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  • 1
    Electronic Resource
    Electronic Resource
    Der primitive neuroektodermale Tumor (1997)
    Springer
    Der Chirurg 68 (1997), S. 732-737 
    Details
    ISSN: 1433-0385
    Keywords: Key words: Soft tissue neoplasm ; PNET ; Genetic translocation. ; Schlüsselwörter: Weichteiltumoren ; PNET ; genetische Translokation.
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung. Der periphere primitive neuroektodermale Tumor (PNET) ist ein sehr seltener hochmaligner Weichteiltumor bei Kindern und jungen Erwachsenen. Er resultiert aus einer balanzierten reziproken Translokation an den q-Armen der Chromosomen 11 und 22, die zur malignen Entartung pluripotenter Zellen der Neuralleiste während der Embryogenese führt. Die Behandlung dieses undifferenzierten, kleinzelligen Tumors erfolgt gemäß der Kooperativen Weichteilsarkomstudie (CWS) der Deutschen Gesellschaft für Pädiatrische Onkologie: Durch eine präoperative Chemotherapie mit einem EVAIA-Schema gelingt in 95 % der Fälle eine Remission, wodurch ein verstümmelnder Eingriff vermieden und das Risiko einer intraoperativen Tumorzellaussaat verringert wird; nach Resektion des Resttumors sind eine Nachbestrahlung des Tumorbetts sowie weitere Chemotherapiezyklen vorgesehen. Ist wegen eines Querschnittsyndroms beim paravertebral gelegenem PNET eine primäre Operation erforderlich, ist unmittelbar postoperativ eine Chemotherapie, gefolgt von einer „Second-look-Operation“ und Nachbestrahlung obligat. Gemeinsam mit unseren Pädiatern und Strahlentherapeuten behandelten wir zwischen 1986 und 1994 10 Patienten. Bei 4 der zwischen 4 und 26 Jahre alten Patienten (Median: 14 Jahre) nahm der PNET seinen Ursprung von der Thoraxwand, bei 6 Patienten war er paravertebral-retroperitoneal lokalisiert. Fünf Patienten verstarben nach durchschnittlich 20 Monaten, 5 Patienten befinden sich nach 31, 46, 50, 51 und 91 Monaten in Vollremission.
    Notes: Summary. The primitive neuroectodermal tumor is a rare soft tissue neoplasm occurring in children and young adults. It derives from a carcinogeneic alteration of pluripotent neural crest cells, caused by a balanced reciprocal translocation t(11;22) (q24;q12). Treatment of this undifferentiated, extremely malignant small cell tumor is carried out in compliance with the soft tissue trail (CWS) from the German Society of Pediatric Oncology. Biopsy-proven diagnosis is followed by primary chemotherapy, which in 95 % of cases leads to remission, allowing excision of the remainder of the tumor without mutilation and avoidance of intraoperative tumor cell dissemination. After excision, irradiation of the tumor site and two further sequences of chemotherapy are performed. If PNETs of the paravertebral region cause symptoms of paralysis and immediate surgery is required, postoperative chemotherapy, a second-look operation and irradiation are mandatory. Between 1986 and 1994, in cooperation with our pediatric and radiotherapy colleagues, we treated ten patients. In four patients (median age, 14 years) the PNET originated from the chest wall, in six patients from the paravertebral and retroperitoneal region. Five patients died after 20 months on average, while the remaining five patients are in full remission after 31, 46, 50, 51 and 91 months, respectively.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001040050262
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  • 2
    Electronic Resource
    Electronic Resource
    Secondary acute myeloid leukemia with translocation (4;11) and MLL/AF4 rearrangement in a 15-year-old boy treated for common acute lymphoblastic leukemia 11 years earlier (1995)
    Springer
    Annals of hematology 70 (1995), S. 31-35 
    Details
    ISSN: 1432-0584
    Keywords: Secondary leukemia ; Translocation t(4,11) MLL/AF4 rearrangement ; PCR
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Secondary acute myeloid leukemia occurring in a 15-year-old boy 11 years after initial treatment of a common lymphoblastic leukemia (c-ALL) is described. Initial complete remission was terminated after 4 years by an isolated testicular relapse, followed by first bone marrow relapse within 18 months. After he achieved remission again, an allogeneic bone marrow transplantation from his HLA-identical brother was performed. Five years and 9 months later, the patient developed thrombocytopenia, leukopenia, and anemia, but bone marrow biopsies at this time demonstrated only myelofibrosis, with no blast cell population present. A polymerase chain reaction assay of a peripheral blood sample recognized the mRNA fusion region for the MLL/AF4 rearrangement, i.e., the molecular equivalent of the translocation (4;11)(q21,q23). Four weeks later, a blast cell population with AML-M1 morphology according to the FAB classification appeared in the bone marrow, and translocation (4;11) was detected by cytogenetics. Thus, secondary leukemias with chromosomal 11q23 rearrangement can develop after a long latency period and can be diagnosed earlier with the PCR technique.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01715379
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  • 3
    Electronic Resource
    Electronic Resource
    Secondary acute myeloid leukemia with translocation (4;11) and MLL/AF4 rearrangement in a 15-year-old boy treated for common acute lymphoblastic leukemia 11 years earlier (1995)
    Springer
    Annals of hematology 70 (1995), S. 31-35 
    Details
    ISSN: 1432-0584
    Keywords: Key words Secondary leukemia ; Translocation t(4;11) ; MLL/AF4 rearrangement ; PCR
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary  Secondary acute myeloid leukemia occurring in a 15-year-old boy 11 years after initial treatment of a common lymphoblastic leukemia (c-ALL) is described. Initial complete remission was terminated after 4 years by an isolated testicular relapse, followed by first bone marrow relapse within 18 months. After he achieved remission again, an allogeneic bone marrow transplantation from his HLA-identical brother was performed. Five years and 9 months later, the patient developed thrombocytopenia, leukopenia, and anemia, but bone marrow biopsies at this time demonstrated only myelofibrosis, with no blast cell population present. A polymerase chain reaction assay of a peripheral blood sample recognized the mRNA fusion region for the MLL/AF4 rearrangement, i.e., the molecular equivalent of the translocation (4;11)(q21,q23). Four weeks later, a blast cell population with AML-M1 morphology according to the FAB classification appeared in the bone marrow, and translocation (4;11) was detected by cytogenetics. Thus, secondary leukemias with chromosomal 11q23 rearrangement can develop after a long latency period and can be diagnosed earlier with the PCR technique.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01715379
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    Paper (German National Licenses)
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  • 4
    Electronic Resource
    Electronic Resource
    Severe combined immunodeficiency: treatment by bone marrow transplantation in 15 infants using HLA-haploidentical donors (1985)
    Springer
    European journal of pediatrics 144 (1985), S. 125-130 
    Details
    ISSN: 1432-1076
    Keywords: Severe combined immunodeficiency ; Bone marrow transplantation ; HLA-haploidentical donors
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In 15 infants with severe combined immunodeficiency (SCID), immunological reconstitution was attempted by bone marrow transplantation (BMT) from HLA-haploidentical parents. To prevent graft versus host disease (GvHD), marrow grafts were depleted of contaminating T-lymphocytes using lectin agglutination and rosette formation with sheep red blood cells. Thirteen patients received transplants without undergoing prior cytoreductive conditioning. Eleven of these developed donor-dependent T-cell functions, two failed to do this. One of these two as well as two further patients received cytoreductive treatment prior to repeat and to first transplants and in two, complete lymphohemopoietic reconstitution was observed. Of the 15 patients who received transplants, 11 are currently alive. Two recently treated patients remain in the hospital, nine are at home with stable T-cell functions. Normal humoral immune functions have developed upto now in three patients. In the others, gammaglobulins are regularly substituted. Complications of acute or chronic GvHD were not observed with the exception of one case who developed transient GvHD of the skin. These results suggest that in a majority of patients with SCID, T-cell functions can develop without GvHD following haploidentical, T-cell-depleted BMT. Exceptional patients require preconditioning to allow donor cell engraftment, an approach that also appears to facilitate reconstitution of humoral immune functions
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00451897
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  • 5
    Electronic Resource
    Electronic Resource
    HLA-haploidentical bone marrow transplantation in three infants with adenosine deaminase deficiency: Stable immunological reconstitution and reversal of skeletal abnormalities (1989)
    Springer
    European journal of pediatrics 149 (1989), S. 104-109 
    Details
    ISSN: 1432-1076
    Keywords: Adenosine deaminase deficiency ; HLA-haploidentical bone marrow transplantation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Three infants with severe combined immunodeficiency and adenosine deaminase (ADA) deficiency were treated by T-cell depleted bone marrow transplantation (BMT), using human leukocyte antigen (HLA)-haploidentical parents as donors. In the first patient, two initial transplants failed to engraft and no change of the immunodeficiency was observed. In order to overcome this graft resistance, cytoreductive conditioning was used prior to a third transplant. In the other two patients, similar conditioning was used prior to initial transplants. In all three patients, complete and permanent immunological reconstitution was observed and they survive from 3.5 to 5 years after transplantation. In biopsies obtained from iliac bones prior to BMT, osteochondral abnormalities characteristic of ADA-deficiency were noted in all three patients. After successful transplantation, these abnormalities had completely resolved. Our results demonstrate that cytoreductive conditioning prior to HLA-haploidentical BMT is useful in order to obtain stable engraftment and reversal of abnormalities associated with ADA deficiency.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01995857
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    Paper (German National Licenses)
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