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Volume 27, Issue 5
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Nobuo NARA1986 Volume 27 Issue 5 Pages 651-658
Published: 1986
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSLeukemic blast progenitors from acute leukemia patients are characterized as stem cells; they proliferate for differentiation or undergo self-renewal. Two ways are now available for the studies of leukemic blast progenitors in vitro. One is methylcellulose culture method, and the other is liquid suspension culture system. Leukemic blast progenitors make colonies in the former method, which is important as a clonogenic assay. Leukemic balst progenitors show exponential growth in the latter method. The exponential growth is based on the self-renewal of the progenitors. Thus, suspension culture is useful as a method not only maintaining leukemic blast progenitors for a long-term but also studying self-renewal events. In these methods, the growth patterns and chemosensitivity of blast progenitors are studied. An approach to developing more effective treatment strategies for acute leukemia may be expected from these studies.View full abstractDownload PDF (759K)
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Koichi ARAKI, Osamu SHINZATO, Hiroyuki TAJIMA, Goro MIMURA1986 Volume 27 Issue 5 Pages 659-663
Published: 1986
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSA seroepidemiological survey of antibodies to the adult T-cell leukemia virus (ATLV or HTLV-I)-associated antigen was conducted by passive particle-agglutination method among 2,087 of healthy adults (721 males and 1,366 females) who lived in the mid-Eastern section of mainland Okinawa and in the three islands of Henza, Miyagi and lkei.
1) The overall prevalence rate was 36.9% with the rate in the three islands being 41.8%. However, no significant difference was found between males and females (36.3% for males and 37.2% for females).
2) Thirty-three married couples in Henza lsland were classified into four combinations to study er the transmission of HTLV-I between husband and wife. The proportion of (+) wife with (+) husband was 75.0%, and that of (-) wife with (-) husband 82.4%.
3) Children (0 to 15 years old) in Henza lsland had a high prevalence rate of 5.2%.
4) The prevalence rate of Okinawans (898 healthy adults) who emigrated from Okinawa to Hawaii was 17.9% for Issei, 19.7% for Nisei and 11.9% for Sansei, which were almost the same as those of healthy people of the same age in Okinawa. The transmission of the HTLV-I might occur from generation to generation.
5) Forty-seven patients with chronic renal failure who have been hemodialyzed and blood transfused, showed a higher prevalence rate of 51.1% than that of 55 patients without blood transfusion.
The data suggested that the transmission of HTLV-I might occur:
1) between husband to wife, probably via semen.
2) from mother to child probably via mother's milk.
3) with blood transfusion.View full abstractDownload PDF (414K) -
Hirokuni TAGUCHI1986 Volume 27 Issue 5 Pages 664-669
Published: 1986
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESS1. Forty two patients of adult T-cell leukemia and pre-adult T-cell leukemia have been seen in Kochi Prefecture.
2. Two thirds of these cases were from Hata-district and Muroto-district, western and eastern end of Kochi Prefecture.
3. Distribution of human T-cell leukemia virus (HTLV) antibody positive blood donors was surveyed throughout in Kochi prefecture. HTLV-I positive persons also distributed in Hata and Muroto districts heavily (over 5%) and rarely seen in the central and north part of Kochi prefecture. These results suggest that HTLV-I was first introduced to Hata and Muroto districts separately (probably in almost same time) and has been confined to these relatively narrow districts due to inconvenience of traffic facilities until recently.
4. It is speculated that incidence of ATL is one per 1,500∼3,000 healthy carriers (total number 18,000 in Kochi prefecture) every year.
5. A 36 year old female developed pneumocystis carinii pneumonia.
As depressed rate of OKT4/OKT8 was shown, aquired immunodeficiency syndrome was suspected. Antibody to HTLV-III was negative and HTL-I antibody positivity was demonstrated. She developed overt ATL one year and a half after the pneumocystis carinii pneumonia and died after 9 months' unsuccessful therapy of ATL. Possibility of suppressed immune system by the infection of HTLV-I was discussed.View full abstractDownload PDF (655K) -
—HTLV-I Has Also an Etiologic Role in the Diseases Other than ATL—Kazunari YAMAGUCHI, Takashi KUMAGAI, Norio ASOU, Sung Yul LEE, Tetsuyu ...1986 Volume 27 Issue 5 Pages 670-676
Published: 1986
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSVariation in the clinical features of atypical ATL suggested a division of the spectrum of ATL into five types: acute, chronic, smoldering, crisis and lymphoma. In all cases the serum was positive for anti HTLV-I (ATLA) antibodies and the monoclonal integration of HTLV-I proviral DNA in the malignant cells was confirmed
Smoldering ATL has been found frequently in patients with skin mycosis (fungus infection of the skin), slight lymphadenopathy in which biopsy chronic lymphadenitis, abnormal lung X-ray shadows resembling interstitial pneumonitis, chronic renal failure, and strongyloidiasis.
Smoldering ATL is considered to be associated with an immune deficiency state to some extend. We have also noticed that the association of monoclonal gammopathy and other neoplasms is significantly more frequent.View full abstractDownload PDF (542K) -
Shuichi IKEDA, Kenji NISHINO, Sabro MOMITA, Tatsuhiko AMAGASAKI, Shime ...1986 Volume 27 Issue 5 Pages 677-685
Published: 1986
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSForthy two cases of pre-ATL (preleukemic state of adult T cell leukemia) with ATL cell-like abnormal lymphocyte in 10% to 40% of peripheral blood leukocytes were studied for clinical course, physical findings, laboratory data, chromosomal abnormalities and integration of HTLV proviral DNA.
The clinical courses were classified into three patterns; first, persistent abnormal T lymphocytosis (14 cases), second, declining of abnormal T lymphocytosis (11 cases) and third, advancement to ATL (5 cases). None of the cases showed signs and symptoms whitch are characteristic of ATL, but skin rash was observed in a few cases.
In almost all cases tested, monoclonal integration of HTLV proviral DNA was detected in the peripheral lymphocytes.
Chromosomal abnormalities were found in 9 cases in this series. Six cases had abnormal clones. Three cases showed clonal change to new ones when examined later. In 5 cases abnormalities of chromosome No. 14 at band 14q11 was identified.
Thus, in pre-ATL, abnormal clones are already established. However, they still lack potential to show malignant proliferation full enough to reveal clinical signs and symptoms.View full abstractDownload PDF (721K) -
Hiroyuki SATO, Kazuo OKOCHI1986 Volume 27 Issue 5 Pages 686-692
Published: 1986
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSIn a retrospective follow-up study of recipients of blood transfusion, antibodies against adult T-cell leukemia associated antigen (anti-ATLA) became detectable in those who had received anti-ATLA positive blood units containing cell components. By using monoclonal antibody against p19, a internal core protein of HTLV-I, ATLA bearing T-cell clones were isolated from 6 recipients who had produced anti-ATLA antibodies after blood transfusion. These clones were confirmed to be HTLV-I infected cells by testing proviral DNA of HTLV-I integrated into cellular DNA. These findings suggest that blood transfusion can be a route of transmission of HTLV-I. Furthermore, a followup study that the transmission was avoided by screening of donors' blood by testing anti-ATLA antibody, was documented.View full abstractDownload PDF (536K) -
Tadashi MATSUMOTO, Makoto MATSUMOTO, Hiroshi KIKUCHI, Kazuo YUNOKI1986 Volume 27 Issue 5 Pages 693-700
Published: 1986
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSSurface markers on lymphoid tumor cells were studied in 58 patients with ATL and 55 with PTCL. A high positive rate (91%) of anti-ATLV/HTLV-I test in PTCL suggested that most patients with PTCL in this study were strongly associated with ATL. Surface antigens (T3, T4, T6, T8, T9, T10, and Ia) were detected by indirect immunofluorescence methods using a panel of OK-monoclonal antibody series. The results were summarized as follows.
1. ATL and PTCL were subdivided into 4 categories by T4 and/or T8 expressions; T4+, T8+, T4•T8+, and T4•T8- groups. Surface phenotype in PTCL was considerably heterogenous and 31, 7, 8, and 9 patients were distributed among these subgroups, respectively. Almost all of ATL patients showed T4+ surface phenotype, except for two with T4•T8- and one with T4•T8+ on node samples.
2. T3 expression, which is specific to almost all of the normal T cells, was observed only a part of patients with ATL (35/58) and PTCL (19/51). More interestingly, no patients with T3 were observed in the T4•T8- phenotypic subgroup.
3. A majority of PTCL patients with T9 or Ia belonged to the histologic subgroups of large cell type and pleomorphic type. On the other hand, T10 expression was observed mainly in patients with medium-sized cell type and ATL. In patients tested for these three markers simultaneously, 10/33 in ATL and 13/20 in PTCL were positive to one of these markers at least, but only 3 and 2 cases showed double and triple markers, respectively.
4. Some shifts of surface markers were observed between different sites of organs and different stages of the disease. The shift to gain of loose T4 or T8 was of paticular interest about the phenotypic diversity of T4 and/or T8 expressions in ATL and PTCL.View full abstractDownload PDF (574K) -
Kazuo TAMURA1986 Volume 27 Issue 5 Pages 701-707
Published: 1986
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSAdult T-cell leukemia (ATL) is a extremely difficult disease to treat. It is also known to have a wide range of clinical presentation, course and prognosis. It appears that there was a group of patients who presented a large number of leukemic cells (≥30% of leukemic cells in ≥20,000/μl of leukocyte count) and/or hypercalcemia and were expired from complications after diagnosis (high risk group), while the patients with a low percentage of leukemic cells and absence of hypercalcemia lived longer regardless of modalities of treatment (low risk group).
In a high risk group, fourteen patients were treated with a combination chemotherapy including VEMP or VEPA regimen, and the median survival was only 2 months with a response rate of 36%. In contrast, total body irradiation with a total dose of 100 to 150 rad administered over 2 to 3 weeks was given to 9 patients with 100% response rate including 2 complete responses and the median survival of over 6 months.
Ten patients fell into a low risk group. Five patients lived over 8 months with various treatment modalities ranging from no treatment to radiation therapy or mild chemotherapy. Lymphocytapheresis and extracorporeal irradiation of the blood were tried on one of the latter patients who developed crisis from the low risk group. The rapidly rising leukemic cells became stabilized during these treatments. Since both treatments do not seem to give direct untoward effects against the hosts, they might be the treatment of choice in selected patients in conjunction with other modalities of treatment.
ATL is a disease of leukemia with immune deficiency and, indeed, most of the patients died of overwhelming infections. Since antineoplastic agents or radiation therapy have an inherent problem with immunosuppression, interferon may be considered as an ideal agent which possesses antitumor and antiviral effects as well as a property of immunostimulation. So far, eleven patients have been treated with recombinant β- or γ-interferon and partial or complete response was noted in 5 patients, although the duration of response was usually shortlived.
Apparently multiinstitutional cooperative studies are needed to treat this ominous disease.View full abstractDownload PDF (508K)
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Noriko OHBA, Takeshi ISHIZAKI, Kazuko HASHIZUME, Hiroshi HORITA, Hisak ...1986 Volume 27 Issue 5 Pages 710-716
Published: 1986
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSA crossed immunoelectrophoretic method with a first dimension gel containing heparin (16.6 u/ml) was applied to investigate various forms of plasma antithrombin III (ATIII) in typical cases of disseminated intravascular coagulation (DIC) with low ATIII levels, with high or normo ATIII levels, patients treated with L-asparginase and bone marrow transplanted patients which developed to DIC.
Compared with the normal fractional pattern of ATIII (ATIII1=S1=82.6±2.2%, ATIII2=S2=9.6±1.3, ATIII3=S3=7.8±1.6), relatively increased levels of S3 (S1=81.1±6.5%, S2=8.5±1.9, S3=10.4±5.0) were observed in typical DIC with decreased levels of ATIII. In high-or normo-ATIII group increased levels of S1 fraction (S1=84.8±4.2%, S2=8.2±2.0, S3=7.0±2.2) with partial uniting S1 with S2 were seen, while in L-asparginase trated cases with low ATIII levels, did relatively high peak of S1 area with lower levels of S2 and S3 (S1=90.9±2.5%, S2=6.8±1.1, S3=6.7±1.2) with spiked peak formation of S2 in other one and increased S3 in last one.
From these results, it was concluded that the increment of S3 was diagnostic clue to DIC, and that in high-or normo-ATIII patients with DIC or L-asparginase treated patients the ATIII fraction with high-affinity to heparin (S1) relatively increased and that in DIC developed after the bone marrow transplantation, the impairement of ATIII production may cause the pattern of the ATIII fraction.
Although crossed immunoelectrophoretic methods are time-consuming in practice, it seemed to be useful to know dynamic biological profile of ATIII in various hemostatical conditions.View full abstractDownload PDF (502K) -
Isamu SUGIURA, Tomohiko OGASAWARA, Tomohiro KINOSHITA, Yoshinori ITO, ...1986 Volume 27 Issue 5 Pages 717-722
Published: 1986
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSFifty-two patients with non-Hodgkin's Lymphomas presenting to our hospital from July 1977 to May 1984 were reviewed to evaluate the New Staging Classification. Twenty-two patients (44.2%) were alive on May 1 in 1985, with a median follow-up of 49 months. Median survival of all cases was 20.5 months. According to the Ann Arbor staging classification, the percent of stage I, II, III and IV were 21.2%, 30.8%, 19.2% and 28.8%, respectively. Median survival of early cases (stage I+II) was 24 months, and that of advanced cases (stage III+IV) was 12 months. There was no difference in survival curves of these two groups (p=0.41). Whereas, according to the New Staging Classification, the percent of stage I, II, III and IV were 21.2%, 25.0%, 17.3% and 36.5%, respectively. Median survival of early cases was 34 months and that of advanced cases was 11 months, and the difference of survival curves turned to be significant (p=0.03). We recommend to use the New Staging Classification for non-Hodgkin's lymphomas, in which correlation between stage and prognosis is clearer than in Ann Arbor staging classification.View full abstractDownload PDF (440K) -
—Studies in Normal Volunteers and Thromboembolic Patients—Yutaka OGUMA, Hiroshi HASEGAWA, Hiroshi NAGATA, Yoshikazu KAWAKAMI1986 Volume 27 Issue 5 Pages 723-729
Published: 1986
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSWe investigated the relations among heparin (Hp)•antithrombin III (ATIII) complex (HAC), plasma Hp concentration and APTT in 15 nomal volunteers and 12 thromboembolic patients. The activities of HAC and the concentrations of plasma Hp were assayed by Hp monitor test (HMT) and by Testozym S-2222 method (TST), respectively. The ratio of HMT/TST exhibited the relative amounts of HAC in plasma. HMT and TST had equally good correlations to APTT and had a good (p<0.01) correlation to each other in all cases. But in each case, they showed quite different regression lines. HAC synthesis was related not only to the levels of Hp and ATIII but also to many other factors which fluctuated significantly according to the indivisual case. Recovery from the thromboembolic conditions was seen in cases whose levels of TST and HMT revealed more than 0.4 and 0.1U/ml, respectively and it was demonstrated that subcutaneous administration of Hp with the dose of 250U/kg at every 12 hours was quite useful to obtain these therapeutic levels.View full abstractDownload PDF (482K) -
Koichi SHINMYOZU, Yoshikazu MARUYAMA, Mitsuhiro OSAME, Akihiro IGATA, ...1986 Volume 27 Issue 5 Pages 730-737
Published: 1986
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSIn order to detect other blood coagulation/fibrinolytic defects with congenital plasminogen abnormality, the coagulation studies were examined in 83 cases with congenital plasminogen abnormality (2 homozygotes and 81 heterozygotes).
Unusual decrease of plasma fibrinogen level under mean-2SD of normals was found out in 13 cases, which was considered the heterozygous state (hypofibrinogenemia) of congenital afibrinogenemia because functional assay for fibrinogen concentration yielded essentially the same values as did immunologic assay. Unusual decrease of plasma von Willebrand factor level under mean-2SD of normals was detected in 20 cases. Based on the crossed immunoelectrophoresis of von Willebrand factor, 19 of these 20 cases were diagnosed as type I (classical) von Willebrand disease, while the remaining one as type II (variant) von Willebrand disease. Combined abnormalities of hypofibrinogenemia and von Willebrand disease were found out in 5 cases. Moreover, 3 of these 5 cases with combined abnormalities had the increased erythrocyte membrane fragility resulting in hemolysis.
From these results it is postulated that 4 abnormalities, i.e. plasminogen abnormality, hypofibrinogenemia, von Willebrand disease and increased erythrocyte membrane fragility may be highly-combined diseases or states with each other- a new hereditary syndrome?View full abstractDownload PDF (621K) -
Masahito TSURUSAWA, Naoyuki KATANO, Yoshifumi YAMAMOTO, Susumu KAWAI, ...1986 Volume 27 Issue 5 Pages 738-746
Published: 1986
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSExpression of common ALL antigen (CALLA) on the DNA stemlines of leukemic marrow blasts in 21 children with common acute lymphoblastic leukemia (ALL) was quantitatively determined by flow cytometric analysis of J-5 monoclonal antibody binding. Mean amounts of CALLA per cell widely ranged among the patients. The cells at S phase showed higher mean amounts of CALLA than the cells at G0/G1 phase, whereas the density of CALLA per unit of cell surface area in the cells at S phase was lower than in the cells at G0/G1 phase. Subpopulations of CALLA-positive leukemic cells and CALLA-negative normal marrow cells at a given DNA stemline were easily discriminated from the findings of two and/or three dimentional diagrams of CALLA-DNA. No significant relationships of various patient's features (sex, age, DNA ploidy and surface markers) to CALLA amounts were observed. Children who remained disease-free had significantly higher amounts of CALLA for G0/G1 phase cells than children who relapsed.
These findings showed that CALLA amounts for leukemic blasts might be valuable to predict treatment response of children with common ALL.View full abstractDownload PDF (618K)
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Kouya HONDA, Tomoyoshi KAMIYA, Hideo OOKAWA, Yasuyoshi KIRYU, Yukito Y ...1986 Volume 27 Issue 5 Pages 747-751
Published: 1986
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSA 46-year-old man was referred to Nippon Kokan hospital in July 1981 for dizziness. Physical examination showed severe anemia but no splenomegaly or lymphadenopathly. Blood examinations on admission revealed WBC 1,600/μl, RBC 133×104/μl, Hb 4.0 g/dl, and Plts 2.6×104/μl. Bone marrow biopsy showed marked fibrosis and atypical megakaryocyts. He remained well until March 1982, when he was readmitted because of progressive anemia with 19% of blasts in the peripheral blood. These blasts were identified in megakaryoblast as they were positive for electron microscopic platelet peroxidase. These findings were compatible with acute megakaryoblastic leukemia (acute myelofibrosis). The paient died of pneumonia in June 1982. Appearance of monoclonal gammopathy (IgG λ) and a few plasma cells in peripharal blood in the terminal stage, and marked nodular proliferation of plasma cells including some atypical form of in the spleen and bone marrow at autopsy suggest neoplastic change of plasma cells. It is of interest that co-existence of acute megakaryoblastic leukemia (acute myelofibrosis) and plasma cell malignancy may be due to neoplastic change in the stage of pluripotent hemopoietic stem cells.View full abstractDownload PDF (631K) -
Koichi SHINMYOZU, Yoshikazu MARUYAMA, Ikuro MARUYAMA, Mitsuhiro OSAME, ...1986 Volume 27 Issue 5 Pages 752-757
Published: 1986
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSA new type of congenital platelet function disorder was demonstrated in a 34-yr-old woman with a life-long history of bleeding such as easy bruising, epistaxis and menorrhagia. Bleeding time was markedly prolonged. Platelet count, morphology, clot retraction, adhesiveness to collagen, adenine nucleotides and platelet factor 3 availability were all normal. Platelet function abnormalities were characterized by absent second wave aggregation in response to ADP or epinephrine, markedly reduced aggregation in response to collagen and defective ADP release, representing a defect of platelet release mechanism. To elucidate the pathogenetic mechanism, further functional studies were performed. No appreciable arachidonate-induced aggregation was observed, whereas thromboxane A2 (TXA2) formation appeared to be normal and normal ionophore A23187-induced aggregation was observed. Finally the patient's platelets failed to aggregate in response to TXA2 formed in normal platelet-rich plasma induced by arachidonate.
These results indicate that the defect of platelet release mechanism observed in this patient is probably due to an impaired platelet response to TXA2.View full abstractDownload PDF (416K) -
—A Study of Soluble Factor—Kazuaki ISHIBASHI, Shuuichi HANADA, Shuuji HASHIMOTO1986 Volume 27 Issue 5 Pages 758-763
Published: 1986
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSIn spite of some clinical reports of T cell malignant lymphoma (T-ML) accompanied by M proteinemia, the meaning of a M component in the lymphocytic proliferation remains uncertain. In this paper, a case of T-ML, which tumor cells carry OKT8 antigen, associated with IgM-λ M proteinemia is reported. And the activity of B cell differention factor (BCDF) is examined in the supernatant of T lymphoma cells cultured for short term (T-F).
Peripheral B cells obtained from the patient and normal human control were cultured each with the various % of T-F in vitro. 72 hours later, IgM-λ production was induced in both B cells dose dependently. Furthermore, the patient's bone marrow lymphocytes were subjected to cytoplasmic (c-) Ig stainning. Consequently, c-μ and c-λ positive cells were observed more frequently than other Ig classes.
It is assumed that T-F derived from T-ML cells act on the patient's B cells in vivo to induce preferential IgM-λ production in this case.View full abstractDownload PDF (1072K) -
Atuko WADA, Kiyoshi KITANO, Hiroshi SAITO, Seiichi FURUTA, Tatutoshi N ...1986 Volume 27 Issue 5 Pages 764-769
Published: 1986
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSA case of acute leukemia with proliferation of myeloblasts and megakaryocytic lineages of various stages in maturity is reported.
A 31-years-old male was admitted to Suwa Red Cross Hospital because of general malaise. On admission he was moderately anemic and had a splenomegaly. Peripheral blood examination showed hemoglobin 7.8 g/dl, platelet 68×104/cmm and WBC 14.6×103/cmm, with differential count of 83% blast cells. Platelet adhesiveness and aggregation were impaired. Bone marrow aspirations showed proliferation of megakaryocytes (1,494/cmm) and 68% blast cells. Bone marrow biopsy revealed no fibrosis and chromosomal analysis showed normal karyotype on 3 occasions.
Electronmicroscopic histochemical studies on bone marrow aspirations revealed an increased number of platelet peroxidase positive megakaryoblasts, megakaryocytes of various stages in maturity, and myeloperoxidase positive myeloblasts.
Cell cultures using bone marrow cells and circulating cells yielded substantial number of both CFU-GM and CFU-MK.
These findings led to the diagnosis of myelomegakaryocytic leukemia, which was considered as a result of leukemic transformation of pluripotent stem cells.View full abstractDownload PDF (587K) -
Yasuyuki ENDO, Shigeo MAMIYA, Hidetaka NIITSU, Keiko IWAMOTO, Sumiko H ...1986 Volume 27 Issue 5 Pages 770-774
Published: 1986
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSA 7-year old man with a history of bleeding tendency showed the prolongation of bleeding time and the mild decrease of platelet adhesiveness. The platelet of patient, however, had a normal aggregation induced by ADP, collagen, epinephrine, arachidonic acid, bovine fibrinogen, ristocetin, A-23187 and thrombin. Furthermone, electro microcopical morphology of these platelets was normal. Therefore, we could not let this disorder belong to each known platelet dysfunction, for example the abnormalities of glycoprotein, storage pool, release reaction and so on.
On the other hand, it is noted that an increase in clot retraction, a reversal of ATP/ADP and an elevation of plasma levels of released substances from platelets, such as β-thromboglobulin, platelet factor 4 and metabolic products of thromboxane were observed in both of the patient and his mother. This case might be regarded as very rare case, because of association of hypoand hyperfunction of platelets and might show the pattern of sex-linked inheritance.View full abstractDownload PDF (413K) -
Kosaburo SHICHIJO, Kohji FUJISAWA, Sanpei MIYAGAWA, Junichi AKATSUKA1986 Volume 27 Issue 5 Pages 775-779
Published: 1986
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSA 12-year-old boy was admitted to the hospital because of frequent relapsing nephrotic syndrome. He had been treated with predonisolone. When total dose of predonisolone was 10,500 mg, petechiae and ecchymosis appeared on truncus, then gradually spread out extremities with oozing from the intrvenously injected site. Serum ascorbic acid level revealed under 0.3 mg/dl (normal level: 0.7∼1.5 mg/dl). He had been treated with a lot of ascorbic acid, then purpura remarkably disappeared.
Based on the decrease level of asconbic acid in the serum, clinical course and thrombocytopenia, the diagnosis of steroid scurvy was made.View full abstractDownload PDF (544K) -
Masao ISHII, Kazunari YAMAGUCHI, Lee Sung Yul, Tetsuya ODA, Kiyoshi TA ...1986 Volume 27 Issue 5 Pages 780-783
Published: 1986
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSA patient with adult T-cell leukemia (ATL) was successfully treated with adenosine deaminase (ADA) inhibitor, 2'-deoxycoformycin (DCF) when he was refractory to conventional chemotherapy.
A 42-year-old man was admitted in February, 1981 because of pleural effusion. The conventional chemotherapeutic agents were effective, and he was discharged. He was readmitted with pleural effusion in August 1984. Cell surface marker studies showed the OKT4, OKT8 and OKT11 positive cells. Serum ATLA antibody and HTLV-I proviral DNA were positive. The diagnosis of ATL was made and VEPA therapy was effective.
In June 1985, he was readmitted because of ascites and leucocytosis. Ascites was improved by conventional chemotherapy but the number of ATL cells in peripheral blood and marrow did not decrease by two chemotherapy regimens. Thus, 7.5 mg/day of DCF was given by intravenous drip infusion. The number of ATL cells decreased prominently and he was discharged. During the therapy, no cytopenias were observed. DCF seemed to impair selectively the proliferation of ATL cells. The side effects were nausea, vomiting and slight liver dysfunction but these were acceptable.
DCF could be applied to a case of ATL which is refractory to the conventional treatment.View full abstractDownload PDF (288K) -
—Observation of the Large Membrane Complexes and a Hypothesis About the Basophile Granules Formation—Akira YOSHIOKA, Hiroaki NONAKA, Shuichi IKEDA, Naoki SADAMORI, Kenichi ...1986 Volume 27 Issue 5 Pages 784-791
Published: 1986
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSA case of acute basophilic leukemia is reported. Patient is a 36-year-old female, whose peripheral blood WBC count and percentage of the leukemic cells were 148,000/cmm and 96%, respectively. The size of the leukemic cells was 10 to 12μm. They appeared to have naked nuclei, and didn't have any granules. They had transparency in the central portion of their nuclei. Cytochemistry revealed PO+, PAS-, acid phosphatase+, naphol ASD chloroacetate esterase+, toluidine blue metachromasia+. Chromosomal analysis revealed normal karyotype. Tests for cell surface marker revealed HLA DR- and My 7+. Three types of granules could be distinguished electron microscopically, namely, flocculent form, particulate form and dense body. In the cytoplasma, membrane complex of labyrinthine form was observed, too. This structure was thought to be a kind of smooth endoplasmic reticulums, and has not been reported previously. It was observed that membrane complex merged with rough endoplasmic reticulums, and produced immature granules. PO activities were detected in many granules but not in the membrane complex, Golgi apparatus, rough endoplasmic reticulum or perinuclear cisternae. This process of granule formation that basophile granules originate from the membrane complex is different from the usual theory that granules originate from the Golgi complex.View full abstractDownload PDF (1273K) -
Hiroyuki OISHI, Teppei KABEMURA, Hiroshi IDEGUCHI, Junji NISHIMURA, Hi ...1986 Volume 27 Issue 5 Pages 792-797
Published: 1986
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSA 51-year-old man with angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) terminating in peripheral T cell lymphoma with cytotoxic/suppressor phenotype was presented. He was admitted to a hospital because of fever and urticaria in May 1980. He was pointed out swollen tonsils, generalized lymphadenopathy, hepatosplenomegaly, eosinophilia, hypergammaglobulinemia and positive of direct Coombs' test. AILD was diagnosed by biopsy of inguinal lymph node. He was treated by prednisolone, then fever, lymphadenopathy and hepatosplenomegaly disappeared. In February 1983, he was admitted to our hospital because of fever, generalized lymphadenopathy, hepatosplenomegaly and eosinophilia. Malignant lymphoma (diffuse, medium cell type) was diagnosed by inguinal lymph node biopsy. Surface markers of lymphoma cells were OKT-8 (+), OKT-11 (+), Tac (+) and OKT-4 (-). He was treated by prednisolone, vincristine, adriamycin, cyclophosphamide, L-asparaginase and etc, but died of generalized aspergillosis in August 1983.
We reviewed the cases with malignant lymphoma arising from AILD or immunoblastic lymphadenopathy (IBL) and discussed the mechanism of malignant evolution.View full abstractDownload PDF (659K) -
Tetsuji KOYAMA, Hirohiko IKEGAWA, Mitsuhiro HIGUCHI, Tokuo NAKAJIMA, E ...1986 Volume 27 Issue 5 Pages 798-803
Published: 1986
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSThis is a case of chronic relapsing TTP seen in a postpartum 26-year-old woman after transient remission obtained by plasma exchange alone. Thrombocytopenia could not be prevented by oral administration of antiplatelet agents (aspirin, ticlopidine and trapidil), dextran sulfate or danazol. Therefore, she required transfusion of either 400 ml of fresh frozen plasma (FFP) or a cryosupernatant fraction every three or four weeks to prevent TTP relapses for 14 months. Instead of transfusion of FFP, we tried transfusion of albumin fraction or filtrated plasma with a holofiber 1760 column (Asahi medical corp.), but failed to prevent thrombocytopenia.
We speculate that efficient factors of plasma to prevent thrombocytopenia in TTP relapses might exist in a high molecular fraction of over 150,000 daltons of plasma.View full abstractDownload PDF (431K)
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1986 Volume 27 Issue 5 Pages 804-827
Published: 1986
Released on J-STAGE: February 02, 2009
JOURNAL RESTRICTED ACCESSDownload PDF (2173K)
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