The therapeutic effect of donor leukocyte infusion (DLI) in Japan has been compared with those in Western countries. The effect of DLI is often offset by severe GVHD after infusion of allogeneic donor lymphocyte. Since there is a relatively low incidence of acute GVHD and a similar GVL effect, DLI may be more beneficial to patients in Japan, which is probably due to the tolerance of a relatively high dose of donor lymphocytes. Because of minor histocompatibility antigens (mHa) being involved in both the GVHD and the GVL effect, we provide insights into their roles in donor leukocyte infusions and allogeneic bone marrow transplantation.

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We emphasized the importance of total care to improve the quality of life (QOL) in pediatric hematology/oncology patients and introduced the activities in our institute. Pediatricians must know the mentality of their patients, must share all the truth of their sickness with them, and must have the philosophy for their QOL. These efforts are of prime importance in establishing good relationships with the patients and helping them to cope with their sickness. Furthermore, suitable facilities such as an in-hospital school, a family house, and a terminal care system should be made available to support the lives of the patients and their families. In terminal care, we are to advance home care involving local doctors or hospice care, as well as care by a health-care team at a hospital.

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Chemotherapy for malignant neoplasms sometimes causes unconjugated hyperbilirubinemia without liver dysfunction. We examined the bilirubin UDP-glucuronosyltransferase gene (UGT1A1) from three leukemic patients in whom chemotherapy evoked a hyperbilirubinemic response. The gene of three patients revealed an identical heterozygous missense mutation in exon 1 (G71R). Thus mutation of UGT1A1 may be the genetic cause of chemotherapy-induced unconjugated hyperbilirubinemia.

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Human parvovirus B19 (HPVB19) infection often causes an aplastic crisis in a patient with hereditary spherocytosis (HS). Recent reports showed that the mechanism of aplastic crisis inHS patients is probably associated with hemophagocytic syndrome (HPS). We encountered a boy and his father diagnosed with HS at the onset of aplastic crisis caused by HPVB19 infection. Bone marrow smears from both patients revealed hemophagocytosis and serum cytokines, such as sIL-2R, IL-6, and TNF-a, were remarkably elevated at the time of diagnosis. Besides the arrest of erythroid cell maturation, we consider hemophagocytosis is a cause of aplastic crisis in patients with HS because of HPVB19 infection.

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Recently, a few cases of lysinuric protein intolerarlce (LPI), a congenital transport disorder of dibasic amino acids, associated with hemophagocytic lymphohistiocytosis (HLH) have been reported. We report a 19-year old man with LPI-associated HLH that developed at age 13. In this patient, HLH had a self-limited and nonrelapsing course but splenomegaly, high serum levels of lactate dehydrogenase and ferritin, and an abnormal lymphocytes subpopulation persisted. Metabolic examinations revealed increased urinary secretion and decreased plasma concentrations of dibasic amino acids as well as hyperammonemia after protein loading. Moreover, serum levels of interleukin-2 receptor and interleukin-1 βincreased even long after the HLH episode, suggesting that an immunologic dysregulation involved in LPI may have played a ro1e in the development of HLH. Because hyperammonemia in LPI is manageable with citrulline replacement therapy and LPI-associated HLH is possibly benign in nature, we should be aware of LPI as one of several underlying conditions in HLH.

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A 15-year-old boy visited a nearby hospital because of paleness and general fatigue. He also exhibited pancytopenia, so we admitted him to our hospital. The bone marrow showed normocellular and trilineage dysplastic changes with 4.9% type I blasts. Bone marrow cell analysis showed a normal 46, XY karyotype by G-banding without monosomy 7 by FISH analysis. A diagnosis of refractory anemia (RA) was made. He received red cell transfusions or platelet transfusions once a week. A month after antihuman thymocyte immunoglobulin (ATG) therapy, he became independent of transfusions and continued stable hematological responses with no other therapy. The bone marrow examinations were performed every 4 or 5 months after the ATG treatment. Dysplastic changes in marrow cells and blasts have disappeared. The chromosomal abnormalities have not appeared. We suggest that ATG therapy is beneficial for patients with RA without cytogenetic abnormalities and increase of marrow blasts.

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We report a 15-year-old boy who developed bronchiolitis obliterans organizing pneumonia (BOOP) 6 months after allogeneic bone marrow transplantation from an HLA-matched elder sister for acute myeloblastic leukemia during the refractory phase. We successfully confirmed engraftment 17 days after infusion of 5.8 X 10⁸/ kg mononuclear donor cells to the patient. The patient received cyclosporin A and short-term MTX for graft-versus-host disease (GVHD) prophylaxis. He showed grade I acute GVHD of the skin. We performed two courses of donor lymphocyte transfusion (DLT) to eradicate leukemic cells. The patient developed a nonproductive cough without signs of chronic GVHD 6 months after BMT. The results from the pulmonary function test, imaging study, and bronchoscopic lung biopsy were consistent with the diagnosis of BOOP. The patient was treated with oral prednisolone (1 mg/kg/day), and his clinical status and chest imaging findings improved dramatically after 2 weeks.

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We report three cases of mediastinal T-cell non-Hodgkin's lymphoma (T-NHL) that failed to achieve complete remission. All patients were diagnosed histologically as having lymphoblastic lymphoma and categorized as stage III according to Murphy's classification. Patient 1, a 14-year-old boy, underwent allogeneic bone marrow transplantation during the disease's active stage. The tumor was found on day 43 to have regrown both at the primary site and at the pleural cavity. Patient 2, a 10-year-old girl, was treated repeatedly with intensive chemotherapy. She had an enlargement of a residual mediastinal mass, and her disease had disseminated both into the pleural cavity and into the abdomen at 10 months after diagnosis. In both patients, the tumors became refractory to the chemotherapy and local irradiation after the disease had progressed. They died approximately 1 year after diagnosis. Patient 3, a 7-year-old boy, received a surgical resection of a huge mediastinal mass 2 months after the initial chemotherapy. He received autologous peripheral blood stem cell transplantation after the surgical resection and has remained in complete remission for 30 months since diagnosis. Our present report suggests that an aggressive surgical resection of the residual tumor will be an effective therapeutic option in the treatment of mediastinal T-NHL, especially for those having a poor initial response to chemotherapy.

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