Hemophilia--recent advances in an old disease.
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Factor VIII↗
Biomedical subjects
H Lackner
Publications and source records attributed to H Lackner.
[Actinomycins, XXVI; syntheses of actinomycins and actinomycin-like chromopeptides, I. Actinocinyl-bis-L-threonine].
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Chemical Phenomena↗
[Actinocinyl-bis-peptide].
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Chemistry, Organic↗
[Clinical experiences with adrenaline as therapy and prevention of E. coli-L-asparaginase-induced anaphylaxis].
Management of E.-coli L-asparaginase induced anaphylaxis mainly consists of application of i.v. epinephrine but also in the withdrawal of this drug. Despite of this firm recommendation we have shown in 7 patients with E. coli L-asparaginase induced anaphylaxis, that further application of the drug is possible in the presence of a continuous epinephrine infusion (0.01-0.02 mg/kg) started one hour before and finished one hour after the concomittant infusion of the E.-coli L-asparaginase. In none of the patients there was a second event of anaphylaxis even though most of the patients still had to continue on E.-coli L-asparaginase with an average of more than 6 infusion.
Anaphylaxis↗
The influence of maximum supportive care on dose compliance and survival. Single-center analysis of childhood acute lymphoblastic leukemia and non-Hodgkin's-lymphoma treated within 1984-1993.
BACKGROUND: The administration of intensive, risk-adapted multiagent chemotherapy has markedly improved the event-free survival in childhood acute lymphoblastic leukemia and Non-Hodgkin's lymphoma. Current treatment protocols may achieve complete remission rates of more than 95% and event-free survival rates of approximately 70% in non-B-acute lymphoblastic leukemia/non-B-Non-Hodgkin's lymphoma patients. Due to a higher proliferation rate and aggressiveness of B-cells treatment of B-acute lymphoblastic leukemia/B-Non-Hodgkin's lymphoma must be more intensive, but can be shorter to attain similar event-free survival rates. METHODS: Between 1984 and 1993 79 primarily admitted patients up to 18 years of age (range 0.6-17.9 years) received therapy according to the treatment protocols ALL A 84, ALL 86, NHL 86, ALL 90 and NHL 90 of the Berlin-Frankfurt-Münster Study Group. Intensive phases of treatment were given on an inpatient basis with maximum supportive care in order to achieve the prescribed doses. RESULTS: Median follow-up time from diagnosis is now 8 years (range 40 to 157 months) with a probability of event-free survival of 92% for the entire group. CONCLUSION: Multiagent chemotherapy tailored to the individual risk profile of each patient but given in the prescribed dose is currently the mainstay to achieve high complete remission rates with the aim to cure most acute lymphoblastic leukemia/Non-Hodgkin's lymphoma patients. The administration of intensive supportive care is important for the achievement of complete drug dosage and for the reduction of therapy-related toxicity.
Adolescent↗
Treatment of children with anagrelide for thrombocythemia.
PURPOSE: Essential thrombocythemia (ET) is a rare myeloproliferative disease characterized by hyperproliferation of megakaryocytes and persistent elevation of platelets. Major complications are thrombosis, bleeding, and microvascular occlusive symptoms, sometimes resulting in serious morbidity. In patients with platelet counts > 1000 x 10(9)/l, cytoreduction is important to reduce the risk for thrombotic or hemorrhagic events. However, the frequent side effects and the carcinogenic potential of many cytoreductive agents limit their use, especially in younger patients. Anagrelide, a noncarcinogenic agent with moderate toxicity, has been successfully used in adult patients with ET, but experience with anagrelide in pediatric patients with ET is limited. PATIENTS AND METHODS: Three children who received anagrelide (Induction dose 1 mg/day, maintenance dose 1 to 2.5 mg/day) as therapy for ET are described. RESULTS: All children had a rapid and marked decline of their platelet counts, and only one child developed mild and transient abdominal side effects. CONCLUSIONS: Treatment with anagrelide seems to be a promising approach for children with ET. It should be considered as first-line therapy because of its selective activity against platelet production, tolerable toxicity, suspected negligible cancerigenic effect, and the possibility of oral administration.
Adolescent↗
Adoptive immunotransfer with viable donor mononuclear cells for recurrent chronic myelogenous leukemia after allogeneic bone marrow transplantation in two children.
Two children with Ph+ chronic myelogenous leukemia (CML) relapsed in the chronic phase after allogeneic bone marrow transplantation (BMT). They were treated with transfusions of peripheral blood mononuclear cells (PBMC) obtained from the former bone marrow donors in combination with interferon alfa-2. In one child, CML was successfully controlled as shown by disappearance of Ph+ metaphases as well as negativity for BCR-ABL fusion gene transcripts demonstrated by polymerase chain reaction (PCR) analysis. The patient has remained in complete remission without evidence of disease for 12 months after donor PBMC transfusions. The other child showed disappearance of BCR-ABL gene transcripts by PCR analysis only in peripheral blood cells, but PCR positivity persisted in bone marrow samples. These results indicate that adoptive immunotherapy may be a further alternative in children with relapse of CML after allogeneic BMT as previously described for adult patients.
Adolescent↗
Partial nephrectomy in well-responding stage I Wilms' tumors: report of three cases.
We report on 3 of 21 stage I Wilms' tumor patients with tumor volumes of 190, 890, and 1300 mL, respectively, who responded dramatically to preoperative chemotherapy according to the Austrian/Hungarian Wilms' Tumor Study 89 by tumor volume reductions to 20%, 23%, and 13%, respectively. Radical resection of the tumors with renal preservation was achieved in all patients. Postoperative studies did not show any functional deficit in the preserved kidney. The patients have been in complete remission for 26, 33, and 60 months, respectively. Our preliminary results indicate that tumor resection should be feasible in patients with good responding stage I Wilms' tumors.
Antineoplastic Combined Chemotherapy Protocols↗
Disseminated intravascular coagulation during surgery for scoliosis.
Disseminated intravascular coagulation (DIC) was recently observed intraoperatively in two patients who required correction and stabilization of scoliosis with Harrington instrumentation and spinal fusion. Despite negative bleeding history and normal preoperative coagulation parameters, each patient developed sudden massive bleeding soon after decortication of spinous processes and facet joints. Coagulation profile revealed decreased platelets, plasma coagulation factors, and fibrinogen in association with elevated fibrin split products. Cessation of all bleeding occurred within a few hours. There was rapid correction of the coagulation parameters with blood component replacement therapy, indicating that the defibrination was short-lived and had ceased by the end of surgery. A review of the literature revealed six similar cases of DIC occurring during elective orthopedic surgery, four of which involved spinal arthrodesis and/or bone grafts. We suggest that injury secondary to decortication or chipping at bone can serve as a trigger for defibrination. This type of DIC is self-limited, and ends with completion of the operation. The treatment is blood component replacement. Heparin should be avoided.
Adolescent↗