Twenty years of Polish experience with three consecutive protocols for treatment of childhood acute myelogenous leukemia.

Until 1983, results of treatment of acute myelogenous leukemia (AML) in Poland with different regimens were very poor. In 1983, the Polish Pediatric Leukemia/Lymphoma Study Group introduced a unified treatment protocol--a modified version of BFM-83 protocol. This led to an increase in the curability of AML from 15% to approximately 32%. In 1994, a modification was made: the high-risk patients (>5% blasts in bone marrow on day 15 of therapy and all M5 cases) received two additional cycles with intermediate-dose cytarabine (ID-ARAC). This led to a nonsignificant improvement in the 5-year event-free survival (EFS) rate from 32 to 36%. A new treatment protocol employing idarubicin in place of daunorubicin was introduced in 1998 and produced better initial responses, increase in the number of patients attaining remission after induction therapy and proportional increase of standard-risk patients. The probability of 5-year EFS (pEFS) for the whole group of patients increased from 36 to 47%. In standard- and high-risk groups, the 5-year pEFS was 62 and 33%, respectively. The probability of 5-year disease-free survival was 58% in the whole group, and there were no differences between risk groups. Unsatisfactory treatment results in children classified into the high-risk group are principally due to the low remission rate.

[The role of chemotherapy in treatment of nephroblastoma in children. Report of the Polish Pediatric Group re. treatments of solid tumors]. / Udzial chemioterapii w leczeniu nerczaka zarodkowego u dzieci. Raport Zespolu Polskiej Grupy Pediatrycznej ds. Leczenia Guzów Litych.

Retrospective analysis of chemotherapy results of children with nephroblastoma was performed in 220 patients aged from 1 yr to 14 yrs of live in 12 centers. Stage I nephroblastoma was documented in 24.5% but stage II--in 55.3%. Histologically 74.6% cases were diagnosed as medium malignant and 12.7%--high malignant. Therapy results were similar to observed in other centers.

[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.

[Bone marrow transplantation in children]. / Transplantacja szpiku kostnego u dzieci.

The most frequent indications for bone marrow transplantation and complications of this method of treatment are reviewed. The results are compared with results of conventional therapy. Attention is focused on increasing possibilities of autologous transplantations in children having no HLA-compatible siblings.

[Results of treatment for severe acquired aplastic anemia in children]. / Wyniki leczenia ciezkiej, nabytej niedokrwistosci aplastycznej u dzieci.

The authors evaluated results of treatment of 106 children with acquired aplastic anemia. The patients were divided into 3 groups depending on the severity of their disease. Thirty-nine patients were classified as very severe, 30 as severe and 37 as non-severe according to the modified Camitta criteria. Among them, 47 children were treated with oxymetholone and prednisolone. In this group 32 died. Antilymphocyte globulin (ALG) was given to 48 patients and 20 received cyclosporin A (CsA). The results obtained by these two methods are nearly the same and 5 year survival was 61% and 59% respectively. Bone marrow was transplanted in only one child, who is still in complete remission. Statistical analysis showed a steady increase in incidence of aplastic anemia in the years 1987-1989, which might coincide with the Czarnobyl explosion. However, further research is required to prove this point.

Standard and intermediate risk acute lymphoblastic leukemia in Poland: a report of the Polish Children's Leukemia/Lymphoma Study Group.

A total of 527 children with acute lymphoblastic leukaemia (ALL) from the most frequent risk groups: standard risk group (SRG) and intermediate risk group (IRG) were treated between 1987 and 1991 according to an intensified treatment program (based on the BFM protocol) including the use of an intermediate dose of methotrexate in the IRG. A comparison of the treatment results in this group from 513 children treated between 1981 and 1987 indicates that the chance for a 6 year event-free survival has increased to 73% (previously 55%).

[Acute lymphoblastic leukemia in infants--results of treatment according to infant leukemia protocol--POG 8493]. / Ostra bialaczka limfoblastyczna u niemowlat--wyniki leczenia wg infant leukemia protocol--POG 8493.

Analysis was made of ALL treatment results according program POG 8493 in 9 infants. In 4 children WBC exceeded 100.0 G/l, and in 5 children CNS was affected. Eight infants had remission, six had relapse (mainly BM): early up to 8 month in 5, late--in 52 month in one girl. Four children died after relapse, four are still living: 2 I RC (2 and 23 months), 2 in II RC (1 month and 23 month). Probability of 4-year EFS was 0.25 and that survival--0.44. In view of continuing poor ALL prognosis, in infants it is necessary to look for more effective methods of treatment.

[Duration of second remission of Hodgkin's disease in children. Results of treatment achieved by the Polish Leukemia and Malignant Lymphoma Therapy in Children Group]. / Trwalosc drugiej remisji w chorobie Hodgkina u dzieci. Wyniki leczenia Polskiej Grupy ds. Leczenia Bialaczki i Chloniaków Zlosliwych u Dzieci.

Recurrence was noted in 18.5% of 194 children, in which chemotherapy with MVPP regimen produced complete I remission. In 6 out of 36 children with recurrent disease MVPP regimen was repeated, while the remaining children were treated with B-DOPA alone or combined with MOPP regimen. Local radiotherapy was used in 17 children. The second complete remission was achieved in 30 (83.7%) children. Thirteen out of 36 patients died because of the progress of the disease (11 children), and for complications (2 children). Percentage of persisting 5- and 10-year II remissions are 58.2% and 54.6%, respectively. A 5- and 10-year survival rates in children with recurrent disease are 80.5% and 60.5%, respectively. Our relatively favourable results we associate--first of all--with the chemotherapy intensity.

[Exchange transfusion in children with blastic hyperleukocytosis in acute lymphoblastic leukemia]. / Transfuzja krwi wymienna u dzieci z hiperleukocytoza blastyczna w przebiegu ostrej bialaczki limfoblastycznej.

In 10 children with acute lymphoblastic leukaemia (mean leucocyte count 452 x 10(9)/l) 17 exchange transfusions were done for rapid reduction of this count. The control group comprised 11 children with similar values of white blood cell counts (mean 324 x 10(9)/l) treated initially with small, increasing doses of corticosteroids. In contrast to acute myeloid leukaemia the results do not support the usefulness of exchange transfusions in children with hyperleukocytosis during ALL.

[Allogeneic bone marrow transplantation in proliferative blood diseases in children and adolescents (preliminary observations)]. / Transplantacja szpiku allogenicznego w chorobach rozrostowych krwi u dzieci i mlodziezy obserwacje wstepne.

Transplantation of allogeneic bone marrow was done in 4 patients during the second remission of acute lymphoblastic leukaemia (3 cases) and B-cell non-Hodgkin lymphoma with dissemination to bone marrow (1 case). The engraftment and complete haematological reconstruction was achieved.

[Results of treatment of Hodgkin's disease in children. Observation of 203 cases treated with modified MVPP regimen combined with local radiotherapy]. / Wyniki leczenia choroby Hodgkina u dzieci. Obserwacja 203 przypadków leczonych zmodyfikowana chemioterapia MVPP skojarzona z miejscowym napromienianiem.

Totally 209 children were treated within 1971-1982. Complete remission was achieved in 95.6%. Recurrence was noted in 36 patients (18.6%); 20 patients (9.9%) died due to progression of the disease and 9 patients (4.4%) because of complications. The first remission remains constantly in 150 patients for 63-206 months, including that after completion of therapy during 45-206 months. Asymptomatic 5- and 10-year survivals are the following: 82.9% and 80.1% respectively; 5- and 10-year survivals 91.9% and 89.8%, respectively. Expanded experience served as base to design a new regimen (introduced in 1988) which is being supposed to reduce untoward reaction by decreasing the number of MVPP cycles and irradiation doses.

[Unintentional intrathecal administration of a tenfold dose of methotrexate]. / Niezamierzone podanie dokanalowe dziesieciokrotnej dawki metotreksatu.

Unintentional administration of methotrexate intrathecally in a dose 10 times higher than normal was not followed by any adverse effects in the treated child. The possibility is discussed of using higher than presently used doses of this drug in the prevention and treatment of leukaemic infiltrations in the central nervous system.

Acute lymphoblastic leukemia in children below two years of age.

From among 1464 children with ALL 167 (11.4%) at diagnosis had two or less than two years, 53 of them were infants. Most of them had a great tumor bulk, 15 had initial CNS infiltration and 31 WBCc greater than or equal to 100000/ML. 66 were treated according to St. Jude or LSA2L2 programs (the I group), 101 according to BFM programs (the II group). Complete remission was obtained in 85% of patients. In 63 children relapses occurred in the course of treatment whereas in 5 after the therapy cessation. In majority of cases, there were isolated relapses, mostly, they involved bone marrow. CNS involvement was found, in both mixed and isolated relapses, in 28 children (20%). 64 children are alive, in 43 of them the therapy was stopped. Kapla-Meier estimates for event free survival (EFS) after 8 years were 19 +/- 4.7% in the first group vs 37.38 +/- 6.3% in the second group. Due to therapy intensification the treatment results have been improved (p = 0.05) but still they are not satisfactory, particularly in case of infants and children with WBC greater than or equal to 100000/ML. Analysis of the lot of children showed that apart from age at ALL diagnosis and the initial WBC the methods of treatment are also the prognostic factors.

Acute lymphoblastic leukaemia therapy in Poland. A report from the Polish Children's Leukaemia/Lymphoma Study Group.

Treatment results of 1304 children with ALL obtained in Poland are presented. A group of 524 patients has been subject to a detailed analysis. These patients have been treated in the years 1981-1985 according to the BFM Protocol. In this group the 6-years CCR probability amounts to 0.56.