemia, and refractory anemia with excess of blasts. Twenty-three patients were examined; in ten cases isolation of blasts was performed on Ficoll-Verografin and in the remaining 13 patients further separation of T-lymphocyte precursors by means of sheep erythrocytes was performed. Remarkably, a 100% success rate was attained in all cultivations. The optimum harvesting time was 72-96 hours; the rate of cell division per cultivation was determined by means of bromodeoxyuridine incorporation; second mitoses were revealed only after 96-hour cultivation. In all patients, except one, abnormal karyotypic changes were ascertained in separated blasts of peripheral blood cultivations. In most cases the morphology of chromosomes obtained from separated blasts of peripheral blood cultivations was of excellent quality.

Acute Disease↗
PubMed1981

Transplantation of bone marrow cells with changed haemoglobin type.

Haemoglobin was used as a marker for the detection of radiation chimera in rats. After a single blood loss, the synthesis of haemoglobin type had changed, as observed in peripheral blood one week after bleeding. After one month the haemoglobin pattern was the same in the experimental rats and in the control animals. Twenty-four hours before bone marrow transplantation the donors were bled. Haemoglobin synthesis had changed also in the recipients. In contrast with a single blood loss, this change of haemoglobin type persisted as observed one year after transplantation. The cells with changed synthesis can repeatedly be retransplanted.

Animals↗
PubMed1977

Bone marrow cell separation on Ficoll gradient.

Cell fraction from rat and murine bone marrow were obtained by sedimentation at unit gravity. A linear gradient of polymerized sucrose (Ficoll) was used and some properties of individual cell fractions were studied. The 3rd cell fraction containing the lymphoblasts and lymphocytes contained also stem cells.

Animals↗