[Lateral cervical cyst in 13-year-old girl]. / Torbiel boczna szyi u 13-letniej dziewczynki.

We present a clinical description of a 13-year-old girl with lateral cervical cyst. We particularly emphasize the need for ultrasonographic examination during routine diagnostics of so-called cervical tumors.

[Extramedullary (bone) relapse 16 months after allo bmt in a 7-year-old girl treated for acute lymphoblastic leukaemia from the second year of life]. / Pozaszpikowa (kostna) wznowa u 7-letniej dziewczynki leczonej od drugiego roku zycia z powodu ostrej bialaczki limfoblastycznej--W 16. Miesiacu po allo bmt.

7-year-old girl treated for acute lymphoblastic leukaemia since June 1995 after marrow relapse (June 1997) presented with painful oedema of the upper part of left hip and limping, 16 months after allogeneic bone marrow transplantation (BMT). Bone marrow examination excluded medullary relapse. Histopathological investigation of periosteum and bone scrapings revealed massive leukemic infiltration. Radiotherapy resulted in local arrest of the malignant process. However, bone marrow relapse was diagnosed in the child two months later with subsequent death after one month.

[Treatment regimen for children and adolescents with Hodgkin's disease designed to decrease late complications of radiotherapy]. / Program leczenia choroby hodgkina u dzieci i mlodziezy zmierzajacy do ograniczenia powiklan po radioterapii.

Between 1997 to 1999 in 9 centres of the Polish Paediatlic Leukemia/Lymphoma Study Group, 167 children and adolescents (aged 2-19 years) with stage 1 to IV Hodgkin's disease (HD) were treated according to a regimen with a limited use of radiotherapy (RT). All patients received B-DOPA and MVPP chemotherapy. The number of cycles of chemotherapy was adjusted in respective risk groups. In 13 children with stage IA and IIA disease with favourable prognostic factors chemotherapy alone was used. In other patients the dose of RT applied to lymphatic regions was 15-46,4 Gy. In case of a small tumour at presentation and good response to initial chemotherapy the RT dose was 15-16 Gy. In other cases doses of 25-30 Gy were planned. The use of higher doses, particularly exceeding 35 Gy, in eleven patients, was not justified. Among all the 167 patients, three oftliem (1.2%) with advanced disease (Stage III-1V) did not achieve first remission. The 4-year overall survival (OS), relapse free survival (RFS) and event free survival (EPS) were 99%. 93% and 90%, respectively. Relapses occurred in 8 children (first remission lasted for 4-29 (median = 9 months). All 13 children in whom chemotherapy alone was used remain in first remission. In the group of children who received RT in the dose of 15-16 Gy relapse occurred in one child. Our preliminary analysis indicates that limited use of RT in selected cases of HD in children and adolescents did not show worse results of treatment. However, the assessment of possible influence of this regimen on the decreased rate of late complications requires longer follow-up.

[The incidence and treatment of the first relapse in children with Hodgkin's disease]. / Wystepowanie i leczenie pierwszej wznowy choroby Hodgkina u dzieci.

From January 1988, to December 1997, among 447 children with Hodgkin's disease (HD) who underwent initial treatment in seven centres of the Polish Paediatric Leukaemia/Lymphoma Study Group, 442 patients obtained a complete remission (CR). The initial treatment consisted of multidrug chemotherapy (B-DOPA and MVPP) combined with local radiotherapy. Relapses occurred in 35 cases (7,9%). Two patients from other centres were also included in this analysis. Four patients were lost to follow-up; 33 patients with relapses were analysed. Early relapses (first complete remission (CR) shorter than 12 months) occurred in 17 cases. Treatment of the first relapse consisted of different types of multidrug chemotherapy. Six patients underwent high-dose chemotherapy and peripheral blood stem cells transplantation. Radiotherapy was used in 19 children. Second CR was achieved in 28 patients (85%). In 10 children (36%) second relapse occurred after 4 to 21 months (median = 10). In 17 cases the second CR lasted 12-14 (median=54) months. The probability of the 7-year freedom from second relapse was 64%. Eleven patients died; one of them in second CR due to toxic liver damage. Results of treatment in children with early relapses were significantly worse. In 17 patients with early relapse, and 16 children with late relapse, the second CR was achieved in 70% and 100% of cases, respectively. The probability of the 7-year overall survival, freedom from second relapse and event-free survival in children with early and late relapse was: 42, 58, 40%, and 94, 69, and 66%, respectively. The therapeutic results in the subgroups of children with relapses treated with different methods were not comparable because of the small number of children in each group. The use of multidrug chemotherapy with or without radiotherapy allows to achieve a long lasting second CR in more than 50% of children with HD who relapsed after initial combined modality treatment. The optimal treatment of relapsed HD in patients initially treated with multidrug chemotherapy with or without of radiotherapy, is currently unknown.

[Evaluation of cochlear function by means of otoacoustic emission in children treated with cytostatic drugs in the course of bone marrow proliferative diseases]. / Ocena funkcji slimaka za pomoca otoemisji akustycznej u dzieci leczonych cytostatykami w przebiegu chorób rozrostowych ukladu krwiotwórczego.

The aim of study was cochlear function estimation by means otoacoustic emission in children treated by leukaemia cytostatic drugs in course of the bone marrow proliferative diseases. The children after treatment of acute lymphoblastic leukemia or nongranulomatic malignant lymphoma according to BFM programme were examined in this study. Audiologic examination of children included tonal audiometry, tympanometry and otoacoustic emission as well. The results were compared to the findings of control group consisted of healthy children have never suffered from ear diseases and have never used ototoxic drugs. No significant differences in tonal audiometry and TEOAE were observed. Aggressive treatment of leukaemia does not result in hearing loss.

Childhood stage IV Hodgkin disease: therapeutic results of the Polish pediatric leukemia/lymphoma study group.

BACKGROUND: The therapeutic management in patients with stage IV Hodgkin disease is still controversial. PROCEDURE: Among 783 children with Hodgkin disease treated from 1971 to 1996, 56 patients (7.3%) were diagnosed with stage IV. The treatment consisted of MVPP or MVPP/B-DOPA chemotherapy combined with involved-field radiotherapy in 50 children. RESULTS: The results of treatment of stage IV patients were compared in the three sequential time periods, during which the therapy was modified. In these periods, the first complete remission was obtained in 67%, 86%, and 90% of children, respectively, and the 10-year event-free survival was 42%, 64%, and 85%, respectively. CONCLUSIONS: Alternate multidrug chemotherapy combined with low-dose involved-field radiotherapy is at present a satisfactory therapeutic method in children with stage IV Hodgkin disease.

[Does residual mediastinal mass in children with completed treatment of Hodgkin's disease affect the incidence of relapses?]. / Czy resztkowy guz sródpiersia przetrwaly po zakonczeniu leczenia choroby Hodgkina u dzieci ma wplyw na czestosc wystepowania wznów?

Four hundred and four children with Hodgkin's disease (stage I-IV) were treated in seven cooperating centers of Polish Paediatric Leukaemia/Lymphoma Study Group between 1988 and 1996. Mediastinal masses and/or hilar involvement were found in 261 (65%) patients. Remission was obtained in 256 (98%) of this group. In 31 (12%) children residual mediastinal/hilar masses were found after completing the treatment. For this reason in 13 cases the number of chemotherapy courses and/or the dose of radiation therapy were increased. In two cases thoracotomy or thoracoscopy were performed, and in one case gallium scan was performed. In none of these patients active disease was found. Relapses occurred in 4 (12.9%) from the group of 31 children with residual mediastinal/hilar involvement 8-15 months after cessation of the therapy. Twenty seven children have been in first remission for 5-113 months (median, 34). In 225 patients with a complete resolution of their mediastinal/hilar masses, relapses occurred in 13 (5.7%) cases. Patients with residual mediastinal mass should be carefully evaluated before making a decision to complete their treatment, including CT scan, MRI, and gallium scan. In doubtful cases histopathological verification should be done.

[Ultrasonographic evaluation of thyroid in patients cured from Hodgkin disease]. / Ocena ultrasonograficzna tarczycy pacjentów wyleczonych z choroby Hodgkina.

Long-term prognosis in patients cured from Hodgkin's disease (HD), diagnosed in childhood has dramatically improved. This is why, early detection and elimination of treatment side-effects has become essential. The study aimed at thyroid morphology evaluation in 46 patients remaining in complete remission more than five years after completion of treatment for HD. Thyroid morphology was assessed by palpation and ultrasonography. Control group consisted of 43 age- and sex-matched healthy people. 17.4% of patients after HD treatment had abnormal thyroid morphology on physical examination (gland enlargement, increased consistency or presence of nodules). Thyroid volume in this group was significantly decreased comparing to control group (p < 0.005). 32.6% of patients had heterogenic echogenicity. Ultrasonography revealed pathological lesions in 21.7% of examined individuals. Single hypoechogenic lesions in 5 patients, multiple hypoechogenic in 4 patients and multiple hypo- and hyperechogenic in 1 existed. High frequency of pathological thyroid lesions in thyroid of patients cured from HD emphasizes the need for systematic ultrasonographic thyroid evaluation.

[Acute lymphoblastic leukemia in children with Fanconi anemia]. / Ostra Bialaczka limfoblastyczna u dzieci z zespolem Fanconiego.

Fanconi anaemia (FA) is a rare autosomal recessive disorder. Manifestation of the disease is pleomorphic and may include many congenital malformations and marrow aplasia. Congenital disorders include: skeletal abnormalities, hypo- or hyperpigmentation of the skin, renal or heart anomalies and many others. FA is an invariably fatal disease owing to progressive marrow aplasia or the development of acute leukaemia or squamous cell carcinoma. We present two children with Fanconi anaemia who developed acute lymphoblastic leukaemia in the 4 and 12 year of life.

[Tumor lysis syndrome in the course of neoplastic disease of hematopoietic system in children]. / Zespól lizy guza w przebiegu chorób rozrostowych ukladu krwiotwórczego u dzieci.

Tumor lysis syndrome is characterized by hyperuricemia, hyperkalemia and hyperphosphatemia followed by hypocalcemia. Sometimes they are accompanied by hyperglycemia. It can lead to acute renal insufficiency, neurological and cardiological disorders. In the Clinic of Paediatric Haematology and Chemotherapy in Zabrze, in the period from Jan.01, 1990 to Dec.31, 1997, 305 children were treated due to neoplastic diseases of haematopoietic system: 169 with ALL, 34--ANLL, 48--NHL and 54--HD. Tumor lysis syndrome was found in 11 children, what was 3.6% of all patients. They included 3 children with ALL/1 from group SRG and 2 with HRG/, 2 with ANLL, 6 with NHL/2 with NB-NHL and 4 with B-NHL/. Tumor lysis syndrome did not occur in any patient with HD. Two symptoms of tumor lysis syndrome were found in 13 children (4.26%) including 3 with ALL (1 SRG and 2 HRG), 2 with ANLL and 8 NHL/3-NB-NHL and 5-B-NHL/. Diabetes occurred in two children with ALL. Acute renal insufficiency occurred in 6 children with ALL, 2 with ANLL and 8 with NHL. Two patients with B-NHL required dialysis. One renal insufficiency treated with hemodialyses was the first symptom of lymphoma. To prevent the consequences of tumor lysis syndrome hydration with alkalization, constant monitoring of the above mentioned biochemical parameters are necessary. Acute renal insufficiency, not responding to conservative treatment, should be immediately treated with hemodialyses and chemotherapy should be introduced under its 'shield'.

[Acute renal insufficiency as the first symptom of malignant lymphoma in children]. / Ostra niewydolnosc nerek jako pierwsze ujawnienie nieziarniczego chloniaka zlosliwego u dzieci.

Acute renal insufficiency in children with malignant lymphoma may be the first symptom of illness. Intensive care with hemodialysis is the therapy of choice. It allows to eliminate the increased volume of liquid, toxins, continuing diagnostic and beginning chemotherapy. We present two children with NHL, who showed acute renal insufficiency. Intensive care with hemodialysis was required to treat renal failure before establishing diagnosis and during initial chemotherapy.

[The problem of blood transfusion ++ in Jehova's Witnesses' children with oncological disease]. / Problem przetaczania krwi w chorobach nowotworowych ukladu krwiotwórczego u dzieci wyznawców swiadków Jehowy.

We present 2 cases of Jehovah's Witnesses' children suffering from oncological diseases (non-Hodgkin Lymphoma and Acute Lymphoblastic Leukaemia). During their treatment we used erythropoietin and no blood products were transfused.

[Non-Hodgkin gastric lymphoma in a 14-year-old boy]. / Nieziarniczy chloniak zlosliwy zoladka u 14-letniego chlopca.

Lymphomas of the gastrointestinal tract in children are localized most often in the ileocoecal region. We described a rare case of gastric lymphoma in 14-year-old boy.

[Assessment of the efficacy of treating infections in hematopoietic proliferative diseases: Monotherapy with ceftazidime and tobramycin combined with amoxycillin/ampicillin]. / Ocena skutecznosci leczenia zakazen w przebiegu chorób rozrostowych ukladu krwiotwórczego: Monoterapia ceftazydymem i skojarzenie tobramycyny z amoksycylina/ampicylina.

Efficacy of the ceftazidime monotherapy in 120 febrile children with neoplastic diseases and granulocytopenia was compared with that of tobramycin combined with amoxycillin/ampicillin. The obtained results were similar in both types of antibiotic therapy. However, granulocytopenia was higher and septicemia was more frequent in children treated with ceftazidime. Isolated bacteria were more sensitive to ceftazidime than to tobramycin with amoxycillin/ampicillin. Both regimens were tolerated well. Despite a low number of patients in both groups, one may conclude that ceftazidime is more efficient in patients with granulocytopenia. Less adverse reactions, lower number of infections, less frequent medical procedures, elimination of the potentially toxic aminoglycosides and lower cost of therapy advocate the use of ceftazidime monotherapy.

[T-cell acute lymphoblastic leukemia E(-) with antigen Ia expression]. / T-komórkowa ostra bialaczka limfoblastyczna E(-) z ekspresja antygenu Ia.

A case is presented of a rare T-phenotype acute lymphoblastic leukaemia. The blast cells were negative in the rosette test and demonstrated expression of the Ia antigen.

[Stages of B-lymphocyte differentiation in acute lymphoblastic leukemia]. / Stadia zróznicowania limfocytów "B" w ostrej bialaczce limfoblastycznej.

Blasts phenotype was determined in 61 children with the acute lymphoblastic leukemia. Non-T-cell acute lymphoblastic leukemia was diagnosed in 51 children. Stages of blasts differentiation were determined with the aid of monoclonal antibodies set using alkaline phosphatase-anti-alkaline phosphatase technique. Blasts in 50 patients belonged to B subpopulation confirmed by the presence of panB CD19 and CD22 antigens. Common antigen was seen in 76.5% of the examined patients with non-T-cell acute lymphoblastic leukemia. Cases of non-T-cell acute lymphoblastic leukemia were divided into 8 subgroups depending on the antigens of B-cells differentiation. An identification of pre-B subgroups of the acute lymphoblastic leukemia indicates heterogenicity of the acute lymphoblastic leukemias in childhood and enables their classification into groups corresponding to the early stages of lymphoblasts maturation.

[Use of an immunoenzyme method with monoclonal antibodies in cytologic studies for the optimal diagnosis of non-Hodgkin's lymphoma in children]. / Zastosowanie metody immunoenzymatycznej z uzyciem MoP w badaniach cytologicznych do optymalizacji diagnostyki non-Hodgkin-lymphoma u dzieci.

From January 1987 to April 1988 six children we studied cytologically with the use of alkaline phosphatase-anti-alkaline phosphatase (APAAP) method and monoclonal antibodies (MoP) in order to establish the diagnosis of Non-Hodgkin Lymphoma. (NHL). Cytologic studies concerned pleural fluid (3 patients) imprints of the lymph node (3 patients), bone marrow smears (2 patients) and cerebrospinal fluid (1 patient). We performed simultaneously routine cytologic and histopathologic studies of the lymph nodes. Antigen T6 (thymocytes) was present in blasts of all patients, which permitted us to classify the blasts as common stage II group according to Reinherz. In all cases we found at least two positive antigen detected by MoP pan T (CD2, CD3, CD5, CD7) in one case--no expression of antigen T3 (CD3) and in two cases no antigen detected by an antibody CD2. Antigen Ia was found in one patient, and weak expression of antigen CALLA (CD10) in one patients. In three patients we showed a simultaneous expression of antigens T4, T8, whereas in two patients they were not observed. One child possessed mature phenotype T4, T6. By using APAAP method with MoP in cytologic studies it was possible to diagnose T-lymphoblastic lymphoma in six children before the results of histopathologic examination of the lymph nodes.