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R Blütters-Sawatzki

Publications and source records attributed to R Blütters-Sawatzki.

15 recordsLinked to original sources
PubMed2001

Peripheral primitive neuroectodermal tumor: challenge for multimodal treatment.

The primitive neuroectodermal tumor (PNET) is an extremely aggressive soft tissue neoplasm that occurs in children and adolescents. We retrospectively reviewed our therapeutic experience with a multidisciplinary approach, combining surgery, chemotherapy, and radiation therapy. Treatment of PNET was carried out in compliance with the soft tissue protocol (CWS) from the German Society of Pediatric Oncology. Biopsy-proven diagnosis was followed by chemotherapy, which in all cases led to partial remission, allowing excision of the remainder of the tumor without mutilation. After excision, irradiation of the tumor site and two further sequences of chemotherapy were performed. When PNET of the paravertebral region caused symptoms of paralysis and immediate surgery was required, postoperative chemotherapy, a second-look operation, and irradiation were undertaken. Between 1986 and 1998 we treated 13 patients (median age 15 years). In five patients the PNET originated from the chest wall and in eight patients from the paravertebral and retroperitoneal region. Five patients died after 20 months on average, and the remaining eight patients are in full remission after 7, 16, 46, 55, 70, 74, 75, and 115 months, respectively. Close cooperation between surgeons and their pediatric and radiotherapy colleagues is obligatory when treating PNET. Chemotherapy as the first stage is mandatory to avoid a mutilating surgical procedure and intraoperative tumor cell dissemination.

Adolescent↗
PubMed2001

Pharmacokinetics of native Escherichia coli asparaginase (Asparaginase medac) and hypersensitivity reactions in ALL-BFM 95 reinduction treatment.

Repeated asparaginase treatment has been associated with hypersensitivity reactions against the bacterial macromolecule in a considerable number of patients. Immunological reactions may range from anaphylaxis without impairment of serum asparaginase activity to a very fast decline in enzyme activity without any clinical symptoms. Previous investigations on a limited number of patients have shown high interindividual variability of asparaginase activity time courses and hypersensitivity reactions in about 30% of patients during reinduction treatment. Therefore, monitoring of reinduction treatment was performed prospectively in 76 children with newly diagnosed acute lymphoblastic leukaemia (ALL). According to the ALL-Berlin-Frankfurt-Münster (BFM) 95 protocol, 10 000 U/m2 body surface area of native Escherichia coli asparaginase (Asparaginase medac) was given on d 8, 11, 15 and 18. In 45/76 children, trough and peak activities were determined with every dose, and also on d 4 and d 11 after the last administration. Data on asparaginase activity were not available from the remaining 31 patients, but information with regard to hypersensitivity reactions only was given. Eighteen out of 76 patients (24%) suffered a clinical hypersensitivity reaction; however, no silent inactivation was observed. Activity in the therapeutic range of greater than 100 U/l for at least 14 d was determined in 43 of the 45 patients who were analysed for enzyme activity.

Adolescent↗
PubMed1999

Leptin and leptin receptor expression in a lipoblastoma in an 8-year-old girl.

Leptin is a hormone that is produced by adipocytes. Leptin acts on specific receptors in the hypothalamus. RNA was isolated from a lipoblastoma of an 8-year-old girl and the expression of leptin and leptin receptor mRNA was analyzed by RT-PCR. The lipoblastoma tumor, a rare form of childhood tumors, expressed leptin and leptin receptors in a fashion similar to normal adipose tissue. We hypothesize that the peripheral action of leptin via its receptors could play a role in the development and/or progression of lipoblastoma. Whether or not leptin and leptin receptor expression play a role in the development and/or progression of lipoblastoma and other tumors is not clear to date. Copyrightz1999S.KargerAG, Basel

Adipocytes↗
PubMed1997

[Primitive neuroectodermal tumor].

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.

Adolescent↗
PubMed1996

[Primary neuroectodermal tumor (PNET): a rare highly malignant soft tissue tumor in children and young adults].

The PNET is an extremely malignant soft tissue neoplasm resulting from a balanced reciprocal translocation t (11; 22) (q24; q12). Treatment of the undifferentiated small cell tumor is carried out in compliance with the protocol of the soft tissue trail (CWS) from the German Society of Pediatric Oncology. Biopsy-proven diagnosis is followed by primary chemotherapy which leads in 95% of cases to remission. After excision of the remainder of the malignancy, an irradiation of the tumor site and two further sequences of chemotherapy are performed. Between 1986 and 1994, in cooperation with our pediatric and radiotherapeutical 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 22, 37, 41, 42 and 82 months, respectively.

Adolescent↗
PubMed1995

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.

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.

Acute Disease↗
PubMed1987

[Treatment of Fanconi anemia by bone marrow transplantation].

We report on our experience with allogenic bone marrow transplantation in the treatment of Fanconi anemia. Eight patients were treated, ranging in age from 5 to 17 years. Beside severe hemopoietic insufficiency, all patients exhibited typical cytogenetic abnormalities with an increased rate of chromosomal breaks, while constitutional signs of the disorder were rather variable. Marrow donors were HLA-identical siblings. For conditioning, we used cyclophosphamide at 5 mg/kg on 4 consecutive days followed by thoraco-abdominal irradiation at 5 Gy with full lung shielding. For prophylaxis of graft versus host disease, cyclosporin A was given except in 3 cases who received T-cell depleted marrow. In 2 of the latter cases, graft failure was observed, successfully reversed in one by retransplantation. All others showed prompt and stable engraftment of donor cells. Complications of graft versus host disease developed in 2, requiring prolonged immunosuppressive treatment. Of 8 transplanted patients, 7 survive. With the exception of a recently treated girl, they have normal stable marrow functions. Our results confirm that successful treatment of Fanconi anemia is possible in a majority of patients with HLA-identical donors.

Adolescent↗
PubMed1985

Severe combined immunodeficiency: treatment by bone marrow transplantation in 15 infants using HLA-haploidentical donors.

In 15 infants with severe combined immunodeficiency (SCID), immunological reconstitution was attempted by bone marrow transplantation (BMT) from HLA-haplo-identical 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, gamma globulins 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.

Bone Marrow Transplantation↗
PubMed1985

[Cytomegalovirus hyperimmune globulin prophylaxis in bone marrow transplants in children with various diseases].

The effect of hyperimmune globulin for the prevention of cytomegalovirus (CMV) infections following bone marrow transplantation (BMT) was evaluated in 21 children with various diseases and compared to a historical group of 23 children without prophylaxis. A higher incidence of interstitial pneumonia (19%) as well as CMV-associated infections with or without pneumonitis (29%) could be demonstrated in patients with CMV-prophylaxis as against the rate of interstitial pneumonia (4%) and CMV-infections (8%) in children without prophylaxis. This surprising observation was very likely due to selection of patients with high risk features and higher incidence of GVHD in the prophylaxis group. The analysis of patients with prophylaxis failure shows a low CMV-infection rate in initially seronegative marrow recipients and a high risk for CMV-infections in seropositive patients. Therefore, even though CMV-infections could not be completely abrogated, hyperimmune globulin administration might have reduced CMV-complications in seronegative patients. In CMV-seropositive marrow recipients, however, this type of prophylaxis remains unsatisfactory in preventing severe CMV-infections caused by virus reactivation.

Adolescent↗
PubMedDate not supplied

[Cutaneous lesions and blood count changes in a 9-month old girl with glutaric aciduria type I].

Non-specific cutaneous lesions are common in patients suffering from acute myeloid leukemia (AML). Leukemic skin infiltrates are present in about 30% of cases of monoblastic or myelomonocytic leukemia. The appearance of specific skin lesions can precede bone marrow involvement. We report the case of a 9-month-old girl with acute myelogenous leukemia (FAB M5) and glutaric aciduria type I which initially presented with cutaneous lesions, anemia and leukopenia.

Amino Acid Metabolism, Inborn Errors↗
PubMedDate not supplied

[Treatment of hemophagocytic lymphohistiocytosis, HLH, with bone marrow transplantation].

Hemophagocytic lymphohistiocytosis (HLH) is a rare disease of infancy and young childhood. The clinical presentation includes recurrent unexplained fever with hepatosplenomegaly. Cytopenia, hypofibrinogenemia and/or hypertriglyceridemia and hemophagocytosis in bone marrow, spleen and lymphnode confirm the diagnosis. Hemophagocytosis may not be present at the beginning. In these cases, diagnosis is facilitated by a positive family history, a relapsing course of the disease, the frequent involvement of the central nervous system and positive findings on immunological work-up. Treatment by chemotherapy and immunosuppressants can achieve sustained remissions in most patients and reinduction of remission after relapse is possible. Most children however, eventually die from progressive disease. At present, allogeneic bone marrow transplantation is the only curative therapeutic option. Between August 1992 and May 1997 eleven consecutive patients with HLH received bone marrow from unrelated (n = 7) or matched sibling donors (n = 4). The conditioning regimen consisted of busulfan, VP-16 and cyclophosphamide. Patients engrafted after a median time of 16 days (13-43). Only one patient developed grade III acute GVHD, another patient, grade II acute GVHD. Although regimen-related toxicity was extensive, all patients have survived without signs of HLH after a median follow up of 20 months (8-63). One patient suffers from chronic GVHD, three patients reveal psychomotoric retardation and one patient has severe impairment with spastic tetraparesis, amaurosis and seizures. Our experience shows that HLH can be successfully treated by allogeneic BMT from unrelated donors.

Bone Marrow↗
PubMedDate not supplied

Severe cardiotoxicity of high-dose 5-fluorouracil in combination with folinic acid, cisplatin and methotrexate in a 14-year-old boy with nasopharyngeal carcinoma (Schmincke tumor).

Cardiotoxicity by 5-fluorouracil has been reported as a rare complication in treatment protocols for adult patients with different malignant diseases. The combination with folinic acid as a biomodulatory substance and other cytotoxic drugs has been introduced to render protocols more effective and less toxic. We report severe but completely reversible cardiotoxicity of a treatment protocol for nasopharyngeal tumor (Schmincke tumor) of pediatric patients consisting of 5-fluorouracil, methotrexate, cisplatin and folinic acid in a 14-year-old boy who had no preexisting cardiac disease. The pathomechanism is briefly discussed.

Adolescent↗