Haemorrhagic cystitis as a complication after allogenic bone marrow transplantation.

OBJECTIVE: The main objective of the following work is to present our own material and the ways in which we have dealt with haemorrhagic cystitis (HC) following allogenic bone marrow transplantation in children. MATERIALS AND METHOD: From 1994 to 2002, allogenic transplantation of haematopoietic cells was performed in 129 children at the Oncological and Haematological Child Clinic, Wroclaw University of Medicine. The procedure was carried out in patients with neoplastic diseases. In 33 cases, HC symptoms of various intensity were observed. The intensity of the symptoms was evaluated according to Arthur's four-point scale. To confirm the diagnosis USG was carried out in each case. Special attention was given to the ultrasonographic structure of the bladder wall. Cartoni's technique was followed in the examination. RESULTS: Out of 129 children who underwent allogenic transplantation of haematopoietic cells 33 (20.75 %) revealed HC symptoms. The symptoms occurred between the 2nd and the 124th day after transplantation (mean 29 days). The treatment included antiviral medicines, estrogens, reduction of immunosuppression and mechanical urological procedures. The children diagnosed with 2nd grade disease and higher were catheterised and diuresis was forced by the administration of larger amounts of liquids intravenously. Antihaemorrhagic drugs and vitamin K were also given. Blood was substituted if needed as were blood derivatives. Eighteen children with massive haematuria with clots underwent catherisation with a suprapubic catheter so as to continuously rinse the bladder. In 8 cases tamponade of the bladder occurred. The clots were removed from the bladder during cystoscopy under general anaesthesia. Twelve children died from HC. This amounts to 36 % of all the cases identified as HC and 9 % of all the children who underwent allogenic marrow transplantation. CONCLUSIONS: In conclusion it must be emphasised that HC in children after allogenic transplantation of haematopoietic cells is an extremely severe disease, which, if not cured, is terminal. The decision whether to perform embolisation of internal iliac arteries or to remove the bladder when non-radical methods have been exhausted, is worth considering.

[Abdominal presentation of B-cell non-Hodgkin's lymphoma (B-NHL) - surgical treatment and its results. Report of the Polish Paediatric Leukaemia/Lymphoma Study Group]. / Manifestacja brzuszna chloniaka B-komórkowego u dzieci - interwencja chirurgiczna i wyniki leczenia. Raport PPGLBC.

The aim of this study was to analyse the effect of LMB-89 protocol and surgical procedure at initial laparotomy on the outcome in children with abdominal B-cell NHL. The initial surgery intervention was: complete resection (20% pts), subtotal resection (20%), partial resection (4%), biopsy (36%). Postoperative complications occurred in 5 children. Complete recovery (CR) was achieved in 92% pts. There were 4% non responder patients. Two patients died before CR evaluation (tumour lysis syndrome; bleeding and multi organ failure after initial surgery). One patient died in CCR from sepsis probably influenced by the previous local operation. 10.8% patients relapsed. The estimate EFS for all patients with AB-NHL is 81%, 85% for stage III and 73% for stage IV. Major surgery in advanced stages is not recommended since it delays chemotherapy and fails to improve overall survival.

[Results of treatment of osteosarcoma in children according to the SFOP-94 protocol in the experience of the Polish Paediatric Solid Tumour Study Group]. / Wyniki leczenia dzieci chorych na miesaka kosciopochodnego wedlug protokolu SFOP-94 w materiale Polskiej Grupy Pediatry Cznej ds. Leczenia Guzów Litych.

Between April 1994 and December 1999, 34 children aged from 5 to 20 years (23 females and 11 males) suffering from osteosarcoma, were treated according to the SFOP-94 protocol. The primary preoperative chemotherapy consists of adriamycin and high-dose methotrexate administration. There were 28 patients with non-metastatic tumours of the extremities and 6 children presented disseminated disease with pulmonary metastases. The primary localization included femur - 20 patients, tibia - 9 patients and humerus - 5 patients. In 26 patients limb-salvage surgery was applied. The programme of chemotherapy was changed in 4 children because of toxicity of methotrexate (1 patient) and progression of disease (3 patients). 26 out of 34 (76,5 %) children are alive including 24 out of 28 patients with localized disease. EFS calculated according to Kaplan-Meier analysis was 60 % at 67 months.

[The analysis of failures in therapy of osteosarcoma in children treated according to SFOP-94 protocol in the studies of Polish pediatric solid tumors treatment group]. / Analiza niepowodzen leczenia miesaka kosciopochodnego u dzieci wedlug protokolu SFOP-94 w materiale Polskiej Pediatrycznej Grupy ds. Leczenia Guzów Litych.

Between December 1989 and April 1998 twenty eight children aged from 5 to 20 years (18 female and 10 male) suffering from osteosarcoma were treated according to the OS-SFOP-94 protocol. Twenty four patients presented with localized tumor of extremities and four with pulmonary metastases. The majority of primary tumors exceeded 150 ml of volume. The primary preoperative chemotherapy consisted of adriamycin (70 mg/m2 every four weeks) and high-dose methotrexate (12 g/m2 every week). In 20 patients limb-salvage surgery was applied, in three children--amputation and in one child tibia resection with genu arthrodesis was applied. Five of 28 patients died, one because of treatment related infection, 2 non-responders with metastatic osteosarcoma due to progressive disease, and one because of local relapse with pulmonary metastasis non-responding to therapy, one because of treatment refusal. Twenty one from 25 children are alive from 5 to 51 months. Event frae survival of children with localized disease calculated according to Kaplan-Meier analysis was 64.17% in the 51st month. The main cause of failure in the treatment of osteosarcoma in children is primary and secondary progression of disease. The toleration and results of treatment for osteosarcoma in children according to the OS-SFOP-94 is satisfactory.

[The role of local surgical and radiological control in the treatment of soft tissue sarcoma sensitive to chemotherapy in children. Report of Polish Pediatric Solid Tumors Treatment Group]. / Udzial lokalnej kontroli chirurgicznej i radiologicznej w wynikach leczenia nowotworów tkanek miekkich wrazliwych na chemioterapie u dzieci. Raport PPG/GL.

In this paper the role local surgical and radiological control in the treatment of soft tissue sarcomas in children was analyzed. All children were treated according to CWS-91 and SIOP-IV protocols. Eighty three children with RMS A + E, EES/PNET, SS, UDS were included in the analysis. The primary surgery consisted of R0 (5%), R1 (18%) or R2 (16%) resection. In majority of cases (61%) primary surgical intervention was limited to diagnostic biopsy. Conventional or hyperfractionated radiotherapy was performed in 42.8%, 73.8% and 75% of children with disease stage II, III and IV, respectively. Delayed surgery was performed in 20 out of 53 (37.7%) children with stage III of the disease. In 5 patients without primary focus (urinary bladder in 3 and prostate in 2 cases) removed, progression of the disease occurred. In 5 children (stage IV) with progression of the disease no secondary surgery was performed. In 4 of them the primary tumor exceeded 10 cm in diameter. No delayed surgery was performed in 69% of relapsed children with stage III of the disease. Planned radiation therapy was not performed in 15.9% of cases. Primary local surgical control of primary tumor is of great importance for remission duration. In children who underwent delayed surgery the estimated EFS was of 0.7, in comparison with 0.5 EFS of those without secondary surgical treatment.

[Renal neoplasms in children with tuberous sclerosis]. / Nowotwory nerek u dzieci ze stwardnieniem guzowatym.

Two cases of 14 and 11 years old children with tuberous sclerosis were admitted to Department of Paediatric Surgery in Wroclaw and Department of Paediatric Hematology and Oncology in Lublin because of renal tumors. Radical nephrectomy was performed in both cases. Histopathological diagnosis revealed malignant rhabdoid tumor (MRT) in a 14 years old boy and benign angiomyolipoma in the latter case. The authors stress the rarity of MRT in older children and successful treatment due to nephrectomy and maintenance chemotherapy.

[Results of treatment for angiosarcoma in children hospitalized in Department of Pediatric Hematology and Oncology (Wroclaw University School of Medicine) in 1989-1998]. / Wyniki leczenia miesaka naczyniopochodnego u dzieci leczonych w Klinice Hematologii i Chorób Rozrostowych dzieci AM we Wroclawiu w latach 1989-1998.

Angiosarcoma is a neoplasm included to sarcoma of soft tissue. In the period 1989-1998 9 children with angiosarcoma were treated in Department of Paediatric Hematology and Oncology.

[Atypical sarcomas localized within a head and neck in children]. / Nietypowe miesaki wieku dzieciecego zlokalizowane w obrebie glowy i szyi.

Seven children treated surgically because of non-rhabdomyosarcomatous soft tissue sarcoma (NRSTS) localized extrameningeally on the head and neck were presented. Three of the patients were operated on haemangiopericytoma, two--fibrosarcoma, one child--neurofibrosarcoma and one--liposarcoma. The pre- and postoperative TNM classification was employed as a staging system. The surgical resectability--R was used to establish tumour margins. Four patients (two with fibrosarcoma and two with haemangiopericytoma) survived free of disease. The influence of the complete surgical resection on the outcome of the head and neck extrameningeal NRSTS was proved.

[Significance of immunological system examinations in the clinical course of Langerhans cell histiocytosis in children]. / Ocena stanu ukladu odpornosciowego w przebiegu histiocytozy komórek Langerhansa u dzieci.

The Langerhans cell histiocytosis (LCH) is an enigmatic disease, usually occurring in the young. Its etiology is unknown, its pathogenesis is not correctly understood and the clinical course is unpredictable. In the years from 1975 to 1998 in Department of Paediatric Haematology and Oncology (Wroclaw University School of Medicine) 32 children with diagnosis of the Langerhans Cell Histiocytosis (LCH) were treated. The study group included 19 boys and 13 girls, aged from 3 weeks to 14 years, median age 7.5 years. Investigations of immunological system were performed in the examined group. In all children lymphocytes' subsets were examined. We examined apoptosis with ELISA test in 3 patients, in 3--marked antigen CD44, in 12 marked sVCAM-1 by ELISA method. IL-1 in growing cell supernatants was determined in 17 children. In 15 children lower IL-1 production was observed before the beginning of therapy in comparison with control group. In all patients decreased concentrations of lymphocytes T were noted, but in 8 children lower CD4/CD8 rate was observed. In the study group higher expression of CD44 was noted. In 3 patients cell apoptosis was normal. In 12 patients lower expression of sVCAM-1 was observed. Abnormal expression of adhesion molecules may influence the pathogenesis of LCH. Disorders of immunological system were observed in the patients with LCH.

[The role of leukemic cell apoptosis and adhesive molecule sICAM-1 in the clinical evolution of acute lymphoblastic leukemia in children]. / Rola apoptozy komórek bialaczkowych i czasteczki adhezyjnej sICAM-1 w ewolucji klinicznej ostrej bialaczki limfoblastycznej u dzieci.

Total number of 160 children with ALL, 98 boys and 62 girls, aged from 0.5 to 18 years was included in the study. Apoptotic cells death acc. to annexin V and ICAM-1 levels in serum and cell culture supernatants according to conventional antibody sandwich ELISA Genzyme assay were studied. Blood samples were drawn from children at different stages of their disease: at the time of diagnosis, during intensive therapy (induction, consolidation), during maintenance therapy and after completing the treatment. Thirty seven healthy children served as the control group. ICAM-1 before therapy was higher than that obtained in control group of healthy children (5.619 v 2.53 g/l). ICAM-1 decrease (3.228) after starting of chemotherapy of ALL was noticed. It was found that in children with ALL during the whole period of therapy the ICAM-1 serum levels were significantly lower than that observed in the control group of healthy children (p < 0.005). After cessation of the therapy ICAM-1 grew up (6.27 g/l). Therapeutics that modulate the regulation of apoptosis and/or expression of sICAM and cytokines production provide a new opportunity for the treatment of childhood ALL.

[Early and late complications of the use of life-cath type catheters in children with malignancies requiring urgent surgical intervention]. / Wczesne i pózne powiklania stosowania cewników typu life-cath u dzieci z choroba nowotworowa, wymagajace pilnej interwencji chirurgicznej.

Permanent access to the centrally positioned veins is necessary for the treatment of children with malignancy. The Broviac-Hickman's catheters and venous port system fulfill this require. Since January 1992 to February 1998, 276 intravenous life-cath devices were installed. In 7 patients life-threatening complications after catheter implantation occurred, which demanded urgent surgical intervention. The complications appeared in both early and late periods after catheter insertion. Pneumothorax (three children), bleeding to the mediastinum (one patient), fragmentation, disconnection and migration of a separate part of catheter into the right heart (two patients) were observed. The way of treatment and its results were described.

[Vascular complications of liver transplantation: surgical treatment]. / Complications vasculaires de la transplantation hépatique: traitement chirurgical.

Vascular complications constitute a major cause of morbidity and mortality after liver transplantation. They are dominated by arterial complications, the most frequent being hepatic artery thrombosis. Venous complications essentially consist of portal vein thrombosis. The preventive treatment of vascular complications is based on a better understanding of the risk factors. Close cooperation between surgeon, and radiologist is essential for effective surgical correction, which requires a rapid diagnosis and is designed to save not only the patient's life, but also, whenever possible, the liver transplant.