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Volume 19, Issue 12
Displaying 1-15 of 15 articles from this issue
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[in Japanese], Susumu INOUE1978 Volume 19 Issue 12 Pages 1601-1613
Published: 1978
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSDownload PDF (1004K)
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Isao KOBAYASHI, Hoju TAKAHASHI, Kaoru TAKAHASHI, Masakazu ITO, Nobuo S ...1978 Volume 19 Issue 12 Pages 1614-1621
Published: 1978
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSThe replacement therapy of AHG on hemorrhagic episode in hemophilia A patients have faced several troublesome complications, i.e., appearance of inhibitor, hepatitis and allergic reaction.
After obtaining that 1-Deamino-8-D-Arginine Vasopressin (DDAVP) increased factor VIII activity in normal subjects, the usefulness of this drug to these patients was evaluated.
Intravenous infusion of 10μg DDAVP to seven normal subjects and to four mild hemophilia A patients were monitored serially by coagulation and fibrinolysis studies. In normal subjects, factor VIII activity increased from 2 to 5 times, factor VIII antigen from 1.6 to 2.7 times. In four mild hemophiliacs, factor VIII activity increased from 2.2 to 3.3 times. Because the plasminogen activator increments were observed in both normal and hemophiliacs, antiplasmic agent to block the fibrinolytic activity was accompanied to the DDAVP infusion (0.4 g/Kg, every 12 hours) in clinical practice. For gastrectomy, AHG was used in addition to the DDAVP to supply the factor VIII level needed for major operation.
For tooth-extraction, only DDAVP was used. In both cases, the hemostatic effect of this DDAVP was prominent and satisfactory.
On the 5th operation day, however, hyponatremia was observed and DDAVP was withdrawn.
DDAVP appeared to be a very useful tool in controlling hemostasis in mild hemophiliacs in hyponatremia was carefully watched.View full abstractDownload PDF (553K) -
Yasuhiro YODA, Shoji OHBA, Kenjiro TANOUE, Tsukasa ABE, Hiromi SARASHI ...1978 Volume 19 Issue 12 Pages 1622-1628
Published: 1978
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSThree patients who, developed perianal complications in the course of acute leukemia, are described. The first case presented a successful cure of huge perianal ulcer. It was considered that an early improvement of neutrophil count and an adequate anal hygene by using a toilet stool equipped with a washer and by a sitz bath with 0.02% chlorhexidine digluconate had contributed to this cure of the ulcer. The second case demonstrated a rapid progression of perianal infection during a period of severe neutropenia below 100/cmm resulting in a prompt death of the patient. The delayed improvement of neutrophils and hypogammaglobulinemia were considered to be responsible for this result. The third case was a patient with an acute leukemia complicated by an anal fissure. In spite of her severe neutropenia during the remission induction, perianal deep infection was successfully avoided by the local hygene. Therefore, we assume that a perianal hygene is essential to prevent this kind of infection. Adequate administration of antimicrobial agents are required as soon as the infection is suspected. Surgical incision may be indicated when the abscess becomes fluctuant. So an improvement of neutrophils is necessary for the cure of this infection. Combination chemotherapy with DOAP (daunorubicin, vincristine, cytosine arabinoside and prednisolone) delayed but not interrupted the improvement of ulcer. Thus the treatment against leukemia should be performed without hesitation, even if the patient has a perianal infection, as long as an improvement of neutrophil count is expected to some extent.View full abstractDownload PDF (837K) -
Yoshiyuki KURATA, Tadahiro TSUBAKIO, Teruo KITANI1978 Volume 19 Issue 12 Pages 1629-1633
Published: 1978
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSVincristine therapy was performed for 10 patients with ITP and a thrombocytopenic patient with SLE. Two excellent, six good and three poor responses to vincristine were observed. No serious side effects were encountered. In order to investigate the mechanism of elevation of platelet count, platelet kinetic studies were performed before and after vincristine administration; four well responded patients were studied. Prolongation of platelet half-disappearance time and decreased platelet turnover rate were obtained in all of four patients after the administration. While immature megakaryocytes were predominant before vincristine therapy on bone marrow examination, mature platelet producing megakaryocytes were abundant after the treatment.
These findings suggest that vincristine suppresses the destruction of platelets. It induces the elevation of platelet count, which may result in the decreased production of platelet through a regulatory feedback mechanism.View full abstractDownload PDF (365K) -
Tomomitsu HOTTA, Noriyuki HIRABAYASHI, Makoto UTSUMI, Motonari TAKITA, ...1978 Volume 19 Issue 12 Pages 1634-1640
Published: 1978
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSThe survival and its correlation with initial hematologic values of 169 patients with aplastic anemia were analysed in this study. According to survival terms, these patients could be divided into 3 groups: short-term (≤6 months), intermediate (6 months-5 years) and long-term (≥5 years) survivors. The correlation between the survival terms of the patients and their initial hematologic values were analysed and the usefulness of individual and integrative indices of initial hematologic values (reticulocytes≥1×104/cmm:1, neutrophils≥500/cmm:1, platelets≥3×104/cmm:0.5, and the percentage of hemopoietic cells in bone marrow≥30%:1.5) as prognostic indices was evaluated.
The integrative score of initial hematologic values was found to be a best prognostic indices. According to the integrative score, the severity of the disease was classified into 3 groups: severe (score 0∼2), moderate (score 2.5∼3.5) and mild (score 4).
As the patients with score 1 or less have extremely poor prognosis under the conventinal treatment, bone marrow transplantation will be recommended as an another therapeutic approach for these patients at this time, if they have a HLA compatible donor(s).View full abstractDownload PDF (566K)
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Yutaka JOSHITA, Kazuo KUBOTA, Hideaki MIZOGUCHI, Seiichi KITAGAWA, Shi ...1978 Volume 19 Issue 12 Pages 1641-1645
Published: 1978
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSA 61-year-old male patient was admitted to our hospital because of melena and abdominal discomfort. On admission, anemia and hepatomegaly were noted. Hematological examinations showed leukocytosis with a low score of neutrophil alkaline phosphatase. Chromosome studies of bone marrow and peripheral blood without PHA revealed to have a cell line with 45, X, Ph1. However, the banding karyotope of peripheral lymphocytes, cultured for 48 hours with PHA, showed 46, XY, normal pattern. He was diagnosed as having Ph1-positive chronic myelogenous leukemia with missing Y. Duodenal ulcer was detected by the fiber gastroscope.View full abstractDownload PDF (385K) -
Fumihiro SAWA, Tsutomu SAJI, Shigeo SATO, Kumiko OIKAWA, Toshiro NAKAG ...1978 Volume 19 Issue 12 Pages 1646-1653
Published: 1978
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSTwo cases of fulminant hepatitis, associated with aplastic anemias in remission stage, were reported. One patient who had been diagnosed as having congenital aplastic anemia at the age of 4 months showed the fulminant hepatitis at the age of 2 years and died after 4 days of treatments. Serum HBs antigens were negative.
Another patient who had been diagnosed as having idiopathic aplastic anemia at the age of 5 years showed the fulminant hepatitis at the age of 10 years and died after 10 days of treatments. He had no HBs antigens. The postmortem examination of his liver revealed submassive necrosis caused by viral hepatitis.
Fourteen out of twenty-four cases of aplastic anemias observed at this department during the last 14 years were found having liver dysfunctions. Four cases were caused by viral hepatitis, one case was caused by toxic hepatitis, nine cases were caused by side effects of anabolic steroid hormones.
Seven out of fourteen patients died, because of hepatic failures (two cases), bleedings (three cases), pneumonia (one case), and traffic accident (one case).
Patients with liver dysfunctions which had been caused by side effects of anabolic steroid hormones have recovered spontaneously after stopping or decreasing of the drugs, except in one case.View full abstractDownload PDF (618K) -
Mikio TAKEUCHI, Atsushi KIMURA, Tooru NAKAO, Ichiro KUROKAWA1978 Volume 19 Issue 12 Pages 1654-1664
Published: 1978
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSEssential athrombia is one of the congenital hemorrhagic disorders caused by the qualitative defect of blood platelets. In the present paper, detailed study of three cases and their nine relatives is reported.
Case 1 (proposita); 12-year-old girl. She is a product of consanguineous marriage, and her parents are also the products of consanguinity. One of her sisters and one of her aunts of father's side are died from severe bleeding in infancy. Laboratory data revealed slight thrombocytopenia, prolonged bleeding time and slightly defective clot retraction. As for the platelet function tests, glass retention and aggregation induced by ADP, collagen, adrenalin and thrombin were impaired. Bovine fibrinogen and Ristocetin induced aggregation were normal. Platelet Factor 3 availability and thrombelastogram gave normal results.
Case 2; 22-year-old female, Case 3; 33-year-old female. Both are the aunts of father's side of Case 1. Laboratory data of both cases revealed slight thrombocytopenia, prolonged bleeding time and defective platelet aggregation resembled with that of Case 1. Clot retraction and glass retention of platelets were within normal range.
As for the nine members of the family, the bleeding time were normal and none of them have clinical bleeding tendency. Some of them, however, were suspected to have defective platelet aggregation when induced by ADP.View full abstractDownload PDF (686K) -
Satoru TANAKA, Kenkichi KITA, Hirofumi YASUE, Tadahiro ITO, Mitsuhiro ...1978 Volume 19 Issue 12 Pages 1665-1671
Published: 1978
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSAn autopsy case of Wiskott-Aldrich syndrome in a 21-year-old male was reported. His death was due to renal failure and digestive tract bleeding. He had suffered from repeated infections, eczema and bleeding tendency since 6 months after birth. The prolonged survive of this patient might be due to deligent management rather than due to a mild form of the disease because he had severe recurrent infections and bleeding tendency during the active clinical course.
The family history revealed that a younger brother of his mother had died of similar syndrome in infancy.
Laboratory data during the last admission showed marked thrombocytopenia, slight lymphocytopenia and eosinophilia. Delayed hypersensitivity responses tested with PPD and DNCB were absent. The in-vitro blastogenic responses of lymphocytes by PHA and Con A were decreased. The level of IgM was decreased to the lower limit of the normal range but IgE level was normal. There was a marked deficiency of isohemagglutinin and of the ability to form antibodies against typhoid and paratyphoid vaccine. E-rossete forming cells were also decreased.
The histological findings at autopsy showed generalized depletion of lymphocytes in the lymphnodes including paracortical regions. Macroscopically the kidneys were small and firm with finely granulated surfaces. Microscopically numerous glomeruli were the sites of intense scarring and extensive tubular atrophy was present supporting histological diagnosis of chronic glomerulonephritis. There were marked degree of interstitial fibrosis and the infiltration of small round cells.View full abstractDownload PDF (702K) -
Morio AIHARA, Kazuyoshi NARITA, Asano KIMURA, Yoichi CHIBA, Yutaka YOS ...1978 Volume 19 Issue 12 Pages 1672-1676
Published: 1978
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSVon Willebrand's disease has been thought to be hereditary bleeding disorder. Recently, about 14 cases of acquired von Willebrand's syndrome were reported in the world. Most of them are associated with immunological disorders such as SLE, monoclonal gammopathy, myeloma and CLL.
In this paper, a 61 year-old woman was described with laboratory data of von Willebrand's syndrome and gastrointestinal bleeding. She had no past and family history of abnormal bleeding.
Laboratory data showed low F. VIII coag, low F. VIII ag, but bleeding time was varied from normal to mildly prolonged value, and PL count and PL adhesiveness were both normal.
Ristocetin induced platelet aggregation was nil, and F. VIII vwf measured with the method of Weiss revealed low value. Later, she suddenly collapsed with massive hematemesis, and was cured by total gastrectomy in emergency with transfusion of AHF.
This is provably the first case of acquired von Willebrand's syndrome with gastrointestinal bleeding unassociated with basic disorder in Japan.View full abstractDownload PDF (443K) -
Hiroshi FUJII1978 Volume 19 Issue 12 Pages 1677-1683
Published: 1978
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSA 46-year-old female was admitted to Kyoto First Red Cross Hospital with the complaint of hematemesis and melena. An ulcerated lesion at gastric antrum was found by the upper gastrointestinal series and endoscopy and biopsy-specimen showed group V. The peripheral blood findings revealed anemia, leukocytosis with immature granulocytes, and normal neutrophil alkaline phosphatase activity. The bone marrow was markedly hypercellular with positive Ph1 chromosome, 46, XX, t (9; 22) (q34; q11). The vitamin B12 level was high.
Since these findings revealed chronic myelogenous leukemia associated with gastric cancer, gastrectomy was performed. The resected specimen showed Borrmann type II gastric cancer, 2×2 cm in size. Histological examination showed tubulary and muconodular adenocarcinoma.
The incidence of patients with leukemia and coexistent primary cancer was found to be 0.56∼0.64% in Japan, which seemed to be lower than that in the United States. Five cases with the coincidence of chronic myelogenous leukemia and gastric cancer have been reported in Japan.View full abstractDownload PDF (902K) -
Hideo ENOKIHARA, Kenji SAITO, Sakae HAZAMA, Koji HIROSE, Hideaki KOMAT ...1978 Volume 19 Issue 12 Pages 1684-1689
Published: 1978
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSA 48-year-old woman was admitted to our hospital because of anemia since several years and abdominal distension since a month. Physical examination revealed anemia, jaundice, giant splenomegaly reaching 14 cm below the left costal margin, and slightly decreased vibration sense of lower extremities. Blood examination disclosed pancytopenia. Bone marrow aspiration showed erythroid hyperplasia with megaloblastic changes. The liver function tests were within normal limits except for a slight increase in indirect bilirubin and a marked increase in LDH. Decreased vitamin B12 absorption with correction by oral intrinsic factor and methylmalonic aciduria confirmed a diagnosis of pernicious anemia. She also had the positivity for the direct and indirect antiglobulin tests, which became negative 10 days after the start of the parenteral administration of vitamin B12. Following the therapy, anemia improved, but the recovery of leukopenia and thrombocytopenia was incomplete. The size of spleen has reduced to 7 cm below the costal margin by three months but did not change thereafter. One year after the diag nosis, splenectomy was performed. The portal pressure was normal. Histological examination of the spleen revealed chronic congestion, and that of the liver showed no abnormalities. The pancytopenia subsided soon after the operation. The significance of the splenomegaly and positive antiglobulin tests observed in this patient was discussed.View full abstractDownload PDF (652K) -
Tadashi KOIKE, Tsuneyasu TSUKADA, Yasuko YAMADA, Yoshitaka AOYAGI, Tik ...1978 Volume 19 Issue 12 Pages 1690-1698
Published: 1978
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSA 12-year-old girl was admitted to our clinic because of fever and anemia in October, 1976. On hematological studies, including morphological, cytochemical and electron microscopical examinations, she was diagnosed as acute lymphoblastic leukemia (ALL) although thrombocytosis and a high level of serum vitamin B12, atypical features of ALL, were demonstrated.
Complete remission was achieved rapidly with vincristine and prednisolone treatment. After approximately 6 months of complete remission, the peripheral blood gradually showed chronic granulocytic leukemia (CGL) features and the Philadelphia chromosome was demonstrated on bone marrow cytogenetic studies. She was treated with busulphan and was in good health until October, 1977, when she developed “lymphoblastic” meningeal leukemia without any prior evidence of blast crisis common to CGL in either peripheral blood or bone marrow.
Intrathecal injection of cytosine arabinoside successfully induced remission. In February 1978, she again developed ALL which was again brought to complete remission with vincristin and prednisolone.
Surface marker studies of blasts in cerebrospinal fluid during her meningeal leukemia revealed E rosettes of 55% and EAC rosettes of 30%.
Those in venous blood during her hematological relapse as ALL indicated null cell type.
The terminal deoxynucleotidyl transferase activity during the ALL relapse showed a moderately high level.
It was speculated that this was a case of CGL presenting transient lymphoblastic conversion twice during the clinical course.View full abstractDownload PDF (888K) -
Yukkio TSUNEMATSU, Michiko SASAKI, Ryo KOIDE, Koichi SHIMIZU1978 Volume 19 Issue 12 Pages 1699-1707
Published: 1978
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSA boy, born in 1961, was first admitted in 1967 and a diagnosis of aplastic anemia was made. He was on androgenic steroids for five and a half years and was re-admitted in November 1973, because of severe anemia and a 12×9 cm mass in the left upper abdominal quadrant. Liver scan demonstrated hepatomegaly with a large filling defect. HBs-Ag and α1-fetoprotein were not detected. Bone marrow examination revealed normoblastic hyperplasia with many ringed sideroblasts. A diagnosis of sideroblastic anemia was made. Chromosome examination revealed aneuploidy in 50% of the analysed cells (44, X-Y, -21/ 45XY, -21/ 46XY lq+). He was treated with vitamine B6 and the response was not sufficient. He died in July 1976 suddenly. On autopsy, a single, well circumscribed 11.8×8 cm tumor was found in the left lobe of the liver. Histologically, the tumor was well differentiated liver cell adenoma. There was a hypercellular marrow with myeloid cells, but no increase of the atypical cells. This is the first case of hepatocellular adenoma induced by long-term androgenic steroids reported in the Japanese literature. Discussion was made on an association between the aplastic anemia and sideroblastic anemia terminating as myeloproliferative disorder.View full abstractDownload PDF (979K) -
Haruya YOSHIKAWA, Kazuya OKAMOTO1978 Volume 19 Issue 12 Pages 1708-1716
Published: 1978
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSA case of acute lymphocytic leukemia relapsed with leukemic pleuritis and paraplegia was reported.
A 46-year-old female was admitted to our hospital because of hemorrhagic tendency. Examination of the blood showed hemoglobin, 5.2 g/dl; leukocytes, 4,600/cmm with 9% lymphoblasts; and platelets, 26,000/cmm. A sternal marrow aspiration revealed 91.2% lymphoblasts in the myelogram. A complete remission for 2 months was induced by VENP-therapy. Then, relapse occurred with leukemic pleuritis and complete transverse spinal palsy at the 5th to 6th thoratic segment. AAAP-therapy (ACNU 50 mg d.i./day×4, adriamycin 30 mg i.v./day×4, methotrexate 10 mg i.v. & 15 mg i.t. on day 1 and 4 and prednisolone 60 mg i.v./day×4) was indicated and soon after one course of this therapy, pleural effusions disappeared and hematological complete remission was reattained. Furthermore she was able to walk without crutches for 4 months despite of repeated hematological relapses and pleural effusions.
Thus, AAAP-therapy was revealed one of excellent chemotherapies for paraplegia complicated with acute leukemia.View full abstractDownload PDF (1465K)
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