Guidelines for safety management of granulocyte transfusion in Japan.

Granulocyte transfusion (GTX) has recently been revived by the ability to stimulate granulocyte donors with granulocyte colony-stimulating factor (G-CSF), resulting in a greatly increased number of cells that can be collected. However, there is a paucity of guidelines for assessing the appropriateness and safety management of GTX. The objective of this study was to establish guidelines for the safety management of GTX appropriate for the clinical situation in Japan. The Japan Society of Transfusion Medicine and Cell Therapy, Granulocyte Transfusion Task Force issued the first version of guidelines for GTX considering the safety management of both granulocyte donors and patients who receive GTX therapy. The current guidelines cover issues concerning: (1) the appropriateness of medical institutions, (2) management of granulocyte donors, (3) quality assurance of granulocyte concentrates, (4) administration of granulocyte concentrates, (5) evaluation of the effectiveness of GTX therapy, and (6) complications of GTX therapy. The simple 'bag separation method' without apheresis may be recommended for granulocyte collection in pediatric patients. The first version of guidelines for GTX therapy has been established, which may be appropriate for the clinical situation in Japan. Care should be taken to perform the safety management of both granulocyte donors and patients who receive GTX therapy.

Multiple immune abnormalities in a patient with idiopathic CD4+ T-lymphocytopenia.

Idiopathic CD4+ T-lymphocytopenia (ICL) is a new disease entity characterized by CD4+ T-lymphocyte depletion without evidence of HIV infection. We report a 27-year-old ICL patient with a long history of multiple immune abnormalities. His CD4+ T-lymphocyte count started to decrease after generalized lymphadenopathy of an unknown cause at age 3. He satisfied the criteria for ICL at age 9, and the decreased CD4+ T-lymphocyte count persisted for more than 18 years. This is probably the first childhood-onset ICL case in which the trigger event for the development was known together with the patient's autoimmune background.

Reconstruction of a pelvic floor defect using a pedicled tensor fascia lata flap: a new technique to prevent radiation injury for pediatric patients with advanced pelvic tumors.

BACKGROUND: In the treatment of pelvic tumors, pelvic floor defects owing to a wide excision tend to increase the occurrence of such morbidities as radiation injury. The reconstruction of these defects would minimize the risk of such morbidities. Authors introduce a new technique for repairing a pelvic floor defect using a tensor fascia lata flap. METHODS: Two boys, 4 years old and 10 months old, presenting with pelvic rhabdomyosarcoma underwent a tumor extirpation associated with a wide excision of the pelvic organs. After the removal of the tumor, a tensor fascia lata flap was designed on the right thigh. The pedicled rotation flap was subcutaneously elevated, guided to the intraperitoneal cavity, and was fixed to cover the superior aperture of the lesser pelvis. RESULTS: The flaps functioned well, and postoperative radiation therapies consisting of 45 and 41.4 Gy to the lesser pelvic cavity were carried out without any complications. As a result, the necessary postoperative protocol combination therapies could be successfully performed in a timely manner. CONCLUSION: The pedicled tensor fascia lata flap is considered to be an alternative option for the stable repair of pelvic floor defects to prevent radiation injury.

Congenital occurrence of solitary infantile myofibromatosis of the spleen.

Infantile myofibromatosis (IM) is a rare soft tissue tumor of infancy and childhood. We report the case of a newborn girl with an abdominal tumor discovered at 32 weeks of gestation by fetal ultrasound. She underwent a laparotomy for an unexplained abdominal mass 20 days after birth. The tumor originated from the spleen and was removed by splenectomy. There were no other abnormal findings on diagnostic modalities. Based on the histological examinations, the tumor was diagnosed as an IM. Although extremely rare during the neonatal period, solitary type IM should be considered as a differential diagnosis of the splenic tumor.

Congenital intrarenal teratoma arising from a horseshoe kidney.

We report a case of intrarenal teratoma arising from a horseshoe kidney. A 6-day-old girl was admitted with an abdominal mass that had been noticed at 37 weeks gestation by routine ultrasonography. At 20 days of age, a tumorectomy with a right nephrectomy was performed. The pathological diagnosis was an immature teratoma. An intrarenal teratoma is extremely rare. In addition, we believe that this case represents the first case of an immature teratoma occurring in a horseshoe kidney. The diagnosis in this type of case is difficult, but we recommend that such tumors be included in the differential diagnosis.

Langerhans' cell histiocytosis after living donor liver transplantation: report of a case.

We report a case of Langerhans' cell histiocytosis (LCH) occurring after a living donor liver transplantation (LDLT) for fulminant hepatitis. A 9-month-old girl underwent an LDLT for fulminant hepatitis of an unknown cause. The histology of the native liver did not show any findings of LCH. On postoperative day 42, her Epstein-Barr virus (EBV)-DNA and cytomegalovirus antigenemia were both found to be positive. As a result, she was treated with antiviral agents and a reduction of the immunosuppression dosage. On postoperative day 98, acute rejection occurred, and she was treated with FK506, methylprednisolone, and finally, anti-CD3 murine monoclonal antibody was added. Subsequently, the EBV was re-activated. Thereafter, skin eruptions, swelling of the systemic lymph nodes, and pancytopenia appeared on postoperative day 127. LCH was diagnosed based on the typical histological findings as LCH, CD1a, and S-100-positive cells in her skin and a lymph nodes biopsy. She was treated by chemotherapy. The symptoms disappeared a few weeks after the start of the chemotherapy, and a clinical remission of LCH was obtained. We could not detect any evidence of EBV infection in the tumor cells. In spite of the fact that her LCH lesions thereafter remained in remission, she died of hepatic failure at 22 months after undergoing the liver transplantation. In conclusion, we discuss the factors influencing the occurrence of LCH in our patient after LDLT, while also evaluating the relationship between LCH and the immunosuppressive therapy administered to this patient.

Identical reconstitution after bone marrow transplantation in twins who received fresh and cryopreserved grafts harvested at the same time from their older brother.

We report here the reconstitution after bone marrow transplantation (BMT) in identical infant twins with acute myelogenous leukemia (AML). They were diagnosed at 8 and 9 months of age. Complete remission was induced after two courses of chemotherapy. After four and five courses of chemotherapy, respectively, they received BMT at 2-month interval from the same HLA-identical older brother. The total dose of marrow nucleated cells (NC) harvested was 77.7 x 10(8). The first patient was transplanted with half of the total dose of NC. The remaining cells were cryopreserved without the use of a programmed freezer and transplanted into the second patient 2 months later. The number of days for neutrophil (>0.5 x 10(9)/L), platelet (>50 x 10(9)/L), and reticulocyte (>1%) recovery were, respectively, 15, 21, and 14 in the first case and 12, 21, and 15 in the second case. The clinical courses after BMT were uneventful in both cases, except for mild acute GVHD, and complete remission has been maintained >4 yr with full recovery of immune and marrow function. Based on the results in these cases, we confirmed that marrow cells that have been cryopreserved without the use of a programmed freezer could reconstitute immune and marrow function as well as non-cryopreserved cells.

Unstable hemoglobinemia, Hb Buenos Aires, Bryn Mawr, followed up for eighteen years.

A 20-year-old man has been under observation for 18 years because of unstable hemoglobinemia, Hb Buenos Aires, Bryn Mawr (beta-globin, Phe85Ser). At the age of 19 years, he was hospitalized because of fever and hemolytic crisis, and the symptoms resolved after infusion of antibiotics. Nucleotide sequencing of the beta-globin gene confirmed that the patient was heterozygous for the mutation. The patient's erythrocytes showed an increased affinity for oxygen and a prolonged glycerol lysis time. We review a previously reported single family and 5 other cases, and discuss the clinical significance of splenectomy and plasma-derived haptoglobin.

Minimal residual disease in early phase of chemotherapy reflects poor outcome in children with acute lymphoblastic leukemia--a retrospective study by the Children's Cancer and Leukemia Study Group in Japan.

We analyzed the minimal residual disease (MRD) in 50 children with acute lymphoblastic leukemia (ALL) by amplifying the clonally rearranged T-cell receptor (TCR) gamma/delta chain and/or immunoglobulin (Ig) kappa chain gene using the allele-specific-PCR method. All children were treated according to the protocols of the Children's Cancer and Leukemia Study Group of Japan (CCLSG). The patients were stratified into four risk-groups according to the leukocyte count and age at diagnosis. We prospectively sampled the patients' bone marrow at 1 month (point 1) and 3 months (point 2) after the initiation of chemotherapy and quantitated the MRD retrospectively. The results of MRD were closely related with the clinical outcome. The relapse rate of the patients MRD-positive at points 1 and 2 was 46% (6/13) and 86% (6/7), respectively, whereas those MRD-negative results at point 1 and 2 were 13% (3/13) and 3% (3/30), respectively. We found significant differences in the event-free survival between MRD-positive children and MRD-negative children like the reports, which have been made by BFM and EORTC groups. We conclude that MRD in an early phase of chemotherapy can be a good predictor of the prognosis of childhood ALL regardless of the protocol of chemotherapy or race.