Using the data obtained in the present study, we describe situations faced in the terminal care of children. It is important to address the problems revealed by this analysis in order to achieve improvements in both the physical and psychological care of children with terminal cancer.

To determine the clinical relevance of in vitro drug chemoresistance in childhood acute myeloid leukemia, we used an MTT assay to test leukemic cells from 132 newly diagnosed children. Patients were diagnosed according to the French-AmericanBritish (FAB) classification as follows: M0 (n = 12), M1 (n = 16), M2 (n = 53), M4 (n = 17), M5 (n = 19) and M7 (n = 15). The results revealed that, compared to leukemic cells from completeresponders (n = 107), those from non-responders who failed induction therapy (n = 17) were 1.4 to 5.0 times more resistant in vitro to cytarabine (P = 0.005), melphalan (P = 0.003), etoposide (P = 0.011), L-asparaginase (P = 0.017), aclarubicin (P = 0.026) and dexamethasone (P = 0.039). For seven other drugs tested, the median lethal dose of 70% and leukemic cell survival of non-responders were higher than those of complete-responders, but the difference was not statistically significant. We sought correlations between FAB subtypes and in vitro drug resistance. Leukemias of the FAB M4 and M5 subtype were more sensitive to L-asparaginase (P = 0.01, P = 0.0036) than those of the FAB M2 subtype. FAB M5 leukemia was more sensitive to etoposide than were the FAB M2, M4 and M7 subtypes (P = 0.001, P = 0.034, P = 0.023, respectively). By contrast, FAB M5 leukemia was significantly more resistant to prednisolone and dexamethasone than were the FAB M0, M1, M2, M4 and M7 subtypes. We sought correlations between in vitro drug resistance and long-term clinical outcome, but found no associations in this case. These results suggest that in vitro resistance to cytarabine, melphalan, etoposide, L-asparaginase, aclarubicin and dexamethasone might represent factors that can predict response to the early course of therapy. Selecting an appropriate anti-cancer drug according to the FAB classification together with drug sensitivity testing may contribute to improved prognoses in childhood acute myeloid leukemia. Leukemia (2001Leukemia ( ) 15, 1892Leukemia ( -1897

Summary. To explore the potential efficacy of l-asparaginase treatment in acute myeloid leukaemia (AML) patients, we studied the in vitro resistance of French-AmericanBritish (FAB) subtypes of childhood AML to l-asparaginase using a methyl-thiazol-tetrazolium assay. We tested leukaemic cells obtained from 177 common acute lymphoblastic leukaemia (cALL) and 228 AML children at diagnosis. The median 70% lethal dose of l-asparaginase (LD70 asp ) (U/ml) was 0AE46 in the cALL and 6AE70 in the AML samples. The median LD70 asp among each FAB subtype of AML was 0AE76 (M0), 0AE46 (M1), 10AE00 (M2), 10AE00 (M3), 1AE18 (M4), 1AE35 (M5) and 10AE00 (M7). Type M3 samples had the highest LD70 asp . The LD70 asp of the M2 samples was significantly higher than that of the M1, M4 and M5 samples. When the LD70 asp values were classified as low (0AE016-0AE159), intermediate (0AE16-1AE59) or high (1AE6-10AE00), the frequency of low, intermediate or high LD70 asp among the M1 samples were similar to those among the cALL samples. In conclusion, cells from AML types M1, M4 and M5 were relatively sensitive to l-asparaginase, and M1 cells were as sensitive as those of cALL, suggesting that l-asparaginase treatment may be effective for these subtypes of AML.

Some advances in the quality of life of the children were recognized. However, these advances were extended to a greater percentage of children in the ST group than in the LL group. The psychosocial problems faced by children and their families are now changing for the better.

Summary:We describe the successful treatment of a 20-year-old patient with chronic granulomatous disease (CGD), by unrelated bone marrow transplantation (UBMT). The patient is relatively old compared to other CGD patients treated with BMT. He had had repeated serious infections from early childhood and was diagnosed as CGD, gp91-phox deficiency. Prolonged antibiotic-resistant pneumonitis worsened when the patient was 18 years old. In addition, he suffered Aspergillus osteomyelitis and acute renal failure due to amphotericin B. He received 94 granulocyte transfusions from 94 adult donors and the infections gradually improved. In September 1998, at 20 years of age, he underwent UBMT from an HLA 6 antigen-matched male donor, with CY and TBI conditioning. He received MTX and CsA as prophylaxis against GVHD. No serious complications occurred and rapid engraftment was achieved. Acute GVHD (grade 2, at day 19) and chronic GVHD (limited, at day 192) occurred. However, both were easily controlled. The patient is alive and well with no late rejection 26 months after UBMT. Bone Marrow Transplantation (2001) 28, 83-87. Keywords: chronic granulomatous disease; unrelated bone marrow transplantation; aspergillosis; granulocyte transfusions Chronic granulomatous disease (CGD) is a primary immunodeficiency disease characterized by recurrent severe bacterial and fungal infections during early childhood. CGD is caused by dysfunctional phagocytosis that is a result of defective NADPH oxidase. Deficiency of this oxidase causes a marked reduction in the phagocyte respiratory burst resulting in defective microbial killing. IFN-␥, supportive therapy and prophylactic administration of therapeutic agents such as sulfamethoxazole-trimethoprim have improved the prognosis.