lysis of DOP-M1 cells revealed 47,X,-X,-13,+19,+20,+mar. Our established cell line, DOP-M1 appears to be a cell line which will be a useful tool for studying the phenotype, morphology, and function of monocytoid cells.
1
TI: Partition of free dolichol in human urine.
AU: Sakakihara-Y; Kamoshita-S
SO: Biochem-Cell-Biol. 1992 Jun; 70(6): 518-21
AB: We have demonstrated that dolichol is present in the urinary supernatant. Most of the dolichol present in the supernatant seems to be associated with cellular debris or membrane fragments. The amount of sediment in healthy subjects correlate well with the volume of urine. Although it is illogical to express urinary dolichol relative to urine volume, a good correlation between the amount of sediment and urine volume has made its use justifiable. Because of the presence of a substantial amount of dolichol in the supernatant, it seems better to use uncentrifuged whole urine as the sample for measurement of dolichol.
2
TI: [Autonomic dysfunction in metabolic diseases]
AU: Sakakihara-Y
SO: Nippon-Rinsho. 1992 Apr; 50(4): 811-7
AB: Among many metabolic disorders, porphyrias and Fabry disease are known to affect autonomic nervous system. In patients with acute intermittent porphyria, hereditary coproporphyria, and variegate porphyria, autonomic symptoms such as abdominal pain, vomiting, hypertension and tachycardia are among the most prominent clinical manifestations. Fabry disease is clinically characterized by severe limb pain, hypohidrosis, angiokeratomas and various autonomic symptoms. In both porphyrias and Fabry disease, pathological changes in the central and peripheral autonomic nervous system have been documented. In porphyrias, a loss of myelinated fibers, axonal degeneration, and segmental demyelination in peripheral autonomic nerves as well as chromatolysis of several brain stem nuclei have been found. In Fabry disease, abnormal amount of the substrates of alpha-galactosidase, i.e. ceramide di- and trihexoside, are found to be accumulated in the central and peripheral autonomic nerves.
3
TI: [E2A gene in t(1;19)-ALL]
AU: Hayashi-Y
SO: Nippon-Rinsho. 1992 Jun; 50(6): 1369-73
AB: The t(1;19)(q23;p13) seen in approximately 5% of childhood acute lymphoblastic leukemia (ALL) has been reported to be associated with leukoencephalopathy. 1;19 translocation can alter the E2A gene, leading to formation of a chimeric E2A-PBX1 gene that retain the activator domain of the E2A gene but substitute a homeobox domain of the PBX1 gene for the helix-loop-helix DNA binding and dimerization domain of E2A. The translocation breakpoints occurs within a single intron of the E2A gene on chromosome 19, and interrupts a homeobox gene, PBX1, on chromosome 1q23. Most cases with t(1;19) have been identified to have rearranged band of E2A by Southern blotting analysis, and to contain identical E2A-PBX1 chimeric transcripts by use of polymerase chain reaction assay. The molecular breakpoints in pre-B cases differ from those in early pre-B cases among t(1;19)-ALL. Thus, molecular analysis is useful for detection of t(1;19)-ALL.
4
TI: Leukoencephalopathy in children with t(1;19) acute lymphoblastic leukaemia [letter]
AU: Hayashi-Y; Hanada-R; Yamamoto-K; Taguchi-N; Shikano-T
SO: Lancet. 1992 Aug 1; 340(8814): 316
5
TI: Different molecular consequences of the 1;19 chromosomal translocation in childhood B-cell precursor acute lymphoblastic leukemia.
AU: Privitera-E; Kamps-MP; Hayashi-Y; Inaba-T; Shapiro-LH; Raimondi-SC; Behm-F; Hendershot-L; Carroll-AJ; Baltimore-D; et-al
SO: Blood. 1992 Apr 1; 79(7): 1781-8
AB: The prognostically important 1;19 chromosomal translocation can alter the E2A gene on chromosome 19p13 in childhood B-cell precursor acute lymphoblastic leukemia (ALL), leading to formation of a fusion gene (E2A-PBX1) that encodes a hybrid transcription factor with oncogenic potential. It is not known whether this molecular alteration is a uniform consequence of the t(1;19) or is restricted to translocation events within specific immunologic subtypes of the disease. Therefore, we studied leukemic cells from 25 cases of B-cell precursor ALL, with or without evidence of cytoplasmic Ig mu heavy chains (cIg); 17 cases had the t(1;19) by cytogenetic analysis. Leukemic cell DNA samples were analyzed by Southern blotting to detect alterations within the E2A genomic locus; a polymerase chain reaction assay was used to identify expression of chimeric E2A-pbx1 transcripts in leukemic cell RNA; and immunoblotting with anti-Pbx1 antibodies was used to detect hybrid E2A-Pbx1 proteins. Of 11 cases of cIg+ ALL with the t(1;19), 10 had E2A-pbx1 chimeric transcripts with identical junctions and a characteristic set of E2A-Pbx1 hybrid proteins. Each of these cases had E2A gene rearrangements, including the one in which fusion transcripts were not detected. By contrast, none of the six cases of t(1;19)-positive, cIg- ALL had evidence of rearranged E2A genomic restriction fragments, detectable E2A-pbx1 chimeric transcripts, or hybrid E2A-Pbx1 proteins. Typical chimeric E2A-pbx1 transcripts and proteins were detected in one of eight cIg+ leukemias in which the t(1;19) was not identified by cytogenetic analysis, emphasizing the increased sensitivity of molecular analysis for detection of this abnormality. We conclude that the molecular breakpoints in cases of cIg- B-cell precursor ALL with the t(1;19) differ from those in cIg+ cases with this translocation. Leukemias that express hybrid oncoproteins such as E2A-Pbx1 or Bcr-Abl have had a poor prognosis in most studies. Thus, molecular techniques to detect fusion genes and their aberrant products should allow more timely and appropriate treatment of these aggressive subtypes of the disease.
6
TI: Establishment of a monocytoid cell line (DOP-M1) from an infant with acute monocytic leukemia.
AU: Sugita-K; Sakakibara-H; Eguchi-M; Hayashi-Y; Furukawa-T
SO: Int-J-Hematol. 1992 Feb; 55(1): 45-51
AB: A monocytoid cell line (DOP-M1) was established from mononuclear cells separated from the cerebrospinal fluid of a 1-year-old girl with acute monoblastic leukemia (AMoL) (French-American-British; FAB-M5a). Judged by morphological, cytochemical, and immunological criteria, the DOP-M1 cells showed immature monocytoid characteristics. They were positive for alpha-naphthyl butyrate esterase, the expression of which was inhibited by NaF, and for myeloperoxidase (MPO). Positive MPO findings in nuclear envelope were detectable by electron microscopy. The cell surface was positive for CD15, CD33, and CDw65, but negative for CD4, CD14, and HLA-DR. HLA-DR expression was detected after treatment with IFN-gamma. Chromosome ana
1
TI: [E2A gene in t(1;19)-ALL]
AU: Hayashi-Y
SO: Nippon-Rinsho. 1992 Jun; 50(6): 1369-73
AB: The t(1;19)(q23;p13) seen in approximately 5% of childhood acute lymphoblastic leukemia (ALL) has been reported to be associated with leukoencephalopathy. 1;19 translocation can alter the E2A gene, leading to formation of a chimeric E2A-PBX1 gene that retain the activator domain of the E2A gene but substitute a homeobox domain of the PBX1 gene for the helix-loop-helix DNA binding and dimerization domain of E2A. The translocation breakpoints occurs within a single intron of the E2A gene on chromosome 19, and interrupts a homeobox gene, PBX1, on chromosome 1q23. Most cases with t(1;19) have been identified to have rearranged band of E2A by Southern blotting analysis, and to contain identical E2A-PBX1 chimeric transcripts by use of polymerase chain reaction assay. The molecular breakpoints in pre-B cases differ from those in early pre-B cases among t(1;19)-ALL. Thus, molecular analysis is useful for detection of t(1;19)-ALL.
2
TI: Leukoencephalopathy in children with t(1;19) acute lymphoblastic leukaemia [letter]
AU: Hayashi-Y; Hanada-R; Yamamoto-K; Taguchi-N; Shikano-T
SO: Lancet. 1992 Aug 1; 340(8814): 316
3
TI: Different molecular consequences of the 1;19 chromosomal translocation in childhood B-cell precursor acute lymphoblastic leukemia.
AU: Privitera-E; Kamps-MP; Hayashi-Y; Inaba-T; Shapiro-LH; Raimondi-SC; Behm-F; Hendershot-L; Carroll-AJ; Baltimore-D; et-al
SO: Blood. 1992 Apr 1; 79(7): 1781-8
AB: The prognostically important 1;19 chromosomal translocation can alter the E2A gene on chromosome 19p13 in childhood B-cell precursor acute lymphoblastic leukemia (ALL), leading to formation of a fusion gene (E2A-PBX1) that encodes a hybrid transcription factor with oncogenic potential. It is not known whether this molecular alteration is a uniform consequence of the t(1;19) or is restricted to translocation events within specific immunologic subtypes of the disease. Therefore, we studied leukemic cells from 25 cases of B-cell precursor ALL, with or without evidence of cytoplasmic Ig mu heavy chains (cIg); 17 cases had the t(1;19) by cytogenetic analysis. Leukemic cell DNA samples were analyzed by Southern blotting to detect alterations within the E2A genomic locus; a polymerase chain reaction assay was used to identify expression of chimeric E2A-pbx1 transcripts in leukemic cell RNA; and immunoblotting with anti-Pbx1 antibodies was used to detect hybrid E2A-Pbx1 proteins. Of 11 cases of cIg+ ALL with the t(1;19), 10 had E2A-pbx1 chimeric transcripts with identical junctions and a characteristic set of E2A-Pbx1 hybrid proteins. Each of these cases had E2A gene rearrangements, including the one in which fusion transcripts were not detected. By contrast, none of the six cases of t(1;19)-positive, cIg- ALL had evidence of rearranged E2A genomic restriction fragments, detectable E2A-pbx1 chimeric transcripts, or hybrid E2A-Pbx1 proteins. Typical chimeric E2A-pbx1 transcripts and proteins were detected in one of eight cIg+ leukemias in which the t(1;19) was not identified by cytogenetic analysis, emphasizing the increased sensitivity of molecular analysis for detection of this abnormality. We conclude that the molecular breakpoints in cases of cIg- B-cell precursor ALL with the t(1;19) differ from those in cIg+ cases with this translocation. Leukemias that express hybrid oncoproteins such as E2A-Pbx1 or Bcr-Abl have had a poor prognosis in most studies. Thus, molecular techniques to detect fusion genes and their aberrant products should allow more timely and appropriate treatment of these aggressive subtypes of the disease.
4
TI: Establishment of a monocytoid cell line (DOP-M1) from an infant with acute monocytic leukemia.
AU: Sugita-K; Sakakibara-H; Eguchi-M; Hayashi-Y; Furukawa-T
SO: Int-J-Hematol. 1992 Feb; 55(1): 45-51
AB: A monocytoid cell line (DOP-M1) was established from mononuclear cells separated from the cerebrospinal fluid of a 1-year-old girl with acute monoblastic leukemia (AMoL) (French-American-British; FAB-M5a). Judged by morphological, cytochemical, and immunological criteria, the DOP-M1 cells showed immature monocytoid characteristics. They were positive for alpha-naphthyl butyrate esterase, the expression of which was inhibited by NaF, and for myeloperoxidase (MPO). Positive MPO findings in nuclear envelope were detectable by electron microscopy. The cell surface was positive for CD15, CD33, and CDw65, but negative for CD4, CD14, and HLA-DR. HLA-DR expression was detected after treatment with IFN-gamma. Chromosome analysis of DOP-M1 cells revealed 47,X,-X,-13,+19,+20,+mar. Our established cell line, DOP-M1 appears to be a cell line which will be a useful tool for studying the phenotype, morphology, and function of monocytoid cells.
5
TI: Treatment of acute lymphoblastic leukemia in the Tokyo Children's Cancer Study Group--preliminary results of L84-11 protocol.
AU: Tsuchida-M; Akatsuka-J; Bessho-F; Chihara-H; Hayashi-Y; Hoshi-Y; Hosoya-R; Furukawa-T; Ikuta-K; Inana-I; et-al
SO: Acta-Paediatr-Jpn. 1991 Aug; 33(4): 522-32
AB: The Tokyo Children's Cancer Study Group (TCCSG) has performed prospective randomized controlled studies since 1984 for childhood acute lymphoblastic leukemia (ALL). Four hundred and ninety-eight newly diagnosed patients were treated with 5 different regimens designated as S1, S2 for a standard risk group (SRG), H1 and H2 for a high risk group (HRG) and HEX for an extremely high risk group of patients. We added intermediate-dose methotrexate as early intensification therapy in the S2 and H2 groups to reduce extramedullary and medullary relapses. Event-free survival of all patients at 4 years 6 months from the start of this regimen (median follow-up period 32 months) was 67.5%. The CNS relapse rate was significantly decreased to 2.2% compared to previously reported studies and our own experience. There were some unexpected complications of the CNS such as myelopathy and/or leukoencephalopathy. For the SRG early ID-MTX seemed to be useful to improve the prognosis, but triple intrathecal injections (TIT) should be limited in number. The 24Gy cranial irradiation (CRX) was effective but possibly excessive for the HRG. As evidenced by the success of the HEX group regimen, more intensive multi-drug consolidation in the early post-remission phase might be necessary to improve further the prognosis and to make it possible to shorten the treatment periods.
7
TI: Treatment of acute lymphoblastic leukemia in the Tokyo Children's Cancer Study Group--preliminary results of L84-11 protocol.
AU: Tsuchida-M; Akatsuka-J; Bessho-F; Chihara-H; Hayashi-Y; Hoshi-Y; Hosoya-R; Furukawa-T; Ikuta-K; Inana-I; et-al
SO: Acta-Paediatr-Jpn. 1991 Aug; 33(4): 522-32
AB: The Tokyo Children's Cancer Study Group (TCCSG) has performed prospective randomized controlled studies since 1984 for childhood acute lymphoblastic leukemia (ALL). Four hundred and ninety-eight newly diagnosed patients were treated with 5 different regimens designated as S1, S2 for a standard risk group (SRG), H1 and H2 for a high risk group (HRG) and HEX for an extremely high risk group of patients. We added intermediate-dose methotrexate as early intensification therapy in the S2 and H2 groups to reduce extramedullary and medullary relapses. Event-free survival of all patients at 4 years 6 months from the start of this regimen (median follow-up period 32 months) was 67.5%. The CNS relapse rate was significantly decreased to 2.2% compared to previously reported studies and our own experience. There were some unexpected complications of the CNS such as myelopathy and/or leukoencephalopathy. For the SRG early ID-MTX seemed to be useful to improve the prognosis, but triple intrathecal injections (TIT) should be limited in number. The 24Gy cranial irradiation (CRX) was effective but possibly excessive for the HRG. As evidenced by the success of the HEX group regimen, more intensive multi-drug consolidation in the early post-remission phase might be necessary to improve further the prognosis and to make it possible to shorten the treatment periods.
　
　　　　　　　　　　　　　　　　　　　　　　　　　

Edited by Toshio Hishi