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Biomedical subjects

R Dopfer

Publications and source records attributed to R Dopfer.

At least 19 recordsLinked to original sources
PubMed1998

Bone marrow scintigraphy with technetium-99m anti-NCA-95 to monitor therapy in malignant osteopetrosis.

We report a case of a 2-mo-old girl with malignant osteopetrosis. Conventional radiological investigations of the skull and left hand showed the characteristic pattern of generalized sclerosis. Bone marrow immunoscintigraphy with 99mTc-labeled antibodies against nonspecific cross-reactive antigen (NCA) 95 was performed before and after bone marrow transplantation. Before transplantation, whole-body images showed bone marrow stores exclusively in the base of the skull. The rest of the skeleton did not reveal any hematopoietic activity. The liver and spleen showed increased antibody uptake as expected in extramedullary hematopoiesis. Repeat scintigraphy after bone marrow transplantation from her haploidentical father demonstrated an almost completely normalized tracer distribution corresponding to her clinical and hematological improvement. Bone marrow immunoscintigraphy appears to be an ideal complement to radiograph diagnostics in malignant osteopetrosis. In primary diagnosis, scintigraphy demonstrates the quantitative extent of bone marrow displacement. It also proves an ideal tool in monitoring the effectiveness of therapy after bone marrow transplantation.

Antibodies, Monoclonal↗
PubMed1997

CNS late effects after ALL therapy in childhood. Part I: Neuroradiological findings in long-term survivors of childhood ALL--an evaluation of the interferences between morphology and neuropsychological performance. The German Late Effects Working Group.

The effect of cranial irradiation on possible therapy-induced morphological central nervous system (CNS) side effects of children cured from acute lymphoblastic leukemia (ALL) is controversially discussed. In a retrospective multicenter study, 118 former ALL patients in first continuous remission were investigated using cranial computerised tomography (CCT) or magnetic resonance imaging (MRI) scans to evaluate CNS related impairments. Corresponding to the different kinds of CNS prophylaxis, the patient sample was divided: group A (n = 39) receiving intrathecal methotrexate (ITMTX) and systemical medium-high-dose methotrexate (SMHDMTX), group B (n = 41) cranial irradiated (in mean 16.8 Gy) and administering ITMTX and SMHDMTX, group C (n = 38) irradiated (in mean 17.1 Gy) and getting ITMTX. Pathologic scans showed atrophy, leukoencephalopathy, calcifications or grey matter changes. These findings were compared with the neuropsychological test results. Abnormal MRI or CCI scans were found in 61/118 patients (51.7%). Fifteen belonged to group A (38.5%), 23 to B (56.1%) and 23 to C (60.5%). Patients with definite CNS changes show reduced neuropsychological test results. The prevalence of brain alterations seems to appear twice increased after lengthening the posttherapeutic interval in irradiated patients as in nonirradiated patients. Irradiated patients as an age younger than 2 years at diagnosis may show a lower prevalence for developing CNS alterations. CNS alterations are not sex-related. Children treated with cranial irradiation in combination with SMHDMTX and/or ITMTX were at greater risk of developing morphological brain alterations than patients with chemotherapy alone. These alterations are partly correlated with reduced neuropsychological performances and seem to stay with a longer posttherapeutic interval.

Adolescent↗
PubMed1997

Allogeneic bone marrow transplantation for a subset of children with acute lymphoblastic leukemia in third remission: a conceivable alternative?

In the BFM Relapse Study registry we retrospectively identified 136 patients with a first marrow relapse who had undergone BMT in second complete remission (CR2) (group A) and 33 patients who received transplants only after a 2nd bone marrow (BM) relapse had occurred (group B). Event-free survival (EFS) rates at 6 years after BMT were 0.49 +/- 0.05 and 0.48 +/- 0.09 for patients transplanted in CR2 and CR3, respectively. In context with the BFM chemotherapy trials for relapsed childhood ALL there is a clear benefit from BMT in 2nd CR for children with unfavorable prognostic features (isolated early BM relapse, very early BM relapse or BM relapse of T cell ALL). Similar control of leukemia can be achieved with either chemotherapy or BMT in late BM relapse of ALL. Assuming a 60% failure rate with chemotherapy for patients in second relapse, a third remission can be achieved in about 60% of patients who have received chemotherapy, rendering them eligible for BMT in 3rd CR. With this strategy 58% of these patients would survive and late sequelae of BMT be restricted to a minority. To withhold BMT in CR2 and not perform BMT before a 2nd BM relapse has occurred, may be a conceivable alternative for children with late ALL BM relapse, at least if no related donor is available.

Adolescent↗
PubMed1996

Expression of PKC isozyme and MDR-associated genes in primary and relapsed state AML.

For investigation of relative differences in mRNA expression levels and of correlations in the expression of genes possibly involved in multidrug resistance (MDR) of acute myelogenous leukemias (AML), a complementary DNA polymerase chain reaction (cDNA-PCR) analysis was established for the genes encoding MDR1/P-glycoprotein, the multidrug resistance-associated protein (MRP), topoisomerase II alpha, topoisomerase II beta, topoisomerase I, glutathione S-transferase pi, protein kinase C (PKC) isozymes alpha, beta 1, beta 2, epsilon, eta, theta and cyclin A. In a first descriptive study comprising samples of childhood or adult AML we calculated the mean values from primary (n=14) or relapsed (n=23) states of the diseases, respectively. We found in the latter significant increases of MDR1, MRP, gst pi, and PKC theta gene expression. MDR1 and MRP gene expression levels were generally correlated (rs= +0.4128, P<0.02, n=37), as well as topoisomerase II alpha and cyclin A gene expression levels (rs= +0.8727, P<0.0001, n=35). Within the group of relapsed state AML a significant negative correlation between the gene expression levels of MDR1 and topoisomerase II alpha (rs= -0.5500, P<0.01, n=22) was observed. Remarkably, highly significant positive correlations were found for MDR1/PKC eta (rs= +0.5560, P<0.001, n=32), MRP/PKC theta (rs= +0.6573, P<0.0001, n=34) and MRP/PKC eta (rs= +0.5241, P<0.005, n=32).

ATP Binding Cassette Transporter, Subfamily B, Mem↗
PubMed1996

[Differential diagnosis of childhood osteomyelitis--classification according to scintigraphic, radiologic and magnetic resonance tomographic characteristics].

We present a retrospective study on children with the final diagnosis osteomyelitis, who have been examined in Tübingen from 1985 to 1991. The different types of infantile osteomyelitis were classified due to the causative organism and findings in 3-phase scintigraphy and X-ray films. For the chronic type of osteomyelitis the study was extended to the years from 1979 to 1991 and the results of an earlier report were included. We worked up 17 cases of acute/peracute osteomyelitis, including 5 cases of early infancy, 2 cases of tuberculosis, 2 Brodie's abscesses, 5 plasmacellular types, 2 cases of primary chronic multifocal osteomyelitis (PCMO), and 5 cases of unspecific chronic osteomyelitis. All cases were examined with scintigraphy, X-ray films and in part with magnetic resonance tomographic imaging. In 23 cases scintigrams and X-ray films were performed in the follow-up. We show the importance of scintigraphy for the early detection and localisation of osteomyelitis, the importance of findings on X-ray films for the specific diagnosis of osteomyelitis, and the importance of magnetic resonance tomography for high-resolution detection of the expansion of osteomyelitis.

Acute Disease↗
PubMed1995

A phase I study of human/mouse chimeric antiganglioside GD2 antibody ch14.18 in patients with neuroblastoma.

9 patients with stage IV neuroblastoma were treated with 19 courses of human/mouse chimeric monoclonal antiganglioside GD2 antibody ch14.18 at dose levels of 30, 40 and 50 mg/m2/day for 5 days per course. The maximum tolerated dose (MTD) per injection was 50 mg/m2/day. 7 patients received more than one course of treatment, and none revealed any human anti-mouse antibody (HAMA) response. Clinical side-effects of patients treated with ch14.18 were abdominal and joint pains, pruritus and urticaria. One patient presented with a transient pupillatonia, while 2 others showed a unilateral atrophy of the optical nerve that was probably attributable to prior therapies. A complete remission was seen in 2 patients, partial remission in 2 patients, a minor response in 1 patient and stable disease in 1 patient. 3 patients showed tumour progression. Thus, our results indicate that treatment with chimeric MAb ch14.18 can elicit some complete and partial tumour responses in neuroblastoma patients.

3-Iodobenzylguanidine↗
PubMed1995

Autologous transplantation with peripheral blood stem cells in children and young adults after myeloablative treatment: nonrandomized comparison between GM-CSF and G-CSF for mobilization.

Recombinant human granulocyte-monocyte colony-stimulating factor (rhGM-CSF) was compared with recombinant granulocyte colony-stimulating factor (rhG-CSF) in 34 patients for mobilization of peripheral blood stem cells (PBSC) and for posttransplantation use. Peripheral blood stem cell mobilization was initiated by a single 1 h infusion of cyclophosphamide (4 g/m2) in all patients, followed by either a continuous infusion of rhGM-CSF (250 micrograms/m2/day) in 17 patients (group A) or a daily subcutaneous injection of rhG-CSF (10 micrograms/kg/day) in 17 patients (group B). PBSC were collected using a Fenwal CS 3000 continuous flow blood cell separator in one to three sessions. All patients suffered from various childhood malignancies. No difference in the number of collected cells among both groups was found. A mean of 2.7 x 10(8)/kg mononuclear cells (MNC) and of 7.9 x 10(4)/kg CFU-GM (colony-forming unit-granulocyte-macrophage) were collected in group A. In group B, 2.3 x 10(8)/kg MNC and 11.8 x 10(4)/kg CFU-GM were collected. In 33 patients, PBSC were reinfused after myeloablative therapy. Patients of group A (n = 17) were treated with rhGM-CSF (250 micrograms/m2/day) starting day +1, and patients in group B (n = 16) were treated with rhG-CSF (10 micrograms/kg/day) i.v. All patients showed a rapid and complete hematopoietic recovery without significant differences in both groups. Time to achieve 0.5 x 10(9)/L granulocytes was 10.9 days in group A and 11 days in group B.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1995

Expression of mdr1, mrp, topoisomerase II alpha/beta, and cyclin A in primary or relapsed states of acute lymphoblastic leukaemias.

In a series of 60 ALL samples drawn during different stages of the disease we used a cDNA-PCR approach to analyse the relative mRNA levels of the MDR-associated genes encoding mdr1/P-glycoprotein, mrp, and the topoisomerase II isozymes alpha and beta. Expression analysis of the cyclin A gene was included to examine cellular proliferation activity. The expression of gapdh served as an internal standard. Calculating the mean values we found: (i) a distinctly lower mdr1 gene expression in primary ALL and first relapses compared to bone marrow from healthy donors, (ii) no change in mdr1 and mrp, but a decreased topoisomerase II alpha gene expression in first relapses of ALL compared to the primary leukaemia, and (iii) increased mdr1 and mrp levels combined to decreased topoisomerase II alpha levels in recurrent relapses of ALL showing significant correlations (mdr1/mrp: rs = +0.6833, P < 0.05; mdr1/topoII alpha: rs = -0.6727, P < 0.05). The expression of the topoisomerase II alpha gene was correlated to that of cyclin A, indicating a link of its expression to cellular proliferation. Our findings suggest that a multifactorial MDR including mrp appears particularly in recurrent relapses of ALL, which often do not respond to chemotherapy. Nonetheless, some individual samples showed gene expression levels very different from the mean values calculated for a particular state of the leukaemia, indicating the need of an individual expression analysis of MDR-associated genes.

ATP Binding Cassette Transporter, Subfamily B, Mem↗
PubMed1994

Rapid method for purification of CD56+ natural killer cells with preferential enrichment of the CD56bright+ subset.

A rapid method for the purification of CD56+ natural killer (NK) cells with the preferential enrichment of the CD56bright+ subset is described. The method is based on the adsorption of CD56+ cells indirectly stained with a biotinylated antibody on an avidin-coated column. The adsorbed CD56+ cells can then be squeezed out mechanically without damaging the viability or the function of the cells. Starting from peripheral mononuclear cells from adult donors, the recovery of the CD56+ cells was 11.9 +/- 9.0% (n = 8), the purity 93.0 +/- 4.9% (n = 10), and the enrichment factor 7.5 +/- 1.7 (n = 8). Further phenotypic classification of the CD56+ cells into CD56dim+ and CD56bright+ cells showed a preferential enrichment of the CD56bright+ phenotype with a recovery of 40.5 +/- 19.6% (n = 8), a purity of 30.3 +/- 13.2% (n = 8), and an enrichment factor of 29.8 +/- 7.2 (n = 8). In conclusion, the described method allows the rapid purification of CD56+ NK cells with the preferential enrichment of the CD56bright+ subset.

Adult↗
PubMed1994

[Diffuse xanthogranuloma as a cause of infantile heterochromia].

A 7-month-old female infant presented with a heterochromia and an anisocoria. A spontaneous hyphema and a secondary glaucoma had developed. Under topical and systemic steroid therapy the eye improved continuously. At the time of the last ocular examination the hyphema had resorbed, and the intraocular pressure was normal. Heterochromia had almost completely vanished, and dilation of the pupil was much better. Since even a massive juvenile xanthogranuloma of the iris responds well to steroids, operative and radiation treatment should be limited to cases refractory to antiinflammatory drug therapy.

Administration, Oral↗
PubMed1994

Successful treatment strategy for Ki-1 anaplastic large-cell lymphoma of childhood: a prospective analysis of 62 patients enrolled in three consecutive Berlin-Frankfurt-Munster group studies.

PURPOSE: To prove prospectively the efficacy of a short-pulse chemotherapy for treatment of Ki-1 anaplastic large-cell lymphoma (ALCL) of childhood. PATIENTS AND METHODS: From October 1983 to December 1992, 62 patients (median age, 9.7 years) with newly diagnosed Ki-1 ALCL were enrolled onto Non-Hodgkin's Lymphoma-Berlin-Frankfurt-Munster (NHL-BFM) studies 83, 86, and 90. The most frequent immunophenotype was T cell. Ki-1 ALCL differed from other subsets of NHL of childhood by the more frequent involvement of bone, soft tissue, and skin, and by the lack of bone marrow (BM) disease. A 5-day prephase course (prednisone/cyclophosphamide) was followed by two different 5-day courses of chemotherapy: course A consisted of dexamethasone, methotrexate (MTX) 0.5 g/m2 (24-hour infusion), intrathecal chemotherapy, ifosfamide, cytarabine (Ara-C), and etoposide (VP-16); course B consisted of cyclophosphamide and doxorubicin instead of ifosfamide, and Ara-C/VP-16, respectively. Treatment was stratified into three branches. Branch 1 (stage I and stage II resected) received three courses; branch 2 (stage II not resected, stage III), six courses; and branch 3 (stage IV), six intensified courses containing MTX 5 g/m2, and Ara-C 2 g/m2. Local radiotherapy was not performed. RESULTS: Four patients failed to enter remission, and one died of infection. Seven patients relapsed within 9 months after diagnosis; two patients had isolated local relapses, but BM and CNS were never involved. Fifty patients have been in first continuous complete remission (CR) for 0.6 to 9.7 years (median, 2.5), and 56 are alive. The probabilities for survival and event-free survival (EFS) at 9 years are 83% +/- 7% (SE) and 81% +/- 5%. Skin involvement was the only negative prognostic parameter. CONCLUSION: Short-pulse chemotherapy over 2 to 5 months without local therapy modalities is effective in the treatment of Ki-1 ALCL.

Adolescent↗
PubMed1994

Immunoglobulin high-dose therapy: RBC-alloantibodies in commercial preparations and haemolytic anaemia: a case report.

A 4-year-old girl with idiopathic thrombocytopenia, who received immunoglobulin (Ig) in high dosage, developed haemolytic anaemia. Incompatible alloantibodies but no autoantibodies were found in her blood. In vitro the Ig preparation led to a strong fixation of alloantibodies and C3 on the girl's RBCs. Because of these reactions we investigated 12 Ig preparations and found irregular alloantibodies in 7 of them. The guidelines for quality assurance and other possibilities for lowering side reactions are discussed.

Anemia, Hemolytic↗
PubMed1993

Superior prognosis in combined compared to isolated bone marrow relapses in salvage therapy of childhood acute lymphoblastic leukemia.

Three hundred twenty-six children with bone marrow (BM) relapse of non-B acute lymphoblastic leukemia (ALL) were stratified according to the time of relapse in three consecutive multicenter trials--ALL-REZ BFM 83, 85, and 87. Employing an intensive polychemotherapy regimen, extramedullary involvement appeared to be predictive of superior outcome in both strata as well as in the whole group (probability of 7-year event-free survival (EFS) 42% in combined vs. 15% in isolated BM relapse, P = 0.015). Children with combined BM relapse occurring later than 6 months after completion of front-line therapy reached EFS estimates of 60%. We conclude that results of conventional polychemotherapy with BFM relapse protocols are equivalent to those achieved by bone marrow transplantation in children with late combined BM relapse.

Adolescent↗
PubMed1993

Epidermolysis bullosa acquisita: efficacy of high-dose intravenous immunoglobulins.

A 16-year-old boy had a 6-year history of a generalized bullous eruption that was resistant to multiple therapies. Findings of immunofluorescent split-skin studies and electron microscopy were consistent with a diagnosis of epidermolysis bullosa acquisita. Treatment with cyclosporine and prednisolone decreased new blister formation. Additional therapy with high-dose intravenous immunoglobulins was successful in controlling the patient's disease.

Adolescent↗
PubMed1993

[BCGitis as a cause of mediastinal tumor].

In a one year old female infant BCG-vaccinated as a neonate, a large mediastinal mass was found after routine examination. X-ray and CT scan suggested a teratoma. Histology and microbiology, however, revealed the diagnosis of an active tuberculosis. Thus, the tumor was caused by mediastinal BCGitis. To our knowledge this complication of BCG-vaccination has not yet been described.

BCG Vaccine↗
PubMed1993

[Cryptosporidium enteritis].

Cryptosporidium has been found to be a common cause of gastroenteritis during the last years. The natural course of the disease in immunocompromised patients is often met by serious complications. A general therapy recommendation has not been reported so far. We report of a two-year-old boy, who was immunocompromised by a multidrug cytostatic chemotherapy. The patient suffered from a long lasting most severe gastroenteritis, caused by cryptosporidium. There was a good clinical response to spiramycin, a treatment suggested in the literature.

Antineoplastic Agents↗