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Volume 20, Issue 7
Displaying 1-13 of 13 articles from this issue
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Mitsuhiro OMINE, Tadashi MAEKAWA1979 Volume 20 Issue 7 Pages 691-699
Published: 1979
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSA series of comprehensive survey study was conducted during 1974∼1976 under the sponsorship of Hemolytic Anemias Research Committee, the Ministry of Health and Welfare, Japan (Chairman, Professor Shiro Miwa, Yamaguchi University), with the principal purposes of evaluating the current status of the two representative types of hemolytic anemia, i. e. autoimmune hemolytic anemia (AIHA) and hereditary spherocytosis (HS).
Relevant clinical and laboratory data were generously provided for analysis by 160 major institutions and clinics on 141 cases with AIHA and 144 cases with HS. Extensive analysis of these materials yielded sufficiently detailed informations on a variety of clinical aspects of the disease, which are of essential importance for the adequate understanding and effective management of these diseases.
Included were onset-age distribution, heredity and familial occurrence, clinical picture at diagnosis, routine and special hematological examinations, biochemical and serological features, treatment and its effectiveness, side effects, clinical course, and overall prognosis.
Characteristic features were outlined individually for AIHA and HS, while several problems awaiting forthcoming clarification were pointed out, including the particular need for elucidation of the eventual development and long-term prognosis of AIHA and also for the recognition and management of HS during neonatal period.View full abstractDownload PDF (695K) -
Seitoku FUJIOKA1979 Volume 20 Issue 7 Pages 700-708
Published: 1979
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSThe clinical and haematological findings of the 119 Japanese PNH patients were investigated on the basis of the reports from the haematologists in the major hospitals, including ours, in 1976. Epidemiological study was done as well. Prospective study on these patients and haematological investigation of other 10 cases with PNH were performed in 1978. The results revealed that Japanese PNH patients, 73 males and 60 females (M: F 1.2: 1), were most common between the third and fifth decades. The 97 cases with haemoglobinuria and the 11 cases with haemosiderinuria were found among the 119 cases. Ham test and sucrose hemolysis test were mostly carried out to detect the PNH red cells. Laboratory examination revealed anaemia, pancytopenia, reticulocytosis, hypoplasia and increased percentages of erythroblast in bone marrow, decreased red cell life-span, variable levels of serum iron, increased levels of serum indirect bilirubin, haemoglobin and lactic dehydrogenase, and decreased haptoglobin. Decrease of leukocyte alkalinephosphatase score was correlated with frequency of haemoglobinuria. Most patients were treated with adrenal and anabolic steroids, however only limited effects were obtained. No patients showed normal peripheral blood counts so far. The 18 patients died. The commonest causes of death were haemorrhage. Leukemia occurred to the 3 cases, two AML and one erythroleukemia.View full abstractDownload PDF (743K) -
Takaoki MIYAJI1979 Volume 20 Issue 7 Pages 709-717
Published: 1979
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSMore than 38 human hemoglobin variants and 63 families with thalassemia have been reported in Japan.
The present report have summarized following main aspects:
1. The serial consequences on seven unstable variants in relation to molecular abnormality, denaturation pathway of hemoglobin, erythrocyte destruction and clinical and hematological features have been discussed.
2. Also, the efficiency of splenectomy was various among patients with unstable variants. It was suggested that ferrokinetic and red cell survival studies which locate the organs destructing the red cells are important indices for the prediction of the effect of splenectomy.
3. Diagnostic significance on the estimation of globin synthesis in thalassemic patients was confirmed.View full abstractDownload PDF (635K) -
Hisaichi FUJII, Shiro MIWA1979 Volume 20 Issue 7 Pages 718-723
Published: 1979
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSIn Japan, 111 cases of the 11 different enzyme anomalies have so far been discovered. G6PD deficiency and pyruvate kinase deficiency are most common erythroenzymopathies. The clinical manifestation depends on the biochemical characteristics of the variant enzyme. The G6PD variants with low enzyme activity and heat instability result in chronic hemolysis.
Pyrimidine 5'-nucleotidase (P5N) deficiency was studied by partial purification and condensation. The residual P5N was characterized by an increased Km and a markedly acidic shift of the pH optimum. We demonstrated the first direct evidence for genetic heterogeneity in P5N deficiency. Basophilic stippling of the erythrocyte is the hallmark of this enzyme defect.
Recently we discovered a case of increased adenosine deaminase (ADA) activity with hereditary hemolytic anemia. The normal and patient enzymes were purified using antibody affinity chromatography. Both enzymes were very similar. The greatly increased ADA activity seems to be an overproduction of normal enzyme and might be due to the failure of the genetic control mechanism.View full abstractDownload PDF (452K) -
Tokugoro TSUNEMATSU, Choku MATSUHASHI1979 Volume 20 Issue 7 Pages 724-734
Published: 1979
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSIt is widely known that the therapeutic drugs such as α-methyldopa (α-MD), penicillin (PC) and cephalosporin (CE) induce positive Coombs' test and hemolytic anemia. There has been no report of comprehensive study dealing with the drug-induced hemolytic anemias in Japan. The Ministry of Health and Welfare Hemolytic Anemia Research Committee (Chairman, Dr. S. Miwa) made an investigation to find the incidences of positive Coombs' test and hemolytic anemia among patients treated with these drugs. Coombs' test was found positive in 10 out of 168 hypertensive patients on α-MD. The incidence was 6.0% which showed lower than those reported from Europe and U.S.A. Hemolytic manifestations were not found in the above-mentioned 10 patients with positive Coombs' test. In the medical literatures in Japan, 9 cases of α-MD induced hemolytic anemia have been reported up to the present time.
PC and CE induced positive Coombs' test in 11.0 and 15.0%, respectively. The patients were consisted of various diseases complicated with infections and treated with the antibiotics. The figures obtained were rather high probably because the numbers of the patients examined were small. Further investigations are thought necessary to find the accurate incidences.
The authors developed the methods to detect the anti-PC and anti-CE antibodies in patients' sera using passive hemagglutination test and antibody-mediated cellular cytotoxicity test. And clinical applications of these tests were made on the sera from patients who had developed the adverse side-reactions of the drugs during treatment. The results obtained were promising for a further development to detect the anti-drugs antibodies.View full abstractDownload PDF (846K)
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Shigenori SUZUKI1979 Volume 20 Issue 7 Pages 740-744
Published: 1979
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESS1) Hemorrhage in the newborn period, especially intracranial and lung hemorrhages in RDS are highly problematic in the field of obstetrics as the cause of death in the perinatal period. Some of the asphyxiated newborn cases showed difinite signs of hypercoagulability and in this paper some attempts were performed to investigate the relationship between the asphyxia in the newborn and the so-called disseminated intravascular coagulation, especially fibrin monomer complex (S.F.M.C.). In these situations factor XIII and changes of α-chain of fibrinogen were followed and found that the latter exerted serious influence on both blood coagulation and fibrinolytic systems.
2) 54 cases of newborns were divided into 3 groups; namely (i) Apgar≤4 (ii) 5≤Apgar≤7 and (iii) Apgar≥8. S.F.M.C. measurements were made by agarose gel-filtration with cord venous and newborn blood. Respective fractions obtained in changed α, β, γ chains of fibrinogen were measured by electrophoresis and their relationships with the factor XIII was also investigated.
3) In the Apgar≤4 groups the value of SFMC was remarkably elevated to be 5.68±1.94% in comparison with the normal value of 3.17±0.55%, and it showed reversed coreelation (γ=-0.73) against the Apgar score. Thus, hypercoagulability in asphyxiated newborns, was clearly proved from quatitative measurements. In PAA electrophoresis patterns of DICplasma, a characteristic decrease of α-chain of fibrinogen was observed as well as factor XIII. One of them, γ-γ dimer was also proved.View full abstractDownload PDF (356K) -
2nd Report —especially changes of coagulofibrinolytic system before and after UK infusion—Senji FUJITA, Tadaharu SHOU, Hidemitsu YAMAMOTO, Hisashi ISHIKAWA, Tak ...1979 Volume 20 Issue 7 Pages 745-753
Published: 1979
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSThe effects of urokinase (UK) on blood coagulation and fibrinolytic activities in 15 cases with cerebral infarction were evaluated.
Each case was administrated with 120,000 or 240,000 international units of UK over two or six hours by means of dripping infusion and this therapy was performed for two to seven days.
In this study, especially the changes of coagulofibrinolytic system, before and after UK infusion were examined and the following results were obtained.
1) The hyperfibrinolytic effects such as shortened euglobulin clot lysis time, decrease of plasminogen and antiplasmin were observed.
2) The increased level of FDP was detected in 83.3% of 24 materials and the increase was more than 4 times before UK infusion in 55.5% of them.
3) The definite changes of prothrombin time, partial thromboplastin time and fibrinogen level were not observed at the end of UK infusion.
4) A trend of decrease of serum antithrombin III was shown in 45.8% of 24 materials. However, the decrease of α1-antitrypsin and α2-macroglobulin was not observed in most of them.
5) It was necessary for detection of the effects of UK on coagulofibrinolytic system to perform serial asssay of these factors after UK infusion.
6) The bleeding complications induced by UK therapy were not observed. Clinical improvement was excellent in 5 cases, good in 2 cases and slight in 6 cases, and no change was observed in 1 case. In one case with transient ischemic attack, its clinical symptoms had already disappeared before UK therapy.
7) The cases with excellent or good clinical course exhibited a remarkably increased level of FDP in the early stage of UK therapy and absence of the findings suggesting hypercoagulability.
These results show that it is important to administrate UK in enough dose to induce an increased level of FDP in the early stage and to prevent the hypercoagulability by anticoagulant therapy when UK therapy is performed in the cases with cerebral infarctions.View full abstractDownload PDF (654K)
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Microscopic Observations of the Liver and KidneyShinichi OHNO, Masaaki NAGASAKI, Kenichi FURUKAWA, Takeshi SAHARA, Ken ...1979 Volume 20 Issue 7 Pages 754-761
Published: 1979
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSA fifty-nine-year-old man had been well until July 1976, when he complained of hoarseness, back pains and general fatigue. He was found to have hypercalcemia, anemia and accelerated erythrocyte sedimentation rate. A bone marrow aspirate revealed 63% myeloma cells. He was diagnosed as myeloma. The serum protein showed two spikes of β-globulin and fast γ-globulin by cellulose acetate electrophoresis. By immunoelectrophoresis, the former spike was found to correspond to light chain λ and the latter to IgD. The concentration of IgD in serum was 1850mg/dl, but other immunoglobulin levels were markedly depressed. He was given cyclophosphamide and prednisolone with temporary response, but died of renal failure in March 1977 and was autopsied.
The electron microscopic observations of the liver and the kidney were performed. The renal tubules had an extraordinary thickening of the basement membrane in which there were lots of round deposits, 0.5 to 1.0μm in diameter, lower in electron density than the basement membrane. Another kind of deposits was also observed, which were 0.1 to 1.0μm in diameter, higher in electron density than the basement membrane and harder than glass. The component of the former was undetermined, but that of the latter was crystalline calcium phosphate.
The studies on the liver revealed a deposition of amorphous substance with high electron density along endothelial cells in the space of Disse. These deposits showed the histochemical properties different from amyloid substance, and appeared to be a mixture of filaments and fibrils showing the characteristic cross banding pattern.
Furhter investigations will be necessary to assess whether these findings are characteristic of IgD myeloma or not.View full abstractDownload PDF (967K) -
Koichi NEMOTO, Tadayuki NAKANISHI, Masamitsu Hiramatsu, Ikunosuke SAKU ...1979 Volume 20 Issue 7 Pages 762-767
Published: 1979
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSAbnormal immunoglobulin molecules have been demonstrated in a patient (CU) with typical multiple myeloma. The serum and the urine contained two monoclonal proteins (IgG-kappa type), one having a sedimentation rate of 7S and other 4.8S. 4.8S IgG myeloma protein (MW 73,000 daltons) were composed of one gamma chain of about 49,000 daltons and one light chain of 22,000 daltones. Enzymatic degradation suggested that gamma chain of the CU half-molecules has a deletion in its Fc portion or hinge region. IgG half-molecules appear to be a very rare type of monoclonal immunoglobulins. Such immunoglobulins have been found in only three other patients in the world, and this patient was the first case in Japan.View full abstractDownload PDF (586K) -
Hideo KIMURA, Tsutomu SHICHISHIMA, Tetsugoro TANAKA, Shin MATSUDA, Tsu ...1979 Volume 20 Issue 7 Pages 768-776
Published: 1979
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSA 48 year-old woman was admitted to the Fukushima Medical College Hospital on June, 1976, because of the swelling of cervical lymphnode. Biopsy finding of cervical lymphnode revealed infiltration of plasma cells and detailed examination confirmed extramedullary plasmacytoma in supra-pharyngeal region, associated with IgA-κ type monoclonal gammopathy.
By the treatment with prednisolone and cyclophosphamide, plasmacytoma and lymphnode got reduced in size, and she was discharged on Nov. 1976. After about 1 year she was admitted again. On examination, supra-pharyngeal plasmacytoma was enlarged and various symptoms such as polyneuropathy, lymphnode swelling, pigmentation of skin, edema, clubbed finger, hepatosplenomegaly, moderate fevering and pleural effusion (chylothorax) were observed.
Laboratory examination showed RBC 365×104/mm3, Hb 7.2g/dl., WBC 4,400/mm3, platelet 39.6×104/mm3, serum protein 6.7 g/dl (β+γ: 52.3%, IgA-k type M protein, IgA 2,640 mg/dl.). Plasma cells were not increased in bone marrow smear.
Anti-tumor agents were not so effective, but plasmacytoma got reduced in size and the various symptoms were getting better or disappeared by irradiation to suprapharyngeal region.
This case was thought to correspond to syndrome of plasma cell dyscrasia with polyneuropathy and endocrine disturbance, first advocated by K. Takatsuki et al.View full abstractDownload PDF (944K) -
Isao AOKI, Keisuke TOYAMA, Tetsuya TSUCHIDA, Hiroshi TSUTSUMI, Yoshihi ...1979 Volume 20 Issue 7 Pages 777-785
Published: 1979
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSThe case reports of chronic neutrophilic leukemia have still been uncommon.
A 74-year-old male was admitted on September 13, 1976, with the chief complaint of general malaise. On admission, his hemoglobin level was 7.5 gm/100ml, the platelet count 58,000/mm3 and the leukocyte count 166,500/mm3. Ninety-one percent of leukocytes were mature polymorphonuclear cells with Döhle bodies and no eosinophil or basophil was found.
Bone marrow smear revealed a hypercellular marrow with hyperplasia of myeloid elements. The leukocyte alkaline phosphatase score was 282 and serum vitamin B12 was 47,850 pg/ml.
A karyotypic analysis showed a 47 xy+c pattern with no evidence of the Ph1 chromosome.
He died of brain stem hemorrhage on October 15, 1976. Postmortem examination revealed a markedly hypercellular marrow with predominantly polymorphonuclear neutrophils, and marked infiltration of mature neutrophils in the liver, spleen, kindneys, lungs, stomach and many other organs.
These findings may propose the diagnosis of chronic neutrophilic leukemia to this case.View full abstractDownload PDF (1161K) -
Noriyoshi UEHARA, Hiroyoshi SAWADA, Masaro TASHIMA, Morihisa YAMAGISHI ...1979 Volume 20 Issue 7 Pages 786-793
Published: 1979
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSAn autopsy case of 74-year-old male with primary myelofibrosis terminating in leukemic transformation with multiple skin nodules was reported. His clinical course was about 1 year after the diagnosis was made.
Autopsy showed prominent fibrosis of the bone marrow and multiple foci of myeloid metaplasia in various tissues where hematopoiesis was normally demonstrable in the embryonic period.
Histologically, many extramedullary hematopoietic tissues including skin and subcutaneous tumors were diffusely infiltrated with myeloblasts associated with atypical megakaryocytes. From above pathohistological findings, it was suggested that myeloid metaplasia in this case was essentially neoplastic rather than reactive or compensatory.View full abstractDownload PDF (656K) -
Hiroshi YOSHINAKA, Shoshi MATSUDA, Teruo YANAGIHARA, Isao NAKAMURA1979 Volume 20 Issue 7 Pages 794-799
Published: 1979
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSA 62-year-old male was admitted to the Yamaguchi Prefectural Central Hospital on March 14, 1977, because of anemia.
Three months before admission he had noted the gradual onset of weakness and fatigue, and two months later he began to complaint of dyspnea, palpitation and leg edema.
On admission physical examination did not show remarkable change other than those related to anemia such as marked pallor of the skin and cardiac murmur.
Peripheral blood examination showed RBC 223×104/mm3, hemoglobin 8.0 g/dl, platelet 10.2×104/mm3 and WBC 5,300/mm3 with 6% atypical cells. Sternal marrow examination reveals hyperplastic and increased of 9.8% megakaryoblast.
A diagnosis of Megakaryoblastic Leukemia was made and administration of 6MP and then prednisolone was started. The response was poor and the patient died after about 5 months from admission.
This case and others in literature were discussed.View full abstractDownload PDF (482K)
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