Mayumi Takamura

Hemophagocytic syndrome and hepatosplenic gammadelta T-cell lymphoma with isochromosome 7q and 8 trisomy.

The authors describe a 15-year-old boy with hepatosplenic gammadelta T-cell lymphoma associated with hemophagocytic syndrome (HPS) along with isochromosome 7q and trisomy 8. He presented with prolonged fever, mild anemia, thrombocytopenia, and hepatosplenomegaly. Physical examination, radiography, and ultrasound tomography revealed no lymphoadenopathy. He had elevated levels of serum ferritin, interferon-gamma, soluble interleukin-2 receptor, and interleukin-6. Bone marrow aspirate showed hypercellularity with 50% lymphoblasts and erythrophagocytosis of macrophage. A cytogenetic study of bone marrow revealed an abnormal karyotype, 47,XY,I(7q),+8, in 5/30 cells. Clonal rearrangement of the genes for T-cell receptor gamma and delta chains was elucidated by polymerase chain reaction. He achieved a complete remission after intensive chemotherapy and underwent splenectomy 18 months after diagnosis. Although the patient was clinically in remission, minimal residual disease (MRD) was detected in the removed spleen by polymerase chain reaction. This might mean that this type of lymphoma is refractory, as reported previously, and might indicate that marrow ablative therapy is needed to achieve a cure. The present case illustrates the usefulness of MRD analysis, and MRD studies in this group of disorders may be helpful in the decision of whether to continue a more aggressive therapeutic approach.

Type la glycogen storage disease with focal nodular hyperplasia in siblings

Glycogen storage disease type I (GSD‐I) is an inherited disorder that is due to a glucose‐6‐phosphatase (G6Pase) deficiency. There have been recent reports of hepatocellular tumors in adults with this disease. Hepatic adenoma is the most common tumor described but others, including hepatocellular carcinomas, hepatoblastomas, and focal nodular hyperplasia (FNH) have been reported.

Comprehensive treatment of advanced neuroblastoma involving autologous bone marrow transplant

Encouraging results are reported with high‐dose chemotherapy and total body irradiation followed by autologous bone marrow transplantation in the treatment of advanced neuroblastoma. However, relapse remains a significant problem.

Successful autologous bone marrow transplant for relapsed Ki-1 lymphoma.

We report on a 16 year old girl with relapsed Ki‐1 lymphoma and a very poor prognosis. The initial manifestation was multiple bone metastases and lymphadenopathy. The patient achieved remission with modified adriamycin, bleomycin, vincristine, daunomycine therapy. However, 14 months after the completion of therapy, relapse occurred in a new cervical lymph node on the left side. After preparation with chemotherapy and total lymphoid irradiation (TLI) the patient underwent autologous bone marrow transplantation (A‐BMT).

Analytical Chemistry in Medical Science and Technology. Continuous measurement of retinoic acid isomers and retinol in a plasmaby column-switching reversed-phase HPLC.

独自の流路系を持つカラムスイッチング高速液体クロマトグラフィー(HPLC)により,レチノイン酸(RA)異性体である13-cis-RA,all-trans-RAと9-cisRAの相互分離を確立した.カラムスイッチングを用いることにより,カラム中に残る生体成分の洗浄が不要となり,カラム洗浄に要する時間が短縮された.又,流路中に独自の流路を加えたことにより,注入した試料を損なうことなく検出できるようになり,より良い感度と再現性が得られた.そのため,1サイクルが25分で生体成分中のRAの連続測定が可能となった.ピーク面積及び高さを用いた絶対検量線法では,共に200ngまでの間で直線の関係を示し,血中濃度を測定するのに十分な結果が得られた.又,メタノール標準試料で0.5～1.9%(n=10,0.26～0.37μg/ml),標準添加血清試料で0.8～2.2%(n=5,0.32及び0.37μg/ml)の再現性が得られた.これらのことにより,従来のRA血中濃度測定の方法よりも簡便性,操作性,再現性の向上が見られ,本法のRA血中濃度測定の有用性が示唆された.