Images in haematology. Cervical mass after cytotoxic chemotherapy, secondary malignancy or epiphenomenon?
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Antineoplastic Combined Chemotherapy Protocols↗
Biomedical subjects
H Lackner
Publications and source records attributed to H Lackner.
Feasibility of high-dose interleukin-2 in heavily pretreated pediatric cancer patients.
BACKGROUND: The administration of high-dose interleukin-2 (IL-2) seems to be a therapeutic option for children with refractory and metastatic solid malignancies. METHODS: We prospectively studied treatment-related toxicities, quality of life and laboratory parameters in 10 children with progressive or metastatic solid tumors (metastatic osteosarcoma, n=4; neuroblastoma stage IV, n=3; metastatic Ewing's sarcoma, n=2; metastatic Wilms' tumor, n=1) during IL-2 therapy. Patients were scheduled to receive five cycles of high-dose IL-2 by continuous infusion for 5 days every 3 weeks. RESULTS: All patients developed fever >39 degrees C and influenza-like symptoms, with a significant decrease in Karnofsky score. In two patients treatment had to be stopped after three cycles because of severe side-effects. During IL-2 therapy a statistical significant increase in white blood cells (WBC), creatinine, gamma-glutamyltransferase, C-reactive protein, glucose and body weight was observed. In contrast, red blood cells, platelets, protein, albumin and cholinesterase significantly decreased. When results from day 1 of the first and of the fifth cycle were compared, an increase of WBC and a decrease of alkaline phosphatase was shown. No constant quantitative changes in total lymphocytes and subsets were observed during IL-2 therapy. CONCLUSIONS: IL-2 treatment in children with refractory and relapsed solid malignancies is associated with severe, but reversible, side-effects. However, five of the 10 patients with diseases of worst prognosis could be rescued by this treatment.
Adolescent↗
Procalcitonin--a marker of invasive fungal infection?
Procalcitonin (PCT) has been described as a marker of bacterial sepsis. However, little is known of its diagnostic value in fungal infections. We calculated the sensitivity of PCT for detection of invasive fungal infections (IFI) by analyzing 55 episodes of proven or probable IFI (three in our series, 52 reported in the recent literature). In the early phase of IFI, PCT was elevated in fewer than half of invasive candidiasis episodes and in only one patient (5.3%) with invasive aspergillosis. Due to low sensitivity and specificity, PCT adds little to the diagnosis of IFI.
Adolescent↗
Non-radiotherapy conditioning with stem cell transplantation from alternative donors in children with refractory severe aplastic anemia.
Conditioning including total body/lymphoid irradiation is widely used to prevent graft rejection in patients with refractory severe aplastic anemia (SAA) undergoing hemopoietic cell transplantation (HCT) from alternative donors and or after graft manipulation. To reduce regimen-related toxicity we transplanted three children with refractory SAA after conditioning with radiotherapy-free regimens. Conditioning included fludarabine 175-180 mg/m2 in all patients. In addition, patient 1 (failing two previous grafts) received thiotepa 10 mg/kg and Campath-1H 60 mg/m2; patient 2 cyclophosphamide 120 mg/kg, thiotepa 15 mg/kg and OKT-3 0.1 mg/kg/day for 4 weeks; and patient 3 cyclophosphamide 120 and ATG 90 mg/kg. Stem cell source was unmanipulated marrow from the same unrelated donor as for the two previous transplantations in patient 1 and CD34+-purified peripheral blood stem cells from an HLA-matched unrelated donor and from the haploidentical mother in patients 2 and 3. Only patient 1 received graft-versus-host disease (GVHD) prophylaxis with cyclosporine A and mycophenolate mofetil. Follow-up is now 30, 51, and 15 months. None of the patients developed GVHD. All patients have normal counts with complete donor chimerism. Fludarabine-based conditioning is powerfully immunosuppressive and may be used for children with refractory SAA undergoing HCT from alternative donors even after rejection following previous HCT.
Anemia, Aplastic↗
Nephron-sparing procedures in 11 patients with Wilms' tumor.
PURPOSE: In unilateral Wilms' tumor (WT), tumor nephrectomy is the standard surgical approach, whereas partial nephrectomy (PN) is controversially discussed. The aim of our retrospective study was to show that in selected cases of unilateral WT kidney-sparing operations could be a reasonable alternative to nephrectomy and to discuss the results of patients with bilateral WT treated by tumor enucleation. MATERIALS AND METHODS: From 1981 to 1998, seven patients with unilateral nephroblastoma (four stage I, one stage III and two stage IV) had tumor resection by PN (five right side, two left side), which was planned when the tumor volume was reduced after 4 to 6 weeks of chemotherapy by at least 50%, when the tumor occupied one pole or was easily resectable, when 50% or more of the kidney tissue remained and when paraaortic lymph nodes were free by intraoperative histological examination. In four patients with bilateral WT (stage V) bilateral tumor enucleation was carried out-except in one patient in whom the contralateral kidney had to been removed because of extension of the tumor via the inferior vena cava to the right atrium. All patients ( n = 11) received pre- and postoperative chemotherapy followed by radiotherapy in four patients. RESULTS: All patients with unilateral WT ( n = 7) are still alive and disease free (follow-up time: mean 6.6 years, range: 28 months to 11 years) with normal renal function, although two patients with secondary nephrectomy revealed creatinine clearance levels at the lower range. In six patients primary PN was performed successfully. In a stage III tumor patient (intraperitoneal metastasis, free lymph nodes), secondary nephrectomy was necessary due to renal arterial thrombosis 2 days after PN. In one stage IV tumor patient (lung metastasis, free lymph nodes), the primary resection was not far enough away from the tumor margin so that an additional slice of tissue with then tumor-free margins had to be resected. This patient evolved a local relapse 19 months after PN and had to be nephrectomised thereafter. In the group of bilateral WT patients ( n = 4), one child died 2 months after surgery during chemotherapy because of central venous line sepsis. One patient who additionally suffered from inferior vena cava tumor thrombosis extending to the right atrium making nephrectomy of the right kidney necessary developed chronic renal failure 4.7 years postoperatively. The other two stage V tumor patients have creatinine clearance levels within the normal range. CONCLUSIONS: Kidney-sparing procedures remain the operative approach of choice in patients with bilateral WT, but bear the risk of chronic renal failure when one kidney has to be removed. PN in children with unilateral WT, carried out by an experienced surgeon, is a reasonable alternative to nephrectomy if strict guidelines such as excellent tumor response to preoperative chemotherapy and easy resectability far away from the tumor margins through healthy kidney tissue are followed. Paraaortic lymph nodes must be free of tumor invasion in order to avoid local radiotherapy. PN prevents the patient from having to have dialysis in cases of contralateral nephrectomy resulting from metachronous WT or subsequent renal trauma.
Algorithms↗
Simultaneous occurrence of retinoblastoma and neurofibromatosis I in a young child.
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Brain Neoplasms↗
Procalcitonin and C-reactive protein do not discriminate between febrile reaction to anti-T-lymphocyte antibodies and Gram-negative sepsis.
Treatment with antibodies against T-lymphocytes usually triggers a febrile response potentially mimicking or masking infection. Procalcitonin (PCT) is considered a sensitive and specific marker of systemic bacterial and fungal infection. It was the aim of this study to investigate the characteristics of PCT and C-reactive protein (CRP) during treatment with polyclonal or monoclonal anti-T-cell antibodies, in order to examine the ability of these parameters to distinguish between systemic bacterial infection and reaction to antibody treatment. Thus, 15 consecutive febrile episodes after T-cell antibody infusion without clinical signs of infection were compared with nine episodes of Gram-negative sepsis. After T-cell antibody infusion PCT and CRP serum levels increased to a similar extent as in Gram-negative sepsis. Therefore, during T-cell antibody treatment neither PCT nor CRP are adequate for differentiating between fever due to infection or to unspecific cytokine release.
Adolescent↗
ELTEC: ten years of activity (1990-2000)--a report of the International BFM Study Group.
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Adverse Drug Reaction Reporting Systems↗
Is intercostal block for pain management in thoracic surgery more successful than epidural anaesthesia?
OBJECTIVE: Currently epidural anesthesia is the gold standard for postoperative pain management in thoracic surgery. In a prospective randomised study, the effect of an intercostal nerve block applied at the end of the operation was compared to that of epidural anesthesia. METHODS: Thirty patients undergoing thoracotomy were randomised to each group. Patients with resection of the parietal pleura, rib resection and rethoracotomy were excluded from the study. Both groups received non-steroidal anti-inflammatory drugs every 8h as a baseline analgesic medication and were allowed to ask for supplemental subcutaneous opiate injection, limited to four injections per day. The patients in the epidural catheter group (group I) were provided with a motor pump allowing continuous infusion of bupivacain 0.125% and 2mg fentanyl/ml at a dosage of 6-10 ml per hour, dependent on the pain level over a period of 5 days. The patients of the second group (group II) received an intercostal nerve block at the end of the operation reaching from the third to the ninth intercostal space with 20 ml 0.5% bubivacaine. Pain was evaluated with a pain score ranging from 1 (no pain) to 10 (worst pain) twice daily in relaxed position and during physical activity like coughing. On the fifth postoperative day, the patients were asked specific questions concerning the subjective pain experience. Costs of both treatments were calculated. Mean pain values and costs of both groups were compared by t-tests for independent samples. A P value of less than 0.05 was considered significant. RESULTS: Eighteen male and 12 female patients, aged between 35 and 71 years (mean 59) were included in the study. Nineteen patients had lobectomy, five bilobectomy, two decortication and three wedge resection. There were 22 right sided and eight left sided procedures. In group I, the mean pain score on the operation day was 3.95 in relaxed position and 6.33 during physical activity like coughing. The mean pain score during the following 4 days was 2.19 in relaxed position and 4.28 with activity. Three patients required additional subcutaneous opiate injection. In group II, the mean score on the operation day was 2.0 in relaxed position and 3.5 during activity. The mean pain score during the next 4 days was 2.84 in relaxed position and 5.65 with activity. Twelve patients received subcutaneous opiates. In both groups, no complications were observed. COSTS: The costs for treatment of one patient was 105 in group I and 33 in group II. Patients' satisfaction was equal in both groups, there were no differences in terms of outcome and recovery. CONCLUSION: Pain management by intercostal block was superior during the first 24h after surgery whereas on the second day after surgery pain control was significantly better achieved by the epidural catheter in relaxed position. A combination of both forms of anaesthesia seems to be an ideal pain management in patients undergoing thoracic surgery.
Adult↗
Organ tolerance following cadaveric liver transplantation for chronic graft-versus-host disease after allogeneic bone marrow transplantation.
A paediatric patient was treated with orthotopic liver transplantation after he developed cirrhosis of the liver due to chronic graft-versus-host disease (GVHD) following allogeneic bone marrow transplantation. His pre-existing chronic GVHD of the skin disappeared and immunosuppressive therapy could be gradually tapered and finally withdrawn 71 months after liver transplantation. Two and a half years after discontinuation of all immunosuppressive therapy, the patient is in excellent condition with neither signs of chronic GVHD nor rejection of the liver graft.
Anemia, Sideroblastic↗
Outcome and long-term side effects after synchronous radiochemotherapy for childhood brain stem gliomas.
Between 1993 and 1999, 11 children with histologically confirmed diffuse and exophytic brain stem glioma (BSG) were treated with intensive induction chemotherapy and simultaneous external beam irradiation. Chemotherapy was performed according to the German/Austrian Pediatric Brain Tumor Study HIT '91 and included two cycles of ifosfamide (days 1-3), etoposide (days 4-6), methotrexate (days 15 and 22), cisplatin (days 29-31) and cytarabine (days 29-31), separated by a 3-week interval. Maintenance chemotherapy with carmustine, carboplatin and vincristine (8 cycles over a 1-year period) was given in those patients who responded clinically or radiographically to induction chemotherapy. Six of 11 patients showed an objective reduction in tumor size on magnetic resonance imaging and 4 of 11 are alive in good general condition >22, >22, >90 and >92 months, respectively, after diagnosis without radiographic evidence of tumor progression (1 complete remission, 2 partial remissions, 1 stable disease), but suffer from moderate to severe long-term side effects. Three patients died due to disease progression after having achieved a partial remission which lasted 5, 6 and 18 months, respectively, whereas only short-term stabilization was observed in 4 patients who died within 1 year after diagnosis. Acute hematologic toxicity was severe but manageable. This intensive combined modality treatment was toxic but yielded objective responses in more than 50% and long-term survivors in one third of childhood BSG patients.
Antineoplastic Combined Chemotherapy Protocols↗
Tunneled femoral central venous catheters in children with cancer.
OBJECTIVE: We discuss the feasibility of long-term femoral venous access by means of a cuffed subcutaneously tunneled central venous catheter (Broviac catheter) in selected pediatric cancer and stem cell transplant patients in whom access via the veins of the upper part of the torso is difficult or contraindicated and in whom alternative routes must be used. PATIENTS AND METHODS: We report on our experience with 9 patients (3 of whom underwent stem cell transplantation) who received femoral Broviac catheters between December 1990 and November 1999. Results. Time in place ranged from 4 to 155 days with a median of 58 days (mean: 71.2 days). Three catheters had to be removed: 1 because of infection of the subcutaneous tunnel and 2 because of catheter obstruction. The remaining 6 catheters functioned well without problems as long as they were needed; 1 of them got accidentally dislodged while the patient was off treatment. No episodes of catheter-related septicemia, thrombosis, kinking, or drug extravasation were noted; there were no catheter-related infectious complications in the transplant patients. CONCLUSIONS: Our experience indicates that in those instances in which customary access to the superior vena cava is precluded, long-term venous access by way of the femoral vein is a feasible and safe alternative in children, even in the setting of stem cell transplantation.
Adolescent↗
Tumor- and treatment-related side effects after multimodal therapy of childhood intracranial germ cell tumors.
UNLABELLED: Multimodality treatment approaches have dramatically improved the outcome of patients with intracranial germ cell tumors and are resulting in an increasing number of long-term survivors. The aim of the present study was to evaluate prospectively the development of side effects in children, adolescents and young adults after treatment for intracranial germ cell tumors. Nine patients with a median age of 14 y at diagnosis and a median follow-up of 7.25 y underwent a detailed long-term evaluation including physical and neuro-ophthalmologic examinations, routine laboratory and endocrine stimulation tests, neuropsychometric testing, audiometry and spirometry at repeated intervals. Endocrine deficiencies requiring hormone replacement therapy occurred in all patients. Neuro-ophthalmologic side effects were observed in 8 of the 9 patients, urinary electrolyte wasting in 4 of the 9, alopecia in 3 of the 9 and high-frequency hearing loss in 2 of the 9. Neuropsychologic examinations revealed pathologic results in all five tested patients. CONCLUSION: The present study indicates that former intracranial germ cell tumor patients suffer from remarkable long-term side effects, and that some of these late effects can develop or worsen months or years after cessation of oncologic therapy. Since life quality is an important parameter of cancer survival, careful follow-up of long-term survivors is mandatory, aimed at counteracting side effects as early as possible and therefore at minimizing long-term morbidity, which may considerably compromise quality of life.
Adolescent↗
Prospective evaluation of late effects after childhood cancer therapy with a follow-up over 9 years.
UNLABELLED: Intensive multimodality treatment has led to a remarkable improvement of prognosis in paediatric cancer patients, however, a great number of long-term survivors suffer from considerable tumour- or treatment-related late effects. Between January 1990 and December 1998, 223 consecutive survivors of childhood malignancies entered a prospective follow-up study designed to evaluate the frequency and severity of tumour- and/or therapy-related long-term sequelae. After cessation of therapy and subsequently once a year, all patients underwent a detailed examination programme including physical examination, laboratory tests, abdominal sonography, echocardiography, electrocardiography, electroencephalography, spirometry, audiometry, ophthalmological examination and endocrine stimulation tests. Median follow-up was 5 years (range 0.4 to 9.6 years). A total of 167 patients (75%) had at least one chronic medical problem of whom 80 needed permanent medical support. The organ systems most frequently affected were the nervous system in 39%, the endocrine system in 32%, the ears/eyes in 22%, the kidneys in 17%, and the liver in 12% of the patients. Some late effects (endocrine deficits, hearing loss, tubulopathy) were primarily diagnosed only several years after the end of oncological therapy. CONCLUSION: The results of this study indicate that a considerable number of former paediatric cancer patients suffer from remarkable long-term side-effects. Since life quality is an important parameter of cancer survival, careful follow-up of long-term survivors is mandatory with the aim to reduce or even abrogate possible side-effects at the earliest time.
Adolescent↗
Structures of five components of the actinomycin Z complex from Streptomyces fradiae, two of which contain 4-chlorothreonine.
Structure elucidation of five components of the actinomycin Z complex (Z(1)-Z(5)) isolated from Streptomyces fradiae is described. The components were separated by Si gel column chromatography and TLC/PLC and analyzed by ESIMS, FABMS, LC-MS of derivatized hydrolysates, and 2D NMR techniques. This permitted determination of the complete structures of actinomycins Z(1)-Z(5). In Z(3) and Z(5,) site 1 of the beta-depsipeptide is occupied by the rare 4-chloro-L-threonine, an amino acid not previously found in an actinomycin. The structural variants of the actinomycin Z complex have the molecular architecture typical of other actinomycins but possess greater structural diversity resulting from the presence of several highly unusual amino acids. Actinomycins Z(3) and Z(5,) but not Z(1), were more potent than actinomycin D in cytotoxicity assays against three tumor cell lines.
Antibiotics, Antineoplastic↗
Unrelated peripheral blood stem cell transplantation with 'megadoses' of purified CD34+ cells in three children with refractory severe aplastic anemia.
Three children with refractory severe aplastic anemia were transfused with high numbers of unrelated matched (n = 2) or C-locus haploidentical mismatched (n = 1) CD34-selected peripheral blood stem cells in the absence of an HLA-identical family donor. Two leukaphereses of the donors yielded a median number of 10.1 x 10(10) nucleated cells (range 9.7-15.4) with a median number of 9.89 x 10(8) CD34+ cells (range 7.46-26.1) and a median percentage of CD34+cells of 0.98% (range 0.77-1.7). After positive selection by magnetic cell sorting the patients received a median of 14.3 x 10(6) CD34+ cells/kg (range 11.7-24.3) and of 1.3 x 10(4) CD3+ cells/kg (range 0.57-5.8). Median time to ANC >/=0.5 x 10(9)/l was 7 days (range 7-12) and to platelets >/=20 x 10(9)/l 13 days (range 13-27). Chimerism analysis of peripheral blood after transplantation revealed permanent 100% donor hematopoiesis in all patients. The patient with the C-locus haploidentical mismatch presented with acute GVHD (grade III-IV) of the skin, liver and lower gastrointestinal tract (onset day +40) and died despite intensive immunosuppressive treatment on day +238. The two survivors developed lymphopoietic recovery of B and T lymphocytes within 3 months after transplantation. To our knowledge this experience represents the first report of transplantation with unrelated CD34+ enriched peripheral blood stem cell in children with refractory severe aplastic anemia. Bone Marrow Transplantation (2000) 25, 513-517.
Adolescent↗
Unrelated partially matched peripheral blood stem cell transplantation with highly purified CD34+ cells in a child with Wiskott-Aldrich syndrome.
Stem cell transplantation is the only curative approach to the treatment of Wiskott-Aldrich syndrome. However, using grafts from partially matched unrelated donors is associated with increased risk of graft rejection and graft-versus-host disease. In an attempt to prevent these problems, a 6-year-old boy with Wiskott-Aldrich syndrome lacking a suitable family donor, was transplanted with large numbers of unrelated highly purified CD34+ peripheral blood stem cells mismatched at one C locus. Conditioning consisted of busulfan 16 mg/kg body weight, cyclophosphamide 200 mg/kg body weight and antithymocyte globulin 20 mg/kg body weight x 3 days. The boy had a rapid hematopoietic engraftment and showed immunologic reconstitution by day +92. Although he did not receive prophylactic immunosuppression he did not develop any graft-versus-host disease and is well and alive up to now, 25 months after transplantation.
Antigens, CD34↗
Peripheral harlequin-like thermal imbalance after Wilms' tumor.
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Body Temperature↗