Granulocyte transfusions in children.
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Biomedical subjects
Publications and source records attributed to D Niethammer.
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Within 1 year 74 children with neuroblastoma were registered, 30 patients with stage I-III (= 41%) and 44 with stage IV-metastatic disease (= 59%). An aggressive chemotherapy regimen employing Adriamycine, Cyclophosphamide, Vincristine, and Dacarbazine yielded 10/24 partial and 9/24 complete remissions after 9 weeks. 5/24 children were treated less than 9 weeks so far. At the end of the chemotherapy protocol (week 33) 6 recurrences were observed; 3 of these children died. 5 patients remained in complete remission, 1 in partial remission. 12/24 of patients were not evaluable because of treatment less than 33 weeks so far. The one year run of the study is too short to evaluate the benefit of Interferon (randomized trial). The toxicity of the regimen is tolerable, including bone marrow depression, vomiting and hyperpyrexia. Breaking off therapy was only necessary in one patient.
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Bone marrow transplantation is a therapeutical procedure which contains various problems. The first part of this paper deals with some of the complications like the extreme susceptibility to infections in the posttransplant period and the graft-versus-host reaction. The different modes of radiation are discussed in addition. The second part deals with the indications for bone marrow transplantation in aplastic anemia and acute leukemia. In the third part the situation in Germany is described. Early contact with one of the four transplant centres is necessary to assure the best therapeutical approach. Finally some ethical problems are discussed.
A case of a pulmonary blastoma is presented. The tumor's histological demarcation is difficult and contested. The diagnosis was delivered by two independent pathologists. Because of the malignancy of the tumor we decided on a combined surgical-cytostatic-radiological therapy, which has not yet been done in this way. The patient was symptom-free for 6 months, she then died however, from a brain metastasis.
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6 years after the beginning of acute lymphatic leukemia and 3 years after the end of its therapy M. Hodgkin stage Ia was diagnosed in a then 18-years-old girl. From the same location--left neck--enlarged lymph nodes had been removed already during the maintenance therapy of leukemia. A malignant disease was neither proven nor excluded at that time, when signs of immunodepression were present. The patient is now in complete remission for both diseases.
Three children with severe combined immunodeficiency have been isolated for 19-22 months. Two of the children have been placed into the isolation system directly after delivery; on child entered the isolation system at the age of 5 months. The influence of this situation on the psychic development of the children was studied by the means of direct observation as well as by psychometric test methods. An additional child which has been isolated between the age of 6 and 32 months was retested at the age of 4 and 6 years. The reported observation which include those of the parents and the team allow the conclusion, that a normal development is possible under these extreme conditions, when an optimal clinical and psychological support is maintained.
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A 12-year-old boy with aplastic anemia developed severe but reversible cardiac failure after treatment with 200 mg/kg cyclophosphamide (4 x 50 mg/kg on four consecutive days) given as preparation for bone marrow grafting. This and previously reported cases demonstrate the possibility of cardiotoxicity after high doses of the drug.
Two children with primary malignant lymphoma of the bone and the progress of the disease are presented. There was a delayed diagnosis in both children. The tumors were first misinterpreted as being benign (epipiphysiolysis capitis femoris atheroma) and later on as being malignant (Ewing's sarcoma neuroblastoma). The course of the disease in one child (leukemia transformation) confirmed finally the diagnosis of malignant lymphoma.
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A patient with severe combined immunodeficiency received three transplants of bone marrow from the HLA-B- and -D-identical mother. The first transplantation led to a severe graft-versus-host reaction followed by immunological reconstitution. A split chimerism was found with engraftment only of the maternal lymphocytes. Five months after the transplantation an autoimmune hemolytic anemia was observed which was due to rhesus incompatibility as well as polyspecific antibodies. At the same time agranulocytosis developed and 9 mth after the first transplantation the child suffered from aplastic anemia. Two further attempts failed to engraft the maternal hematopoiesis. The child died during the treatment with cyclophosphamide as conditioning for a third transplantation.
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