Tetsunori Funabiki

Establishment of an epithelioid malignant schwannoma cell line (YST-1).

SummaryA novel cell line, YST-1, was established from an epithelioid malignant schwannoma (EMS) that occurred in the upper arm of an 8-year-old girl. YST-1 cells were polygonal and stellate in shape, contained abundant free ribosomes, mitochondria, lysosomes and rough-surfaced endoplasmic reticulum, and grew stably with a population doubling time of 40 h. Immunohistochemically, vimentin, S100 protein and S100 protein beta subunit were positive in the cytoplasm. The xenotransplanted tumor in nude mice was composed of cells with an epithelioid arrangement similar to the original tumor. The borders of the tumor cells were connected intimately without desmosomal junctions, and there were abundant organelles in the cytoplasm. YST-1 cells were considered to be of value for studying the nature and histogenesis of EMS.

Establishment and characterization of a malignant melanoma cell line (YP-MEL) derived from a patient with neurocutaneous melanosis.

A cell line, YP-MEL, was established from an intracranial malignant melanoma occurring in a neurocutaneous melanosis (NCMsis) patient. The established cell line was successfully cultured in serum-free medium with a doubling time of 41 h. The cells were refractile and small in size, with occasional pigmented giant cells. Histochemical and immunohistochemical features were compatible with common malignant melanoma and its cell line. Chromosome analysis revealed many supernumerary chromosomes and marker chromosomes including double minutes (DMs). When transplanted into nude mice, YP-MEL formed tumors histologically consistent with the original tumor. Addition of sera to the medium caused cellular spreading and elongation of cytoplasmic processes with an increase of melanin contents and tyrosinase activity. Because there was no melanoma cell line derived from a NCMsis patient, YP-MEL might be a beneficial tool for study on NCMsis.

Long-term Follow-up of Busulfan, Etoposide, and Nimustine Hydrochloride (ACNU) or Melphalan as Conditioning Regimens for Childhood Acute Leukemia and Lymphoma

We retrospectively evaluated early and long-term complications of an intensified conditioning regimen consisting of busulfan and etoposide in combination with either nimustine hydrochloride (ACNU) (BVA regimen, n = 18) or melphalan (BVL regimen, n = 34) in 52 children with acute leukemia or non-Hodgkin’s lymphoma. With a median follow-up of 13.2 years after the BVA regimen and 8.1 years after the BVL regimen, 61% and 76% of patients, respectively, are in continuous complete remission. Transplantation-related mortality was 17% and 6% after the BVA and BVL regimens, respectively, and the corresponding relapse rates were 17% and 15%. The most common and severe toxicity was pulmonary complication in the BVA regimen, which was seen in 67% of patients and was life-threatening in 20%. Thirty-three percent of patients after the BVA regimen and 24% after BVL died of relapse or disease progression (n = 9), interstitial pneumonia (n = 2), fungal pneumonia (n = 1), or chronic graft-versus-host disease (n = 2). One of the long-term survivors developed secondary leukemia. A significant decrease in the height standard deviation score of more than 2 SD from diagnosis to the last follow-up was seen in 17% of the patients, with hypothyroidism in 15%, and alopecia in 42%. Because our experience is limited to a small heterogeneous population of patients who mainly underwent transplantation in the first remission, we cannot draw conclusions on the treatment’s effectiveness. The BVL regimen is tolerable, however, because no regimen-related death was observed, whereas the BVA regimen is not recommended because of the high incidence of pulmonary complications. The effectiveness of the BVL regimen requires further study.

Toxic shock syndrome toxin‐1‐mediated exanthematous disease in a burned infant

Toxic exanthem is a common problem in the pediatric practice setting. Some cases can be attributed to viral infection and others to drug allergy, but several cases are of unknown etiology. Recently, we had a case in which a scalded infant presented with toxic exanthem mediated by toxic shock syndrome toxin-1 (TSST-1). TSST-1, secreted by Staphylococcus aureus , acts as a super antigen and activates polyclonal V 2 + T cells, causing toxic shock syndrome (TSS). 1 Several cases of TSS in burned children have been reported, 2 – 4 but no studies, except one in a non-burned infant, 5 have reported only toxic exanthem. In the present report we propose a possible etiology of toxic exanthem and describe the usefulness of fl ow cytometry analysis as a diagnostic modality.

DOUBLE ALLOGENEIC PERIPHERAL BLOOD STEM CELL TRANSPLANTATION IN A 9-YEAR-OLD PATIENT WITH RELAPSED ACUTE LYMPHOBLASTIC LEUKEMIA: Case Report

A 9-year-old female patient with relapsed leukemia that was refractory to conventional reinduction chemotherapy was successfully treated with double allogeneic peripheral blood stem cell transplantation. The conditioning regimen for the first transplantation consisted of busulfan, etoposide, and melphalan, and that for the second transplantation was total body irradiation and thiotepa. Neither severe regimen-related toxicity nor graft-versus-host disease was observed. The patient is in complete remission without major complications for 5 years. Double transplantation should be considered as one of the possible treatments for refractory acute lymphoblastic leukemia.

Effects of recombinant thrombopoietin on the growth of murine primitive and committed hematopoietic progenitors in serum-free culture.

Abstract Background: The aim of the present study was to determine the effect of thrombopoietin (Tpo) in combination with other cytokines on the growth of murine megakaryocytic, granulocyte–macrophage, erythroid and primitive hematopoietic progenitor cells, excluding possibilities of synergistic effects by serum factor(s).