Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
Volume 29, Issue 5
Displaying 1-26 of 26 articles from this issue
  • Shojiro IKEMATSU, Hisashi YORIFUJI, Morio ARAI, Katsuyuki FUKUTAKE
    1988 Volume 29 Issue 5 Pages 628-634
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    Commercial clotting factor concentrates for the replacement therapy of hemophiliacs are prepared from plasma pooled from large numbers of donors. These concentrates contain a variety of proteins with only a small amout of the desired component. One major consequence of this pooling procedure is the considerable chance of viral contamination, even where such viral prevalence in the checked donor population is extremely low. Attempts to inactivate viruses contained in plasma have extended to physical and chemical procedures. It is the purpose of this report to the various virus-inactivating procedures used in currently available concentrates, and to evaluate the eliminating process which may give an overloading stress on the immune system of patients with hemophilia.
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  • [in Japanese], [in Japanese]
    1988 Volume 29 Issue 5 Pages 635-639
    Published: 1988
    Released on J-STAGE: February 02, 2009
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  • Atsuo IIZUKA
    1988 Volume 29 Issue 5 Pages 640-648
    Published: 1988
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    We report our experience of induction of immune tolerance by factor VIII or IX in low or intermediate dose carried out on 8 haemophiliacs with inhibitors (A; 5, B; 3) in 2 low-responders and 6 high-responders. All of the 2 low-responders and 3 of the 6 high-responders with previous maximal inhibitor levels between 45.6 and 624 Bethesda Units showed a decrease of inhibitor level to below 1.0 Bethesda Units and normal VIII C or IX C recovery in vivo.
    In one of the other 3 patients, the inhibitors level has dropped to 5.0∼10.0 Bethesda Units with no anamnestic response. In the other 2 patients their inhibitor levels have been above 30 Bethesda Units, although they haven't showed typical anamnestic responses.
    In the results of the previous reports including our report, 27 out of 41 haemophiliacs with inhibitors, who have been treated with low or intermediate dose regimens, showed a decrease of inhibitor level to below 1.0 Bethesda Units and normal VIII C or IX C recovery. In comparison with the high-dose long-term F VIII regimen of Brackmann (The Bonn Regimen) which the high cost make it impractical at most haemophilia centers, about the same results was obtained by low or intermediate dose regimens. We recommend that the low or intermediate dose regimen should be indicated for haemophiliacs with inhibitors who have suffered from bleeding episodes, in both aspects of the costs and patients' life.
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  • Akira YOSHIOKA
    1988 Volume 29 Issue 5 Pages 649-654
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    In order to avoid the spread of AIDS among hemophiliacs and to reduce the dependance of the plasma source on the foreign countries, a new factor VIII concentrate with intermediate purity and potency (RCG-5) was developed using fresh plasma from healthy volunteer donors after several screening tests.
    RCG-5 was prepared from a small pool of cryoprecipitate (2∼10 donors) and subsequent defibrination with heat treatment at 54°C for 5 min in the presence of glycine and NaCl. In spite of the heat treatment, about 90% of the factor VIII procoagulant activity (F. VIII: C) and ristocetin cofacter activity (RCof) could be retained, whereas more than 90% of the fibrinogen was removed from the cryoprecipitate. None of new antigenicity was developed by heat treatment. During the defibrination process, some marker viruses such as Sindbis and VSV as well as HIV and HBV could be inactivated and/or removed.
    RCG-5 was found to contain 19.5 u/ml of F. VIII: C on the average, 28.0 u/ml of F. VIII antigen (F. VIII: Ag), 29.0 u/ml of RCof, 30.7 u/ml of von Willebrand factor antigen (vWF: Ag) and 10.4 mg/ml (0.5 mg/unit of F. VIII: C) of fibrinogen. The ratio of F. VIII: Ag to F. VIII: C and vWF: Ag to RCof in RCG-5 was 1.4 and 1.1, respectively. They were very close to cryoprecipitate. These suggest that the inactivation of biological activities of F. VIII and vWF little occurred during production procedure. RCG-5 has a remarkable correcting effect on the defective ristocetin-induced platelet aggregation in the patient with von Willebrand disease (vWD). It also showed large, intermediate and small multimers of vWF just similar to normal plasma or cryoprecipitate.
    From these results RCG-5 is expected to be a new useful preparation for the patients with hemophilia A as well as vWD.
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  • Yutaka OGUMA, Kimihiro SHIMIZU, Nobuo SAKURAGAWA
    1988 Volume 29 Issue 5 Pages 655-661
    Published: 1988
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    This study was intended to improve the stability of F. VIII and IX and to examine the effectiveness of oral administration using these preparations. Two modification methods were used; F. VIII and IX were entrapped in liposome and coated by the intestinally digestive capsules (lipo-VIII and IX) and F. VIII and IX were modified with polythyleneglycole (PEG-VIII and IX). The following results were obtained: 1) 40% of the native F. VIII and IX were entrapped in liposome and aprotinin was necessary to protect the degradation of lipo-VIII and IX. 2) F. VIII: C increased varyingly from 2% to 23% when lipo-VIII 1,000 or 1,200 units was administered orally to the patients with hemophilia A and vWD. 3) After the administration of lipo-IX to dogs, rapid adsorption of F. IX, II and X were observed. 4) PEG-VIII was stable against plasmin and activated protein C, but it showed almost the same change against thrombin compared with native F. VIII. 5) F. VIII and IX increased significantly after the administration of PEG-VIII and IX. These results suggest that oral administration of F. VIII and IX is hopeful if F. VIII and IX are purified precisely and exact mechanism of adsorption and security of liposome and PEG are established.
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  • Kenji OKUYAMA, Yoshiko FUKUSHIMA, Yasuhiro MIURA, Reiko HONMA, Sei-ich ...
    1988 Volume 29 Issue 5 Pages 662-665
    Published: 1988
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    Functions and physicochemical properties of human plasma proteins prepared by filtration through the microporus regenerated cellulose membrane (BMM) hollow fibers were tests.
    Factor-VIII, Factor-IX, antithrombin-III, Fibrinogen and complement activities were not decreased after passed through these membranes having their mean pore size larger than 20 nanometer.
    Physicochemical properties were also maintaned.
    ATLV, HIV, HBV and non-A, non-B hepatitis viruses may be eliminated from human plasma using these membranes.
    This filtration methods are quite useful to get a virus free plasma or blood products.
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  • Tadashi KAMIYA
    1988 Volume 29 Issue 5 Pages 666-670
    Published: 1988
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    Lyophilized, heat-treated, factor VIII and IX concentrates are the predominantly prescribed treatment for bleeding in hemophiliacs. Two major drawbacks currently identified with pooled-plasma concentrates are the risk of viral transmission upon infusion and immunosuppression thought to be due to alloantigen stimulation. Two new AHF manufacturing processes have been recently developed that may eliminate these problems. One process uses monoclonal antibody to purity AHF from plasma; the other uses gene recombination techniques to prepare rDNA AHF. The first method includes purification of F VIII or F VIII/vWF from the cryoprecipitate by immunoaffinity chromatography using mouse monoclonal antibodies raised against F VIII or F VIII/vWF. The specific activity of the resulting F VIII concentrate (Method M, MONOCLATE®) is high (1,500∼3,000 u/mg protein) because it is virtually free of éxtraneous proteins and completely free of alloantigen. rDNA preparations are being developed by several firms. The one developed by Genetics Institute is still in the experimental stage, but phase I studies begun with two patients showed good hemostatic efficiency without side effects. Encouraged by this success, it is expected that the production of rDNA AHF will move from the laboratory to full-scale manufacturing.
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  • Hiroyuki NAGASAWA, Tomio KONDOU, Kanji YASUDA, Yoshihisa YAMAZAKI, Tak ...
    1988 Volume 29 Issue 5 Pages 671-675
    Published: 1988
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    Corticosteroid has been widely given to patients of chronic idiopathic thrombocytopenic purpura (ITP), but its effect has not been thought to be always beneficial. Furthermore methylprednisolone pulse, immunosuppressive agents or high dose gammaglobulin treatment has not always brought a satisfactory result.
    Then, we tried a combination therapy of glucocorticoid and high dose gammaglobulin to three non-splenectomised patients. Two of them were resistant to glucocorticoid and high dose gammaglobulin therapy alone and the third showed only a transient response to each thrapy. In the former two, the prominent increase in platelet count were seen and in the latter, the suitable effect lasted longer than glucocorticoid and high dosegammaglobulin therapy alone.
    Therefore, this combination therapy might be recommended to chronic ITP patients who are resistant to glucocorticoid or high dose gammaglobulin therapy respectively or show only transient effect to each one.
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  • Seiji KOJIMA, Minoru FUKUDA, Keizo HORIBE, Kohji MATSUYAMA, Kohichi MI ...
    1988 Volume 29 Issue 5 Pages 676-681
    Published: 1988
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    Ten children with aplastic anemia (9 severe; 1 moderate) were treated with antilymphocyte globulin (ALG; Ahlbulin, Green Cross Co., Osaka, Japan). Four of them received the combination of ALG and high dose methylprednisolone (HDMP). Seven children were administered with danazol concurrently for at least 3 months. A complete hematological response was noted in 4 children. Three died, two of whom died of infection and the other died of intracranial bleeding. Seven out of the ten children are still alived and the duration vary from 3 to 48 months following the start of the ALG therapy. Complications of the ALG therapy were few. This data indicated that ALG is effective in improving hematopoiesis in some children with aplastic anemia.
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  • Tadami SAKURAI, Kyoko KANEDA, Osamu FUKUHARA, Kuniaki MEGURO, Akira MI ...
    1988 Volume 29 Issue 5 Pages 682-687
    Published: 1988
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    An in vitro method for erythropoietin (Epo) bioassay was investigated, in which 3H-thymidine incorporation into spleen cells from phenylhydrazine treated mice was determined.
    When test samples were assayed in the culture system, the assayable level ranged from 10 to 1,000 mU/ml. Dose-response curves for plasma were parallel to the Epo standard curve. Using this method, we determined plasma Epo titiers in 12 normal subjects, 12 patients with polycythemia vera and 13 patients with aplastic anemia. The Epo titers assayed by this method were well correlated with those of radioimmunoassay.
    Since this method is sensitive, rapid and simple, it may be useful for Epo bioassay.
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  • Hiroshi IMURA, Naoya ITO, Shyoichi SHIRAKAWA, Ryo SOBUE, Fumio MARUYAM ...
    1988 Volume 29 Issue 5 Pages 688-693
    Published: 1988
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    (2''R)-4'-O-tetrahydropyranyladriamycin (THP) is a new anthracycline with effect equal or superior to that of adriamycin, and with less toxicities in experimental animals. Clinical phase I and II studies confirmed its effect on non-Hodgkin's lymphomas (NHL); cardiotoxicity was minial. We incorporated THP in one of the two regimens for an alternating, presumably non cross-resistant chemotherapy for NHL, which consisted of THP 40 mg/m2 iv D1, cyclophosphamide 500 mg/m2 iv D1, vincristine 1 mg/m2 iv D1 & 8, prednisolone 60 mg/m2 po D1-5 (THP-COP). The other regimen consisted of N4-behenoyl ara-C 100 mg/m2 iv D1-5, VP-16 60 mg/m2 iv D1-5, methotrexate 200 mg/m2 iv D8 & 15, and prednisolone 40 mg/m2 po D1-5 (BHAC-VMP). Three courses of THP-COP were administered first, followed by three courses of BHAC-VMP. After complete remission (CR) these two regimens were administered alternately for a total of 12 courses or until relapse. Twenty patients have been entered and all were evaluable: M/F, 12/8; mediun age 57.5 yr (22∼79); clinical stage II 3, III 8, IV 9; histology, follicular medium cell sized 3, and diffuse 17 (small cell 1, medium cell sized 6, mixed cell 2, large cell 7, lymphoblastic 1).
    Sixteen patients achieved CR, among whom 8 only after 1 course of THP-COP. The median remission duration and survival were 7.5 (1∼30) months and 11.5 (3∼31) months, respectively. All 11 patients with stage II and III, and 5 out of 9 stage IV patients achieved CR. Side effects included myelosuppression, mild gastrointestinal symptoms, neurotoxicity, hair loss and others which were all tolerable; no cardiotoxicity was observed within total dose of THP up to 530 mg/m2. THP-COP was quite effective as a CR-inducer in NHL. A longer observation, however, is necessary for evaluation of effectiveness of the alternating non-cross resistant chemotherapy as a whole on survival.
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  • Masayuki SOMA, Yoshihisa MAEDA, Masahiro MURAKAMI, Shinpei KASAKURA, H ...
    1988 Volume 29 Issue 5 Pages 694-698
    Published: 1988
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    This study was to investigate exercise-induced changes of plasma plasminogen activator activity. The plasma levels of t-PA antigen, u-PA antigen and PA activity were measured before exercise and immediately after, 15min, 30min, and 60min after exercise on the treadmill.
    The levels of t-PA antigen and PA activity rose immediately after exercise and then gradually decreased. They returned to the control levels 60 min later. In contrast, the level of u-PA antigen was not affected by exercise. In old-aged individuals, the degree of elevation of t-PA antigen and PA activity after exercise was less as compared with that seen in the young age group.
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  • Kunihiro TSUKASAKI, Shu-ichi IKEDA, Saburo MOMITA, Kazuhiro NAGAI, Ken ...
    1988 Volume 29 Issue 5 Pages 699-704
    Published: 1988
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    Twelve patients with non-Hogikin's lymphoma (NHL) unresponsive to the first line combination chemotherapy were treated with combination therapy with cis-dichlorodiammineplatinum (CDDP). Ten patients were evaluable for response. There were four complete and five partial responses. The durations of complete responses were 2-12 months (mean 6 months). Toxicity included nausea and vomiting in 100% of patients, myelosuppression in 75% and renal impairment in 67%, but all were reversible. This regimen appears useful as a salvage therapy for patients refractory to standard regimens.
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  • Yoriko AOKI, Kohji FUJISAWA, Mayumi IKEGAMI, Naoko ISHITOYA, Jun-ichi ...
    1988 Volume 29 Issue 5 Pages 705-710
    Published: 1988
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    A 5-year-old girl, diagnosed to have idiopathic thrombocytopenic purpura (ITP), was admitted to our hospital, because a cyclic change of platelet counts was noticed. Her platelet counts fluctuated cyclically from 18×103l to 205×103l in 13∼15 days. Examination of the bone marrow revealed icncrease in immature form of megakaryocytes. Immunofluorescence technique detected platelet-associated IgG (PAIgG) in thrombocytopenic phase. After a high-dose intravenous immunoglobulin therapy, the platelet count and PAIgG level returned to normal for about 3 months. However the periodicity of platelet count recurred thereafter.
    These observations suggested that the mechanism of thrombocytopenia in this patients would be chiefly ascribable to the same type of immune-mediated platelet destruction as ITP.
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  • Eiji SHIMIZU, Masato TSUJIMOTO, Hirokazu NAKAMINE, Kiichiro OHTA, Toru ...
    1988 Volume 29 Issue 5 Pages 711-717
    Published: 1988
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    Acquired immunodeficiency syndrome (AIDS) in a 37-year-old Japanese man is reported. The patient was admitted on February 17, 1986, because of generalized weakness, fever, cough and dyspnea which were lasted for 10 days. He had been in San Francisco for 15 years untill May, 1984. He was not a drug abuser but a homosexual with frequent sexual contacts in San Francisco.
    General examination revealed obvious recent weight loss and moderate bilateral lymphadenopathy on the cervical, axillary and inguinal regions. A chest X-ray film showed diffuse infiltration on both lung fields without pleural effusion. Blood chemistry and electrolyte levels were normal expect the elevated level of LDH, hyponatremia and hyperkalemia in the terminal stage. The leukocyte count was 9,500/μl with 78% granulocytes (9% band forms), 4% monocytes and 18% lymphocytes. The ratio of CD4 to CD8 cells was 0.06. The levels of IgA and IgE were increased. He had no response to skin test with purified protein derivative. Mitogenic responses to PHA, ConA and PWM were reduced. Antibody to HIV (HTLV-III (ELISA), LAV (IF)) was detected in the serum, which was confirmed by Western blot analysis.
    From these findings, he was diagnosed as having AIDS. The patient was treated with antibiotics including sulfamethxazole-trimethoprim and flucytosin. However, his condition continued to deteriorate and died of pneumonia on March 7. Autopsy revealed pneumocystis carinii pneumonia, cytomegalovirus adrenalitis with necrotizing adrenalitis and lymphoid atrophy. There was no evidence of Kaposi's sarcoma.
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  • Koiti INOKUCHI, Kenji TAJIKA, Toshiharu ITO, Kiyoyuki OGATA, Takashi Y ...
    1988 Volume 29 Issue 5 Pages 718-722
    Published: 1988
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    Two cases of drug-induced MDS with monosomy 7 were reported. The first case was a 68-year-old woman, who had received alkylating agents such as melphalan and cyclophosphamide after hysterectomy for uterine cancer. Five years after hysterectomy, she complained of palpitation, and pancytopenia developed. The bone marrow was normocellular with 11.6% myeloblasts and decreased megakaryocytes and erythroblasts. The patient was diagnosed to have refractory anemia with excess of blasts (RAEB). Chromosomal analysis of the bone marrow cells revealed monosomy 7. She is alive eight months after diagnosis. The second case was a 60-year-old male. He had received chemotherapy with melphalan, vincristine and prednisolone for five and a half years against multiple myeloma. Six years after the start of chemotherapy, he had pyrexia and anemia. The bone marrow was normocellular with 11.2% myeloblasts and increased megakaryocytes. The patient was diagnosed to have RAEB. Chromosomal analysis of the bone marrow cells revealed four types of abnormal karyotypes each with monosomy 7. He died of severe anemia four months after diagnosis of RAEB.
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  • Yoshinori SHIMAMOTO, Kazutoshi ONO, Miwako MATSUZAKI, Masayuki SANO, Y ...
    1988 Volume 29 Issue 5 Pages 723-727
    Published: 1988
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    Five cases of adult T-cell leukemia (ATL) associated with cancers were reported. The first case was the acute type of ATL complicated with thyroid and tracheal cancers. The second was the lymphoma type of ATL complicated with gastric and colonic cancers. The third, fourth or fifth case was the lymphoma type of ATL complicated with gastric, hepatic or gastric cancer, respectively. It is interesting that two out of five cases were associated with two kinds of other cancers. In our clinic, double cancer had been found in five of forty three patients with ATL (11.6%) during five years and nine months since the opening of October, 1981.
    The incidence of ATL patients with other malignancies seemed to be higher than that of other hematological disorders. The case reports of ATL associated with cancer from the literature were reviewed and discussed. It was suggested that ATL was concerned deeply in the development of other malignancies.
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  • Motofumi HIYOSHI, Hitoshi SAGAWA, Yasuo YOKOMATSU, Takuya KISHIDA, Kaz ...
    1988 Volume 29 Issue 5 Pages 728-732
    Published: 1988
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    A 19-year-old male admitted our hospital because of dyspnea on exertion and palpitation. He was well until October, 1986, when he noticed a mass in the right-nasal cavity and was first admitted for further evaluation including biopsy, which revealed non-Hodgikin's lymphoma, diffuse mixed type, and a diagnosis of stage IAE was made. He received local irradiation therapy on the nasal region and achieved complete remission. Then he discharged and has followed up.
    In April, 1987, he developed dyspnea on exertion and palpitation and was hospitalized again. On admission pericardial and pleural effusion were found on chest x-ray film and ultrasound cardiography. Malignant lymphoma cells were demonstrated in the aspirated pericardial and pleural effusion. He was treated with MCOP protocol, with some relief. But his general condition gradully got worse again, and the pleural and pericardial effusion also increased. This time he received the chemotherapy consisting of vincristine, predonisolone, vindesin and ifosfamide. And mytomysin C and predonisolone were injected into his percardial cavity. Despite of such intensive cares his condition rapidly deteriorated, and he was pronounced dead.
    Cardiac infiltration of malignant lymphoma varies from 17 to 27 per cent in several autopsy series. But the clinical diagnosis of cardic involvement is rarely made. In this case report, we present a rare malignant lymphoma case having pericardial infiltration demonstrated during clinical course.
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  • Masao KOBAYASHI, Takashi SATOH, Yoshinori KAWAGUCHI, Yoshito TANAKA, K ...
    1988 Volume 29 Issue 5 Pages 733-737
    Published: 1988
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    A nontransfused 10-month-old female infant was admitted for the evaluation of persistent neutropenia, with absolute neutrophil counts ranging from 0 to 150/μl. Antibodies against neutrophils were investigated by indirect immunofluorescence and microleukocyte agglutination. The patient's sera reacted with neutrophils from her mother and normal donors having the netrophil antigen NA1, but not with neutrophils from NA1-negative donors. The results of lymphocyte cytotoxicity test were negative. After the absorption of the patient's sera with NA1-positive neutrophils, the antibody activity was completely abolished. No antibody against NA1-positive neutrophils was found in her mother's serum.
    These results indicate that her neutropenia is caused by autoantibody against neutrophil specific antigen NA1.
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  • Kaoru TOHYAMA, Seiichi OHMORI, Takeo UEDA, Yasunori UEDA, Hiroto SAKOD ...
    1988 Volume 29 Issue 5 Pages 738-742
    Published: 1988
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    A-71-year old man was admitted to Kitano Hospital in December 1985 because of palpitation, shortness of breath and leukocytosis. The peripheral blood showed RBC 173×104l, Hb 5.4g/dl, PLT 23×103l, and WBC 49,600/μl with neutrophilia. The bone marrow smear and biopsy showed a myeloid hyperplasia, and NAP score was consistently high. The serum levels of LDH, uric acid, and ferritin were elevated, and that of vitamin B12 markedly high. Both serum and urine lysozyme levels were significantly high. The spleen was moderately enlarged, but no apparent signs of infection or other malignancy were found out. The chromosome analysis of bone marrow cells showed an abnormal karyotype of 7q-, and Ph1 chromosome was not found.
    Clonogenic culture study revealed the formation of endogenous CFU-GM colonies. In addition, CFU-GM colonies formed in the standard culture condition failed to respond to PGE2 or neutrophils.
    Although administration of dibromomannitol was partially effective, he died of subdural and gastrointestinal bleeding. A postmortem histology of the liver showed the infiltration of granulocytes of each maturation stage into both the portal area and liver sinusoids.
    This case appeared unique in the meaning of both cytogenetic and clonogenic culture studies.
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  • Yuji KISHIMOTO, Toshihiro TAMAI, Shigetoshi OHGA, Takahiro NAGANO, Hir ...
    1988 Volume 29 Issue 5 Pages 743-747
    Published: 1988
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    Thirty two year-old woman showed severe anemia, mild leukopenia with 2.5% blasts, and normal platelet count. A bone marrow aspirate revealed a normo-cellular marrow with 22% blasts and 26.8% erythroblasts, and dyshematopoiesis involving three cell lines was observed. Some of the blasts were positive for platelet peroxidase stain, and numerous micromegakaryocytes were seen in both bone marrow and peripheral blood. However, platelet functions were normal. Cytogenetic analysis showed -7, +der (1) t(1;7)(p11;p11), t(3;3)(q21;q26). She was diagnosed as de novo myelodysplastic syndrome (RAEB-T) and treated with low-dose cytosine arabinoside. Without hematological improvement, she progressed to overt leukemia. At the time of overt leukemia, the blasts were negative for peroxidase stain, and chromosomal abnormalities were evolved to -7, t(1;7;8)(p31.2;q36;p21), t(3;3)(q21;q26.2).
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  • Mitsunobu MATSUBARA, Gohta MASUDA, Hideo TERADA, Haruo NIHKURA
    1988 Volume 29 Issue 5 Pages 748-755
    Published: 1988
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    A case of AIDS which presented unusual abnormalities in both clinical manifestations and laboratory examinations is reported.
    The patient is a 30-year-old bisexual American male born in New York City. After three years of persistent lymphadenopathy, he developed gouty attacks, fever, and giant splenomegaly, and was admitted to St. Luke's International Hospital in Tokyo on August 23, 1985. White cell count was 2,300/μl with lymphocyte 722/μl, hemoglobin 7.9g/dl, and platelet 36,000/μl. Uric acid was 10.4mg/dl. M-protein of IgG-K type was found in immunoelectrophoresis. Hepatic and renal function tests were within normal limits.
    Bone marrow examination revealed total cell count 112,000/μl, with atypical plasma cells, striking hemophagocytosis of histiocytes, an increase of platelet non-producing megakaryocytes, and slight granulocytosis containing toxic granules.
    CMV titer rose slightly to x200 (normal range<x100), and VCA-IgG and EBNA showed high titer. Candida albicans was cultured from throat culture and oral cavity.
    OKT4/OKT8 ratio decreased to 0.23 and HTLV III-antigen was positive.
    During the patient's hospitalization, cervical lymphonodes rapidly enlarged with marked pancytopenia, and hepato-renal disorder appeared. He was transferred to Tokyo Metropolitan Komagame Hospital on September 13th, and travelled back to America on September 24th. Three days later, he died probably due to septic shock during hemodialysis.
    Splenomegaly is often reported in cases of AIDS associated with CMV and EBV infections, but giant splenomegaly is not common. However, in this case, neither CMV infection nor EBV infection were definite. Giant splenomegaly and increased histiocytes with marked phagocytosis in bone marrow coupled with severe pancytopenia and hyperuricemia, were thought to be caused by strong reaction to viral infections.
    Besides T-cell abnormalities, various dysfunctions of B-cell system are reported. But the monoclonal gammopathy with increased atypical plasma cells in this case was though to be new types of B-cell abnormalities in AIDS.
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  • Takayuki TAKUBO, Toshiyuki IKEMOTO, Tsuneaki KIKUCHI, Shinya INAI
    1988 Volume 29 Issue 5 Pages 756-759
    Published: 1988
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    The Technicon H6000 Hematology System simultaneously determines blood cell and leukocyte differential counts automatically based on the method of cytochemistry and cell size measurements. A part of the information provided using this instrument is the histogram of peroxidase staining intensity in leukocytes compared with cell size of the leukocyte called “The Leukocyte Peroxidase Distribution Picture”.
    Approximately 340,000 blood sample-days representing the hematological examinations at the Central Clinical Laboratory, Osaka Medical College Hospital, were examined for the individual leukocyte peroxidase distribution pictures of these samples for the 42 month period from January 1984 to June 1987. From the results of this examination, five cases with lowered eosinophil peroxidase activity were found.
    It is anticipated that further cases with lowered eosinophil peroxidase activity will be found as the Technicon H6000 Hematology System is more widely used in routine examinations, in comparison with the other leukocyte differential methods.
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  • Hisao FUJIMOTO, Kazuo OKADA, Hiroko OHIZUMI, Satoshi HASHINO, Masanori ...
    1988 Volume 29 Issue 5 Pages 760-764
    Published: 1988
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    We report here a case of refractory anemia (RA) associated with various antoantibodies and pure red cell aplasia (PRCA)-like syndrome. A 73 year-old man, who had suffered from RA since 1979, was admitted to our hospital in April, 1986, because of severe anemia. On admission, he had a remarkably elevated level of serum γ-globlin, a decreased level of serum haptoglobin, a high titer of antinuclear antibody, positive direct and indirect Coombs' tests, and a decreased number of erythroblasts in the bone marrow. A life span of red blood cells (51Cr T1/2) was shortened in May, 1986. Serum showed cytotoxicity against allogeneic lymphocytes. These abnormalities were not observed at the onset in 1979. Five months after the admission, he was administered with prednisolone (40mg/day) from September 1, 1986. Anemia and erythroid hypoplasia were gradually improved following this therapy. BFU-E and CFU-C were hardly detectable on admission. Although CFU-E of non-adherent cell fraction in November, 1986, was not yet observed, CFU-E of non-adherent and non-T cell fraction was 23/2×105 cells. Decreased OKT 4 positive lymphocytes and increased OKT8 positive lymphocytes in peripheral blood became to normal level after administration of steroid hormone. It was suggested that erythroid hypoplasia was closely related to the inhibition by suppressor cells against erythroid precursor cells, causing the disturbance of maturation and proliferation of these cells, and the involvement of hemolysis might be unlikely as the cause of anemia.
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  • Takahisa YAMANE, Yoshio FURUKAWA, Takeshi INOUE, Hitoshi SAGAWA, Yasuo ...
    1988 Volume 29 Issue 5 Pages 765-768
    Published: 1988
    Released on J-STAGE: February 02, 2009
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    A 64-year-old female, who had been diagnosed as RAEB in T one year ago, admitted to our hospital on February 10, 1987, because of general fatigue. One month later, blast count increased gradually. The bone marrow aspirate was hypercellular and about 36.5% blasts showed peroxydase positive. Flow cytometric examination revealed that the blasts possessed My7 and My9 surface marker. Hence, her status was considered to show conversion of RAEB in T to myelocytic leukemia.
    She received BHAC-DMP therapy from March 16 to 19. Leukemic cells in the peripheral blood and bone marrow were rapidly decreased in number and a nadir was achieved 7 days later. Then, vitamin D (2.5μg/day) and vitamin A (120,000U/day) were administered per os. One month later, WBC count reached 8,900/μl and myelocyte appeared 28% in peripheral blood. In bone marrow aspirate, myelocyte count was 55%. Elevation of serum calcium level and stomatitis were observed as its side effects.
    Our findings suggested that oral administration of vitamin D and vitamin A, combined with the common induction therapy, might be worth trying in the case of overt leukemia subsequent to MDS.
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  • Osaaki ANDO, Yoshimine SAKAKIBARA, Kuniaki SASAKI, Susumu KAWAI, Takeo ...
    1988 Volume 29 Issue 5 Pages 769-775
    Published: 1988
    Released on J-STAGE: February 02, 2009
    JOURNAL RESTRICTED ACCESS
    A case of severe aplastic anemia assosiated with cyclic thrombocytopenia who has been obtained complete remission by intravenous high dose gammaglobulin therapy was reported. This ten-month-old girl with tentative diagnosis of pure red cell aplasia was referred to our university hospital. Laboratory examination revealed hypocellular marrow, negative Coombs' test and normal serum immunoglobulin levels. Inspite of daily prednisolone therapy, anemia was not improved, but transient reticulocytosis was observed during an administration of gammaglobulin (120mg/kg) for complications of interstitial pneumonia. High dose gammaglobulin therapy (400mg/kg×5days) resulted a complete relief of anemia and cyclic thrombocytopenia and she has remained in a state of complete remission for more than a year.
    Mechanism of the response was uncertain, high dose gammaglobulin should be considered one of the valuable treatments for aplastic anemia.
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