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Biomedical subjects

I Slavc

Publications and source records attributed to I Slavc.

52 records · Page 3Linked to original sources

[Malignant bone marrow infiltration in nuclear magnetic resonance tomography].

Magnetic resonance imaging (MRI) is currently the only non-invasive method detecting changes of bone marrow. While yellow bone marrow produces a high signal intensity, which is similar to subcutaneous fat, any other cellular infiltration of the bone marrow causes a decrease of signal intensity because of replacement of marrow fat cells. In this report we describe two patients, who underwent MRI because of clinical symptoms of coxitis, in order to exclude Perthes disease. Both cases showed decreased signal intensity of the bone marrow particularly of the proximal femura, highly indicative of cellular bone marrow infiltration. Bone marrow aspiration in these patients showed acute lymphocytic leukemia in one case and marrow infiltration by neuroblastoma cells respectively in the other case. Because of its high sensitivity, MRI is also suitable in detecting focal bone marrow disease. For these cases a biopsy of these focal bone marrow lesions can be performed in order to stage the disease properly as in the presence of neuroblastoma or malignant lymphoma presenting with localized disease, however, with focal bone marrow metastases on MRI. In addition MRI can also be used to follow up the disease as well as therapy by showing the regression of these bone marrow lesions.

Adrenal Gland Neoplasms↗

B-cell differentiation pattern of cutaneous lymphomas in infancy and childhood.

This article documents four children with cutaneous lymphomas. Two of them had regional disease and the other two already had disseminated disease when they were diagnosed. Immunophenotyping of the lymphomatous infiltration disclosed a cell antigen profile with pre-pre-B (HLA-DR+, CALLA+, VIB-C5+), pre-B (HLADR+, CALLA+, VIB-C5+, cytoplasmatic immunoglobulin+), and transitional pre-B characteristics (HLA-DR+, CALLA+, VIB-C5+, surface immunoglobulin+). The morphology of the blast cells in all patients showed features of lymphoblastic lymphoma. Two patients had the convoluted cell type in common, but they were immunologically different, one displaying pre-pre-B and the other pre-B immunophenotypes. The remaining two cases were diagnosed as lymphoblastic lymphomas "type others" according to the Kiel Classification, one with pre-B and the other with transitional pre-B characteristics. The morphologic and immunohistochemical parameters as well as the clinical data from the four cases clearly indicate the heterogeneity of cutaneous non-Hodgkin's lymphomas in children.

Antineoplastic Agents↗

Endocrine function after bone marrow transplantation without the use of preparative total body irradiation.

Ten children who underwent allogeneic (n = 5) or autologous (n = 5) bone marrow transplantation (BMT) for chronic myelogenous leukaemia (n = 2), acute lymphoblastic leukaemia (n = 1), acute myelogenous leukaemia (n = 2), severe aplastic anaemia (n = 2), malignant histiocytosis (n = 1), neuroblastoma (n = 1) and teratoma (n = 1) were assessed for endocrinological function. Transplant preparative regimens consisted of high-dose cyclophosphamide, high-dose cyclophosphamide in combination with high-dose busulphan, high-dose melphalan as well as BACT (BCNU, cytarabine, cyclophosphamide and 6-thioguanine) chemotherapy. None of the patients received total body irradiation (TBI). Median survival following BMT was 37 months (range 7-115). Growth hormone deficiency was present in only one patient; none of the patients had abnormal thyroid or adrenocortical function. This is in contrast to previous reports in which growth hormone deficiency and abnormal thyroid and adrenocortical function occurred in a much higher percentage of patients after BMT conditioned with TBI.

Adolescent↗

[High-dose busulfan with subsequent bone marrow transplantation in poor-risk forms of leukemia].

The toxicity and potential late side effects of total body irradiation, especially in children, have caused the evaluation of alternative myeloablative agents. We report the results obtained in 4 patients with acute myelocytic leukaemia, 2 patients with acute lymphocytic leukaemia and 1 patient with chronic myelocytic leukaemia who received high-dose busulfan followed by bone marrow transplantation (4 times allogeneic, 3 times autologous), according to the protocol published by G. W. Santos. 4 patients have survived 708+, 413+, 313+ and 234+ days, respectively, to date, 3 patients died, two of whom had acute myelocytic leukaemia in relapse and died of cardiac and combined organ failure 9 and 10 days post transplantation, respectively, without evidence of leukaemic cells in the bone marrow. One patient with acute lymphocytic leukaemia (T-All) in second remission died of an extramedullary relapse 485 days after bone marrow transplantation. We report our experience in the use of busulfan before transplantation not only in patients with acute myelocytic leukaemia, but also in patients with acute lymphocytic leukaemia and chronic myelocytic leukaemia and discuss the effectiveness and toxicity of high-dose busulfan as alternative to total body irradiation.

Adolescent↗

[Use of autologous bone marrow transplantation in pediatric malignant diseases--results and conclusions].

Ultrahigh-dose myeloablative antineoplastic therapy followed by autologous bone marrow transplantation (ABMT) has become an attractive therapeutic option for patients without HLA compatible bone marrow donors. Autologous bone marrow was harvested in 9 patients. In four cases the bone marrow was also treated ex vivo with a stable derivative of 4-hydroperoxycyclophosphamide, ASTA-Z 7654, to eliminate residual tumour cells. Altogether 5 patients, namely a patient with metastatic neuroblastoma, a patient with malignant histiocytosis, a patient with recurrent sacrococcygeal malignant teratoma and two patients with acute myelogenous leukaemia in first remission are in continuous remission so far from 125 + to 821 + days (median 657 + days). These patients were transplanted at the time of minimal tumour load (first remission) and in good clinical condition 3-7 months after diagnosis, while 3 of the four patients who died were transplanted in first or repeated relapse after one to several years of chemotherapy. It is concluded that the earliest possible recognition of a refractory therapeutic situation is of utmost importance for successful ABMT. Patients with an unfavourably responding neoplasm should, therefore, be already primary candidates for ABMT.

Adolescent↗

[Changes in the vertebrae as an initial symptom of leukemia].

Back pain due to vertebral changes as early feature of acute lymphocytic leukemia (ALL) in childhood has been infrequently reported. There are 8 previously described patients with similar clinical and laboratory data, suggesting a biologically unique subset of ALL. Characteristic findings of this rare primary manifestation of leukemia are lack of significant organomegaly or lymphadenopathy, normal or low white blood cell count with predominance of lymphocytes and rarely circulating lymphoblasts, normal platelet count, uric acid and lactate dehydrogenase values. In the following report we make a further attempt to confirm the hypothesis of a subset of ALL, demonstrating two additional patients with characteristic features of ALL presenting with vertebral changes and low leukemic burden.

Child, Preschool↗

Hematological and oncological indications for splenectomy in children.

The most common hematologic and oncologic indications for splenectomy in childhood are hereditary spherocytosis, chronic idiopathic thrombocytopenic purpura, hypersplenism, and Hodgkin's disease. Because of the increased incidence of septic complications after splenectomy, benefits to be gained from the operation should be weighed against the risks. A retrospective study was done on the charts of 42 consecutive children with hematologic and oncologic disorders, who underwent splenectomy between 1967 and 1982. The incidence of septic complications after splenectomy was 12%; sepsis, however, only occurred in patients with severe underlying diseases (three patients with Hodgkin's disease, one patient with systemic lupus erythematosus, and one patient with chronic pseudo-malignant immunoproliferation). In contrast, none of the patients who were splenectomized for other reasons (mainly hereditary spherocytosis and chronic immune thrombocytopenic purpura) had a septic complication. Two patients with end-stage Hodgkin's disease (5%) experienced fatal septic complications. Although splenectomy is well established for diagnostic and therapeutic considerations in patients with Hodgkin's disease, not all of them might benefit from this operation, and studies with a more limited approach to splenectomy might prove to be of the same therapeutical value.

Adolescent↗

[Supportive measures in aggressive cytostatic therapy].

Over the past years significant progress has been made in the treatment of childhood cancers due to newer and more intensive chemotherapeutic regimes. However, with the increased intensity of chemotherapy, more treatment related complications are seen, requiring also more aggressive supportive care. The major complications of the cytotoxic treatment are bone marrow aplasia, immunosuppression, vomiting, anorexia and weight loss and supportive measures as adequate blood component supply, prophylaxis, recognition and effective treatment of infections as well as parenteral hyperalimentation are corner stones of modern cancer therapy. Blood sampling, application of blood products or intravenous drugs and continuous parenteral nutrition is easily performed using a central venous line. Our experience with the continuous venous access of central lines in patients receiving aggressive cytotoxic treatment did not show a higher incidence of infectious complications but had significant advantages in the supportive care.

Anemia, Aplastic↗

[Immunosuppressive treatment of severe aplastic anemia with high-dose methylprednisolone and antithymocyte globulin in 2 patients].

This article describes the treatment of two patients with severe aplastic anaemia (SAA) by means of high-dose methylprednisolone ( HDMP ) and antithymocyte globulin (ATG). A complete normalization of the haematological data was obtained in one patient, which has persisted now for 10 months. ATG and HDMP seem to provide an alternative mode of treatment of SAA to bone marrow transplantation if there is no histocompatible bone marrow donor available.

Adolescent↗

Intrathecal mafosfamide therapy for pediatric brain tumors with meningeal dissemination.

The treatment of childhood brain tumors with cerebrospinal fluid (CSF) dissemination is limited by the relative inaccessibility of the CSF to drugs administered systemically and the paucity of available agents for intrathecal therapy. Mafosfamide is a cyclophosphamide derivative, which does not require hepatic activation and thus can be utilized for regional therapy. Between May 1994 and December 1996, 16 patients 2 to 19 (median 12) years old with various disseminated brain tumors were treated with intraventricular mafosfamide via an indwelling subcutaneous reservoir. The patients received mafosfamide at a dose of 20 mg once or twice weekly until remission was achieved, followed by weekly administrations as consolidation therapy, and every 3 to 4 weeks thereafter for maintenance therapy. Except for transient headaches, nausea and vomiting during and immediately after mafosfamide administration no toxicities were observed. Nine of the 16 patients were evaluable for response by CSF cytology. Eight had complete responses and one patient did not respond. In addition to mafosfamide all patients received systemic chemotherapy as well. However, 4 of the 8 responding patients had developed CSF dissemination under concurrent systemic therapy and cleared their CSF only after administration of intrathecal mafosfamide. At a median follow-up of 21 months, 7 patients are in complete and 4 in partial remission, 2 have stable disease and 3 died of tumor progression. We conclude that mafosfamide at a dose of 20 mg can be safely administered into the CSF and may produce responses and prolong remission of the leptomeningeal disease.

Adolescent↗

[Aseptic bone necroses as a late complication following successful treatment of leukemias and severe aplastic anemia].

Aseptic bone necrosis is a well known complication after corticosteroid treatment in adults and several hundred cases have been reported. Alterations in fat metabolism with vascular occlusion due to fat embolization, as well as microtraumata and osteoporosis are discussed as etiologic factors. In contrast, aseptic bone necrosis in relation to corticosteroid treatment is rare in children and adolescents. We therefore report 3 patients, aged from 10 to 18 years, suffering from severe aplastic anemia, meningeal relapse after acute lymphocytic leukemia and acute myelocytic leukemia respectively, who developed aseptic bone necrosis 6, 11, and 20 months following the onset of corticoid therapy. The patients survive from 28+ to 50+ months after diagnosis of their initial hematologic disease, as it can be expected today for increasing numbers of patients. We therefore believe, that aseptic bone necrosis may represent a serious therapy related complication and suggest that, diagnostic examination in patients with suspicious complaints of the hip, shoulder or knee should also exclude the possibility of a bone necrosis after leucemic relapse has been ruled out. Since radiological changes only develop several weeks to months after the onset of the clinical symptoms and because of the disabling consequences for patients, misdiagnosed at the beginning, a 99 technetium bone scan should be done as early as possible. Corticosteroids, despite their serious side effects are still being considered as a important part of hematologic therapy and are not being omitted in the near future, so that the earliest possible diagnosis of bone necrosis will remain of great importance.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Reversible occlusion shunt for intraventricular chemotherapy in shunt-dependent brain tumor patients.

Intraventricular chemotherapy is increasingly used in the treatment of pediatric brain tumors with leptomeningeal seeding. However, some patients are shunt dependent after surgery, probably due to adhesions in the area of surgery. To avoid drug diversion in these patients we connected the reservoir to a reversible occlusion device. Over a 2-year period a shunt value with an on-off device was inserted into the shunt assembly of eight children with various brain tumors with a poor prognosis undergoing intraventricular chemotherapy. All eight patients had tumor cells in the ventricular cerebrospinal fluid (CSF) and/or metastases by magnetic resonance imaging. The number of intraventricular drug applications ranged from 10 to 51. No shunt malfunctions or shunt-related infections occurred. The temporary closure of the shunt after drug delivery was well tolerated. In all six children with tumor cells in the ventricular CSF a negative cytology was achieved over a 3- to 8-week period.

Adolescent↗

MR findings in methotrexate-induced CNS abnormalities.

MR of the brain was performed in eight patients (mean age, 14.9 years) with osteogenic sarcoma during or after IV treatment with high-dose methotrexate. MR detected brain abnormalities in four patients, three of whom had concomitant neurologic dysfunction. Pathologic findings demonstrated on MR were (1) chronic brain edema, demonstrable over a period of 3-14 months (proved by autopsy in one patient); (2) multifocal white matter necrosis; and (3) deep brain atrophy. MR appears to be valuable in the detection of abnormalities induced by treatment with high-dose methotrexate.

Adolescent↗