H Sugiyama, S Ichiba, Y Okuno, T Takahashi, H Imura, K Nakamura, S Iho, T Hoshino, T Okada, H Furukawa
{"title":"Cytogenetic evidence for a clonal disorder involving CFU-GEMM, BFU-E and CFU-C in patients with myeloproliferative disorders.","authors":"H Sugiyama, S Ichiba, Y Okuno, T Takahashi, H Imura, K Nakamura, S Iho, T Hoshino, T Okada, H Furukawa","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>We grew multilineage hemopoietic colonies in vitro from 4 patients with myeloproliferative disorders (MPD) having chromosomal aberration, and performed cytogenetic analysis of single CFU-GEMM, BFU-E and CFU-C-derived colonies on Day 14 of culture. In Patient 1 with acute myelofibrosis, CFU-GEMM and BFU-E colonies had only a 47, XX, +C karyotype. In Patient 2 with polycythemia vera, CFU-GEMM, BFU-E and CFU-C colonies all showed the same abnormal karyotype of 46, XY, -12, +der(12)t(1;12). Patients 3 and 4 had primary myelofibrosis, and their respective karyotypes were 46, XY, 13q- and 46, XX, -6, +der(6)t(1;6) in CFU-GEMM, BFU-E and CFU-C colonies. In these 4 patients, the karyotype of bone marrow or circulating mononuclear cells was identical to that of hemopoietic colonies. These results indicate a clonal origin of MPD at the level of CFU-GEMM.</p>","PeriodicalId":76233,"journal":{"name":"Nihon Ketsueki Gakkai zasshi : journal of Japan Haematological Society","volume":"52 6","pages":"1022-32"},"PeriodicalIF":0.0,"publicationDate":"1989-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13732699","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
H Sawami, K Ito, M Norioka, S Monden, M Fujita, H Uchino
{"title":"Transfer of the adenosine deaminase (ADA) gene of a B-lymphoblastoid cell line (LCL) to an ADA-deficient LCL by a microcell-mediated chromosome transfer technique.","authors":"H Sawami, K Ito, M Norioka, S Monden, M Fujita, H Uchino","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>As a model of somatic cell gene therapy, a normal adenosine deaminase (ADA) gene was introduced into a B-lymphoblastoid cell line (LCL) established from a patient with ADA deficiency by microcell-mediated chromosome transfer (MMCT). A LCL derived from his mother was used as a gene donor. Seven fusion experiments were performed and hybrid cells were pipetted into 123 wells. After selection in the presence of deoxyadenosine, cells grew in 12 wells at Week 9 after fusion. Among these wells, ADA activity of hybrids was low in 4 wells, 130-280% of that of the donor LCL in 7 wells and very high in one well. Hybrid cells in 4 wells with ADA-positive cells were investigated for the time-course of expression of ADA activity. In one well, ADA activity was expressed until Week 36, while, in 3 wells, ADA activity decreased or was lost between 21-36 weeks after fusion. These findings indicate the transfer of chromosome 20 containing a normal ADA gene into recipient cells and the deletion of this chromosome from some part of the hybrid cells. Karyotyping at Week 35 or 37 revealed 47 chromosomes in about 30% of the cells in 2 wells, which suggests that these hybrids were relatively stable in culture.</p>","PeriodicalId":76233,"journal":{"name":"Nihon Ketsueki Gakkai zasshi : journal of Japan Haematological Society","volume":"52 6","pages":"1033-44"},"PeriodicalIF":0.0,"publicationDate":"1989-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13732700","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
S Nishinarita, Y Iizuka, H Kang, N Kubo, M Sakurada, K Kamei, Y Yanagi, M Akahoshi, K Oshimi, S Sawada
{"title":"CD4/Leu7 and CD8/Leu7 large granular lymphocytosis: comparative studies between NK cells and T cells.","authors":"S Nishinarita, Y Iizuka, H Kang, N Kubo, M Sakurada, K Kamei, Y Yanagi, M Akahoshi, K Oshimi, S Sawada","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Lymphocytes, co-expressing CD4/Leu7 and CD8/Leu7 markers respectively, taken from two patients having large granular lymphocytosis taking an indolent clinical course have been comparatively studied for function as NK cells and T cells. Both large granular lymphocytes (LGLs) were acid phosphatase positive and showed a beta-glucuronidase reaction in their cytoplasmic granules. Studies on case 1 indicated that the CD4/Leu7 lymphocytosis with LGL morphology takes a benign clinical course with mild neutropenia as well as those of CD8/Leu7 LG lymphocytosis. Both CD4/Leu7 and CD8/Leu7 LGLs behave similarly in their lack of NK activity, and manifest decreased IL-2 production in vitro and show a low IL-2 receptor expression unrelated to their T cell phenotype, but behave differently in influencing the immunoglobulin production in vitro and the ADCC activity, depending on their T cell phenotype and on the expression of Fc receptor, respectively. Furthermore, the altered Fc receptors which were undetectable by the Leul 1 antibody but were still effective for ADCC activity might be present in case 2 LGLs.</p>","PeriodicalId":76233,"journal":{"name":"Nihon Ketsueki Gakkai zasshi : journal of Japan Haematological Society","volume":"52 6","pages":"1045-57"},"PeriodicalIF":0.0,"publicationDate":"1989-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13658031","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
S Asakura, S Terukina, K Yamazumi, M Matsuda, H Murayama, A Higuchi, M Musashi, K Sakurada, T Miyazaki
{"title":"Fibrinogen Sapporo: dysfibrinogenemia characterized by the replacement of A alpha arginine-16 by histidine resulting in the delayed release of fibrinopeptide A by thrombin.","authors":"S Asakura, S Terukina, K Yamazumi, M Matsuda, H Murayama, A Higuchi, M Musashi, K Sakurada, T Miyazaki","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Congenital dysfibrinogenemia was found in a 60-year-old asymptomatic female and her daughter. Purified fibrinogen derived from the propositus, apparently a heterozygote for the abnormality, characteristically showed delayed but complete release of fibrinopeptide A upon digestion with thrombin but its defective release by Ancrod, a snake venom enzyme, from half of her fibrinogen molecules. This congenital dysfibrinogenemia with an A alpha arginine (Arg) to histidine (His) substitution was tentatively designated as fibrinogen Sapporo. Although this type of abnormal fibrinogen had been identified among Caucasians, no such cases have so far been reported in Japan.</p>","PeriodicalId":76233,"journal":{"name":"Nihon Ketsueki Gakkai zasshi : journal of Japan Haematological Society","volume":"52 6","pages":"1094-104"},"PeriodicalIF":0.0,"publicationDate":"1989-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13730985","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
E Araki, Y Wada, K Ishihara, S Ueda, K Harano, T Harano
{"title":"Hemoglobin Hope found in a patient with basal cell carcinoma of the genial region.","authors":"E Araki, Y Wada, K Ishihara, S Ueda, K Harano, T Harano","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Hemoglobinopathy was detected in a 72-year-old female with basal cell carcinoma of the genial region during high-performance liquid chromatography of hemolysate for the assay of HbA1c. Structural analysis demonstrated that this abnormal hemoglobin was identical to Hb Hope [beta 136(H14)Gly----Asp]. No clinical or hematological abnormality was observed. A study of the family revealed that a younger sister and two sons of the propositus had the same hemoglobin variant. This is the second observation of Hb Hope in a Japanese family.</p>","PeriodicalId":76233,"journal":{"name":"Nihon Ketsueki Gakkai zasshi : journal of Japan Haematological Society","volume":"52 6","pages":"972-6"},"PeriodicalIF":0.0,"publicationDate":"1989-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13730987","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Y Kato, H Sawada, M Tashima, Y Yumoto, T Okuda, T Ueda, M Yamagishi, H Uchino
{"title":"Heterogeneous features of Ph-negative CML--possible existence of Ph-negative, bcr-rearrangement-negative CML.","authors":"Y Kato, H Sawada, M Tashima, Y Yumoto, T Okuda, T Ueda, M Yamagishi, H Uchino","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>We have examined eight patients with Ph-negative chronic myelocytic leukemia (CML). Two of the patients had the same molecular abnormalities as well as the same clinical and hematological features as those of Ph-positive CML. The other six patients showed no genomic rearrangement. This group was hematologically divided into four subgroups, namely chronic myelomonocytic leukemia, undifferentiated chronic myeloproliferative disorder, chronic neutrophilic leukemia and CML-like syndrome. This last subgroup resembled Ph-positive CML in many points except for rather moderate proliferation of granulocyte lineage, and it was difficult to clinically separate it from Ph-positive CML.</p>","PeriodicalId":76233,"journal":{"name":"Nihon Ketsueki Gakkai zasshi : journal of Japan Haematological Society","volume":"52 6","pages":"1004-12"},"PeriodicalIF":0.0,"publicationDate":"1989-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13732697","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A case of immunoblastic lymphadenopathy (IBL)-like T-cell lymphoma with unusual clinical manifestations.","authors":"K Kurihara, H Kono, J Nishihara, Y Chikamori","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>A 67-year-old male consulted an otolaryngologist with a complaint of dysphagia due to a large polypoid mass in the hypopharynx. A biopsy of the mass revealed immunoblastic lymphadenopathy (IBL)-like T-cell lymphoma of helper/inducer subset occurring in lymphoid tissues of the hypopharynx. Although general lymphadenopathy was not seen, further biopsy of an inguinal lymph node showed small foci of lymphoma cells, suggesting the generalized distribution of this malignancy. To our knowledge, this is the first report of this malignancy mimicking primary pharyngeal sarcoma.</p>","PeriodicalId":76233,"journal":{"name":"Nihon Ketsueki Gakkai zasshi : journal of Japan Haematological Society","volume":"52 6","pages":"1058-63"},"PeriodicalIF":0.0,"publicationDate":"1989-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13732701","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
T Nakamaki, T Ajiri, A Sakashita, S Tomoyasu, N Tsuruoka
{"title":"UV-induced DNA repair in leukemic cell differentiation.","authors":"T Nakamaki, T Ajiri, A Sakashita, S Tomoyasu, N Tsuruoka","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Ultraviolet light (UV)-induced DNA repair during myeloid leukemic cell differentiation was examined. Human myeloid leukemic cells could be induced to differentiate in vitro into mature cells by various chemical inducers that lost their proliferating potencies. In spite of decrease of proliferation capacity, almost all these terminally differentiated myeloid leukemic cells invariably showed UV-induced unscheduled DNA synthesis (UDS) at low energy of UV irradiation (3-5J/m2). This indicated that the terminally differentiated myeloid leukemic cells are functionally quite different from mature granulocytes in chronic myeloid leukemia (CML) or in normal peripheral blood. In HL-60 cells, UV-survival was enhanced in the process of differentiation induced by 1.25% DMSO or 0.6 mM sodium n-butyrate. The degree of enhancement of UV-survival was correlated with the increased amount of UDS. The process of myeloid leukemic cell differentiation which is completed without loss of capacity performing repair DNA synthesis was one of the characteristics of the terminally differentiated myeloid leukemic cells induced by chemical inducers in vitro and this function may support the hypothesis that DNA breaking and rejoining are involved in a mechanism of cytodifferentiation.</p>","PeriodicalId":76233,"journal":{"name":"Nihon Ketsueki Gakkai zasshi : journal of Japan Haematological Society","volume":"52 6","pages":"977-87"},"PeriodicalIF":0.0,"publicationDate":"1989-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13730988","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
K Saito, M Yoshida, A Okuyama, N Watanabe, H Enokihara, S Furusawa, H Shishido
{"title":"[Neutrophil-associated IgG and neutrophil-binding IgG in autoimmune neutropenia].","authors":"K Saito, M Yoshida, A Okuyama, N Watanabe, H Enokihara, S Furusawa, H Shishido","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Neutrophil-associated IgG (NAIgG) and neutrophil-binding IgG in sara (NBIgG) of 77 patients with neutropenia suspected to be caused by autoimmune mechanisms (group A) and 31 patients with aplastic anemia or myelodysplastic syndrome (group B) were assayed by flow cytometry. Auto-NBIgG was elevated in 32% of the patients in group A, particularly in about 70% of those with collagen diseases or ITP, but the level was normal in group B. Elevated NAIgG with normal auto-NBIgG levels was found in 27% of the patients in group A and in 64% of the patients in group B. The assay of auto-NBIgG was useful for detection of anti-neutrophil autoantibodies and for the diagnosis of autoimmune neutropenia. In addition, the level of NAIgG may be non-specifically elevated in non-immune neutropenia.</p>","PeriodicalId":76233,"journal":{"name":"Nihon Ketsueki Gakkai zasshi : journal of Japan Haematological Society","volume":"52 5","pages":"842-8"},"PeriodicalIF":0.0,"publicationDate":"1989-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13731424","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"[Effects of interleukin-2 and alpha and gamma-interferon on B cell colony formation in patients with hairy cell leukemia].","authors":"T Ishiyama, S Abe, Y Wakabayashi, S Hirose","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>We studied the effect of interleukin-2, and alpha and gamma-interferon on the growth of B cell colonies in three patients with hairy cell leukemia (HCL). Cells in the colonies from HCL were morphologically similar to the circulating cells, but HCL colonies were morphologically different from B-CLL colonies. HCL colonies had several satellite cells. Spell out (PHA) was essential for the growth of HCL. HCL responded to PHA-MTCM, the supernatant derived from 24 hr incubation of PHA, silica, monocytes and T cells. HCL cells didn't respond to IL-2 or alpha-interferon. However, gamma-interferon significantly enhanced HCL cell proliferation.</p>","PeriodicalId":76233,"journal":{"name":"Nihon Ketsueki Gakkai zasshi : journal of Japan Haematological Society","volume":"52 5","pages":"880-6"},"PeriodicalIF":0.0,"publicationDate":"1989-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"13658030","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}