Umbilical cord blood (CB) is rich in hematopoietic stem/progenitor cells (SC), which have high proliferative potential. Recently, CB has been used as a source of SC transplantation. Recent results of cord blood transplantation (CBST) from siblings showed that CB contains sufficient numbers of SC to engraft recipients and that the transplantation of these cells was associated with a very low risk.of acute and chronic GVHD ; in Japan also, the results are promising. The New York Blood Center established a cord blood bank in 1992, and more than 500 CBSTs from unrelated donors have been performed. As a result of the success with CBST from sibling donors, a pilot bank for CB banking in Japan began in 1995 at the Kanagawa Children's Medical Center (Kanagawa Cord Blood Bank). In this report, the system of our cord blood bank is presented, and the current status of CBST, in Japan and worldwide, from related and unrelated donors is reviewed.

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We studied the apoptosis of neutrophils obtained from 11 patients (from one to 18 years of age) in remission after treatment with allogeneic bone marrow transplantation. Peripheral blood neutrophils obtained from these patients were cultured, and after a 24-hr incubation, the number of apoptotic cells was determined by morphological examination or DNA content. The proportion of apoptotic neutrophils from patients who were not treated with prednisolone was greater than that of healthy controls (p<0.05); however, neutrophils from patients under treatment with prednisolone was not. The proportion of apoptosis and the percentage of lymphocytes in peripheral blood showed a significant inverse relation (p<0.05). However, neither the level of HLA-DR expression of CD3-positive lymphocytes nor the Fas expression of neutrophils from peripheral blood showed any relation to the level of apoptosis. We further studied in vitro the effect of hydrocortisone (HDC) and patient serum on the apoptosis of neutrophils in vitro. HDC inhibited the apoptosis of neutrophils, and the effect of HDC was more significant on neutrophils from patients (p<0.05), but there was no correlation between the level of apoptotic neutrophils in a patient and the effect of serum on apoptosis.

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We report the clinical usefulness of a sensitive triple-color flow cytometric assay (FCM) to detect residual leukemic cells. For this study, 23 cases of CD45-negative B-precursor acute lymphoblastic leukemia (ALL) were included, because the absence of CD45 expression provided a marker for the leukemic cells in addition to the positive expression of CD 10 and CD22 antigens. Leukemic cells in patients' bone marrow (BM) consistently showed CD 10⁺/CD22⁺/CD45^{- or dim} phenotype. FCM analysis could detect leukemic cells at a level of 1 per 104 cells. The CD 10⁺/CD22⁺/CD45^{- or dim} cell population was absent in normal BM, regenerating BM, and BM collected from leukemic patients in long-term complete remission. This assay could identify residual leukemic cells up to 50 days after starting induction chemotherapy. Furthermore, a gradual increase of leukemic cell population could be monitored by this assay before morphological confirmation of BM relapse. Taken together, a triple-color FCM was a sensitive method of detecting leukemic cells in BM. This assay could be an additional component of testing to monitor MRD in ALL patients.

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The incidence and residence distribution of pediatric hematopoietic malignancies in children under 15 years of age were surveyed. Eighty-three children with hematopoietic malignancies were diagnosed during the period from 1990 through 1994; 57 patients were acute lymphocytic leukemia (ALL) (68.7%), 19 (22.9%) acute nonlymphocytic leukemia (ANLL), 2 (2.4%) myelodysplastic syndrome (MDS), 4 (4.8%) chronic myeloid leuke-mia (CML), and 1 (1.2%) juvenile myelomonocytic leukemia (JMML). The population-based incidence was 3.70 per 100, 000 persons a year as a whole; the incidence of ALL, ANLL, MDS, CML, and JMML was 2.54, 0.85, 0.09, 0.18, and 0.04, respectively. These results were comparable with the results reported from other countries. The incidence in four districts in Kyoto Prefecture was as follows : 1.59 in Tango-Chutan district, 2.04 in Chubu, 4.95 in Kyoto-Otokuni, and 2.24 in Minamiyamashiro-Souraku. The incidence was significantly high in the Kyoto-Otokuni district (p = 0.023) and low in the Tango-Chutan district (p = 0.026). Eight infant leukemia patients (ALL 5, ANLL 3) developed out of 83 leukemia (9.6%) during the same period. The incidence was 6.62, which seemed to be higher than the previously reported incidence of 3 or 4 per 100, 000 persons a year. It should be elucidated why the incidence of pediatric leukemia was high in the Kyoto-Otokuni district.

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We retrospectively surveyed for juvenile chronic myelogenous leukemia in Japan. Fifty patients diagnosed from 1986 to 1995 were registered from 47 institutions. The median age of diagnosis was 2 yr 1 mo and the range was from 2 mo to 5 yr 6 mo. The male female ratio was 2 : 1. The overall survival of the evaluable 49 patients was 23.3%. The survival of 14 patients with allogeneic stem cell transplantation (SCT) and 35 patients without it was 49.0% and 13.7%, respectively. Total body irradiation was performed as the condition-ing regimen before SCT in 13 of 14 patients. Poor prognostic factors of the patients who did not receive allogeneic SCT were high age at diagnosis (≥ 2 yr), low platelet count (< 33, 000/μl), and mild splenomegaly (< 5 cm). Four of five patients who survived in good condition without intensive chemotherapy had been diagnosed before one year of age.

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A 12-year-old boy with pernicious anemia (PA) was reported. He was referred to our hospital because of a general malaise and pallor. The blood examination on admission revealed megaloblastic anemia (RBC 1.21 × 10⁶/μl, Hb 4.7 g/dl, MCV 113.9 fl, MCH 39.2 pg) and neutropenia. Serum vitamin B₁₂ was elevated, and the intrinsic factor antibody and parietal cell antibody were positive. A bone marrow smear showed metaplasia and megaloblastic changes in erythroid cells, and giant metamyelocytes and hypersegmented neutrophils were present. Atrophic gastritis was found by endoscopic examination. Results of a Schilling test with ⁵⁷Co-intrinsic factor and ⁵⁸Co were abnormally decreased (1.43 and 0.41%, respectively). Taken together, he was diagnosed as having McIntyre type II PA. He promptly recovered from anemia by vitamin B₁₂ supplement. However, follow-up endoscopic gastrointestinal examinations are necessary because gastric cancer may occur in the presence of atrophic gastritis.

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There had been many reports about cardiac complications of patients with Hodgkin's disease (HD) after cessation of treatment in Europe and the United States. However, cases of HD accompanied with these complications were extremely rare in Japan. We report a case with HD that developed a cardiac tamponade during the clinical course of chemotherapy and showed a pancytopenia after cessation of chemotherapy. The case was a 14 year-old boy with HD (nodular sclerosis) of anterior upper mediastinum origin. He received modified MOPP therapy and irradiation to the semimantle field and to the mediastinum. The total dose of radiation was 36.3 Gy. He suddenly developed a chest pain, chest discomfort, and dyspnea during the sixth course of chemotherapy. He was diagnosed to have a cardiac tamponade by thoracentesis. The symptoms were improved by continuous transcutaneous drainage. The invasion of HD into the effusion was not detected by cytology, and the chemotherapy was completed after one course. However, he gradually developed a macrocytic anemia and finally developed a pancytopenia. The bone marrow specimen revealed a hypoplasia without evidence of pathological cells. The karyotype of marrow derived cells was normal. With oxymetholone administration, the pancytopenia has improved in four months. He has now been doing well for five years after the cessation of therapy.

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We report an 11-year-old girl who had massive bone marrow metastasis at the onset of rhabdomyosarcoma. She was admitted to our department because of general fatigue and purpura of extremities. The labora-tory findings showed thrombocytopenia, hypercalcemia, hyperuricemia, and an elevation of LDH level in her serum. Initially, a bone marrow examination revealed an involvement of blast cells with abundant basophilic cytoplasm reminiscent of leukemia. However, we performed further work because the surface markers of these blast cells were inconsistent with hematological malignancies. Periproctic tumors were found, and the biopsy findings demonstrated alveolar rhabdomyosarcoma. Its chimeric transcripts were also found when PAX3-FKHR chimeric transcripts were examined by reverse transcriptase-polymerase chain reaction (RT-PCR) Therefore we finally made a diagnosis of progressive alveolar rhabdomyosarcoma with cnromosomal translocation of t (2 ; 13) (q35 ; 14). The chimeric transcript in alveolar rhabdomyosarcoma should be considered and studied as a differential diagnosis of bone marrow metastasis presented with leukemia-like symptoms in childhood.

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We describe the case of an 8-year-old boy who suffered a perforation of Meckel's diverticulum followed by septic shock during the reinduction therapy of acute lymphoblastic leukemia. The patient was diagnosed to have acute lymphoblastic leukemia at the age of 5 years and 2 months. Complete remission was attained ; however, meningeal relapse developed three months following the completion of chemotherapy. He received reinduction chemotherapy with vincristine, prednisolone, daunorubicin, cyclophosphamide and triple intrathecal therapy. On day 7 the cerebrospinal fluid specimen revealed 3 mononuclear cells/ml with negative cytology_ On day 11, he complained of abdominal pain ; however, he was afebrile and there was no sign of peritoneal irritation. On day 13, fever, hypotension, and hypoglycemia developed, and 4 hr later he died. An examination at the autopsy revealed peritonitis resulting from a perforated peptic ulceration of Meckel's diver-ticulum. We suggest that Meckel's diverticulitis is one cause of the acute abdomen in the group receiving chemotherapy.

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Macrophage activation syndrome (MAS) in a 2-year-old boy with systemic juvenile rheumatoid arthritis (s-JRA) is reported. MAS developed 3 days after the administration of oral ibuprofen (20 mg/kg/day) for s-JRA, showing a high fever, wasting, hepatomegaly, systemic lymphadenopathy, profound decrease of blood cell counts, liver dysfunction, and coagulopathy. Bone marrow smears showed a few hemophagocytes, but not many. The patient was treated with intravenous prednisolone (PSL) 2 mg/kg/day and cyclosporine A (CyA) 3 mg/kg/day. Apyrexia was obtained within 48 hr with this combination treatment, and the biological abnormality promptly improved. This kind of therapeutic approach, using CyA in combination with PSL, is considered to be very effective for hypercytokinemia, which indicates a poor prognostic factor in HPS.

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