Even in Japan, at a time now called a “satiation period, ” the problem of iron deficiency still remains to be solved. Iron deficiency is not only a nutritional problem, but also a physical and social problem. To solve these problems, much new knowledge about iron deficiency has recently been reported. Nutritionally, intestinal iron absorption and iron content of food has been investigated, and the mechanism of iron absorption has now been made clear at a molecular level. Physically, the possibility of iron deficiency induced developmental delay and learning difficulty has been pointed out because from early infancy in childhood through adolescence, the body needs additional iron supply for its rapid growth, and it is susceptible to iron deficiency. Socially, dietary habits tend to be corrupted, contrary to “satiation.” The concern about iron deficiency is low, and enlightenment about the pathogenicity of iron deficiency is by no means sufficient. We hope for the establishment of a nationwide screening system for anemia (iron deficiency) in childhood.

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[in Japanese]

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Down syndrome (DS) is known to be associated with leukemia development. Especially under the age of three years, children with acute megakaryocytic leukemia (AMkL) are frequently observed. Here we report the treatment outcome of AMkL in DS. From 1984 to 1998, ten cases of AMkL in DS (age range 8 mo-3 y 5 mo, median 1 y 6 mo) were treated at our center. Transient abnormal myelopoiesis was observed in 5 cases and congenital heart disease in 4. Although 2 cases in early periods were treated with the standard regimen for acute myeloid leukemia in non-DS, 8 cases after 1993 were treated with cytarabine, etoposide, and pirarubicin. Although 3 cases in early periods died of leukemic relapse, the other 7 are all well and free of disease within a period ranging from 9 to 78 months.

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Analyses of MLL, RAS, p16, and p53 genes were performed in 17 pediatric therapy-related leukemias (t-Leukemia), including 12 acute myeloid leukemia (AML), 4 myelodysplastic syndrome (MDS), and one acute lymphoblastic leukemia (ALL). An initial diagnosis was made as non-Hodgkin's lymphoma in 5 patients, neuroblastoma in 4, ALL in 5, AML in 2, and juvenile chronic myelogenous leukemia in one. Rearrangements of MLL gene were found in 14 patients (82%), including 12 of 13 who received VP-16 and 2 of 4 who did not receive it. Homozygous deletion of the p16 gene and alterations of the p53 gene were found in no patients examined, suggesting that t-Leukemia was not associated with Li-Fraumeni syndrome. Mutation of the K-RAS gene, GGT (Gly) to GAT (Asp) on codon 12, was found in one (6%) of 16 patients, though mutation of the N-RAS gene is usually found in about 20% of adult AML or MDS patients. In conclusion, rearrangements of the MLL gene were frequent ; however, alterations of the p53 and p16 genes were infrequent in pediatric t-Leukemia.

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To elucidate the role of bone marrow transplantation (BMT) for childhood de novo acute myelogenous leukemia (AML), 16 patients that had been consecutively treated at Nagoya University Hospital for the past 10 years were analyzed. The patients were intentionally treated : the chemotherapy-oriented group (group C of 6 patients : 1 M1 and 5 M2) and the BMT-oriented group (group B of 10 patients : 1 M4, 5 M5, 3 M7, and 1 M1 with the initial leukocyte count over 1 × 105/p1). All 6 patients in group C achieved complete remission and survived for 27 to 124 months ; two patients with relapse were transplanted in the second remission. Eight of the 10 patients in group B achieved complete remission and survived for 22 to 128 months. Five patients were transplanted from related donors, and three patients without a related donor completed chemotherapy. One of 2 patients without remission was transplanted from a related donor and survived for 111 months in remission. Another was transplanted autologous marrow with an intra-atrial tumor, but died of disease. These excellent results should promote the reconsideration of the indication of BMT for childhood AML.

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We report a case with recurrent idiopathic thrombocytopenic purpura (ITP) who had recurrent thrombocytopenic episodes nine times over a period of 16 years. She was initially diagnosed as having acute ITP with concurrent rubella infection when she was 4 years old. Most thrombocytopenic episodes recurred after upper respiratory tract infections. Her thrombocytopenic episodes responded well to either conventional therapy or steroid/IVIG therapy. During her remission period, her platelet counts were above 200×10³/μl. The mean interval between onset of symptoms and preceding infections was 1.3 days. The mean duration of thrombocytopenic episodes was 7.3 days, which was shorter than that of acute ITP. The serum PAIgG levels were elevated at an acute phase of ITP and fell to within normal ranges during remission periods. The intervals between each thrombocytopenic episode tended to be prolonged after 12 years of age.

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We report a, 6-year-old boy diagnosed as having inv (16) (p13 ; q22) -acute myelocytic leukemia (AML) M4E accompanied with leukocytosis (WBC count, 386, 000/μl). Hyperleukocytosis at the diagnosis was considered a poor prognostic sign even in presenting the inv (16) as a good prognostic factor. This patient was treated with chemotherapy in combination with autologous bone marrow transplantation (auto-BMT) in first complete remission. An analysis of CBFβ/MYH11 fusion transcripts using the RT-PCR at about 6 months after the auto-BMT failed to CBFβ/MYH11 fusion transcripts. Chimerism analysis using sensitive PCR assays for CBFβ/MYH11 fusion transcripts may be useful to predict the possibility of relapse in posttransplant.

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We report a male 1 year and 8 months old with acute myelogenous leukemia (FAB classification M6) and monosomy 7 who underwent unrelated bone marrow transplantation (UBMT). He was admitted in November 1994 with a prolonged fever and cough and was diagnosed with acute myelogenous leukemia M6. We administered low-dose Ara-C for a week, then ANLL 91 protocol chemotherapy, which induced bone marrow remission 3 months later. After 11 months of consolidation therapy, he underwent UBMT from an HLA-and blood-type-matched female donor. He received 10 Gy of total body irradiation, etoposide 60 mg/kg/day, and 4 days of cyclophosphamide 50 mg/kg/day as a conditioning regimen. He received a transplant of 9.83×10⁹ of nucleated bone marrow cells. We administered prednisolone (PSL) for chronic liver GVHD on day 70. He has been in complete remission with negative monosomy 7, but has continued CSA therapy for chronic liver GVHD.

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