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Volume 25, Issue 11
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Yoshitaka SHIMIZU, Tetsuo HASHIDA, Noriko ESUMI, Yoshito MORIOKA, Taka ...1984 Volume 25 Issue 11 Pages 1738-1745
Published: 1984
Released on J-STAGE: January 26, 2009
JOURNAL RESTRICTED ACCESSThirty-six children with malignant lymphomas, including 30 non-Hodgkin's lymphomas (NHL) and 6 Hodgkin's disease (HD), and 8 children with histiocytic medullary reticulosis (HMR) were treated over the past 9 years between July 1974 and June 1983. Thirty-three cases were males and 11 females.
In NHL, 8 cases had their primaries in the abdomen, 8 in peripheral lymph nodes, 6 in the mediastinum and 6 in other sites. Three children were in stage I, 4 stage II, 16 stage III and 7 stage IV, according to the Murphy's staging system. Twenty-four NHL cases of 30 were classified histologically by LSG classification, showing that all were diffuse lymphomas with 5 medium seized, 8 mixed cell, 10 lymphoblastic and one Burkitt type.
In HD, 4 children had their primaries in peripheral nodes and 2 in the mediastinum. Two children were in stage IIA, 1 stage IIB and 3 stage IIIB according to Ann Arbor's classification.
Eight cases of HMR demonstrated high fever, active proliferation of atypical histiomonocytes with erythrophgocytosis in bone marrow and, except one, disseminated intravascular coagulopathy.
Most cases of NHL and HMR were treated with pulse administration of multi-agents chemotherapy (ACOP etc), and those cases with HD were treated with COPP.
Therapeutic results demonstrate that, only 11 of 30 NHL have kept continuous complete remissin (CCR) and 7 of these 11 children have been alive off therapy from 38 to 109 months. All 6 children with HD were alive and well during the period ranged from 8 to 96 months. Five of 8 HMR have had CCR currently during the period ranged from 10 to 35 months' follow-up.View full abstractDownload PDF (584K) -
Masamitsu KARASAWA, Hiroshi YATABE, Hiromasa YAMAUCHI, Toshimasa ARAI, ...1984 Volume 25 Issue 11 Pages 1746-1753
Published: 1984
Released on J-STAGE: January 26, 2009
JOURNAL RESTRICTED ACCESSThe Clinicopathological features of 51 patients with untreated megaloblastic anemia due to vitamin B12 deficiency were analysed. The material consisted of 40 patients with Addisonian pernicious anemia (PA), 10 with postgastrectomy B12 deficiency (PG) and one with blind-loop syndrome. No sex preponderance was noted in PA patients with mean age of 63 years. Mean Broca's indices for PA and PG patients were 88.9% and 77.2%, respectively, showing a poorer nutritional status in PG patients. The mode of onset, clinical symptoms, hematological findings including bone marrow morphology, blood chemistry, ferro-erythrokinetic data and nervous system involvement were all essentially analogous in PA and PG patients. The spleen was palpably enlarged in 25% of PA patients at diagnosis. The correlations between various laboratory parameters were examined.View full abstractDownload PDF (586K) -
Tadao FUNATO, Masatoshi HAYASHI, Yasushi HIRASAWA, Hideki OHTANI, Masa ...1984 Volume 25 Issue 11 Pages 1754-1759
Published: 1984
Released on J-STAGE: January 26, 2009
JOURNAL RESTRICTED ACCESSRelationship between the cell expression property and immuno-regulating function was studied using peripheral ATL cells and cultured ATL cells collected from 2 patients with ATL. The result revealed that ATL cells do not only turn positive with OKT3+4+, but also turns positive with OKT17+ inhibiting the Ig production function of B cells of Ig producing system of the healthy subjects. The inhibitory effect does not need the presence of OKT4+ cells and OKT8+ cells, and is sensitive to irradiation, and also related to expression of ATL cells by OKT17+. Consequently, it was clarified that the ATL cells, although bearing OKT4+, have no helper function, but have the suppressor function with OKT17+. This is of interest in contemplating immunological abnormalities of this disease.View full abstractDownload PDF (392K) -
Noriyuki TATSUMI, Kohji HASHIMOTO, Keiko KAWARABAYASHI, Junko TATSUMI, ...1984 Volume 25 Issue 11 Pages 1760-1765
Published: 1984
Released on J-STAGE: January 26, 2009
JOURNAL RESTRICTED ACCESSVolume changes of red cells were observed in the mixture of isotonic saline solution and isotonic glycerol solution using an electric apparatus, continuous MCV analyzer.
The cell volume increased as the concentration of glycerol in the mixture was increased. Kinetic studies on the permeation of glycerol through the cell membrane revealed that the permeation was a dimer reaction.
When the cells were suspended in mixture having the saline and glycerol mixing ratio of 1:2, the cells swelled to hemolyse. Pathologic cells like iron deficiency anemia cells showed prolongation of the swelling time. As the mixing ratio of 1:2 was corresponded to the Gottfried's condition for glycerol lysis test, the time to reach the peak point on the glycerol MCV change curve (Tmax) showed a high correlation with the GLT50 value of Gottfried.
From these findings it was concluded that volumetric method for glycerol lysis test would be useful for the basic or the clinical study of membrane properties of red cells.View full abstractDownload PDF (381K) -
Yasusuke ONOZAWA, Yasushi YAHARA, Yasufumi IMAI, Machiko HIGASHIDANI, ...1984 Volume 25 Issue 11 Pages 1766-1771
Published: 1984
Released on J-STAGE: January 26, 2009
JOURNAL RESTRICTED ACCESSAclacinomycin is a newly developed anthracycline which is effective in the cases of acute non-lymphocytic leukemia resistant to daunomycin and adriamycin. A combination chemotherapy of BH-AC·AMP as well as BH-AC·DMP regimen has achieved 70 to 80 percent complete remission in ANLL.
However, anaphylactic shock and liver dysfunction have been reported as the side effects of BH-AC. Aclacinomycin is also known to cause cardiotoxicity, hemorrhage of urinary bladder, and GI upset such as nausea, vomiting and diarrhea.
BH-AC·AMP regimen has been used in 19 cases of ANLL and a case of chronic granulocytic leukemia with blast crisis.
Soon after the completion of this treatment, acute pancreatitis developed in 8 cases (40%) and only high serum amylase was observed in 4 other cases. Patients with acute pancreatitis have developed frequent watery diarrhea, upper abdominal pain and tenderness, nausea and vomiting, and also laboratory data of these patients showed elevated serum amylase and lipase as well as abnormal amylase clearance. These pancreatitis except for one were relatively mild and improved clinically in a few days. However, one patient, 68-y.-o. female with AMoL developed profound metabolic acidosis and demised. An autopsy revealed diffuse hemorrhagic pancreatitis.
Acute pancreatitis which we observed under the BH-AC·AMP regimen tended, to occur in the 1st induction chemotherapy and in elderly patients.View full abstractDownload PDF (1159K) -
—An Analysis of 728 Cases as of 1967 to 1977—Ichiro TSUKIMOTO, Masahiro TSUCHIDA, Fumio BESHO, Junichi AKATSUKA, Ta ...1984 Volume 25 Issue 11 Pages 1772-1777
Published: 1984
Released on J-STAGE: January 26, 2009
JOURNAL RESTRICTED ACCESS1) In order to study the long term prognosis in childhood leukemia, 728 cases who have been diagnosed and treated from 1967 to 1977 were retrospectively collected from the institutes that belong to the “Study Group of Childhood leukemia Therapy” (Reseach Committee of Ministry of Health and Welfare), and analized in November 1983.
2) The 728 leukemic patients consist of 483 cases with ALL (66.3%), 224 ANLL (30/8%) and 21 CML (2.9%).
3) One hundred and twenty five (17.2%) have survived 5 years or longer and are still alive. consisting of 110 cases with ALL (88.0%), 11 ANLL (8.8%) and 4 CML (3.2%). Eighty nine patients (12.2%) have been free of relaps 5 years or longer consisting of 80 cases wtih ALL (89.9%) and 9 ANLL (10.1%).
4) Longer than 5 year survival rates were approximately 15% in whole leukemics from 1967 to 1975, and about 25% in those from 1975 to 1977. Longer than 5 year relapse free rate were 3.3% in whole leukemics of 1967, approximately 10% in those from 1968 to 1974, and increased to around 20% in the patients from 1975 to 1977.
5) longer than 5 year survival rate of patients with ALL gradually increased from 10.5% among the cases of 1967 to 34.8% in 1977. Longer than 5 year disease free rate increased from 5.3% of 1967 to 25.8% of 1977.
6) Only about 10% of children with ANLL have survived for 5 years more after the initial treatment throughout those 10 years. All the survivors however are relapse free.
7) About 20% of CML have survived 5 years or longer.View full abstractDownload PDF (441K) -
—Evolution of Clones with Additional Ph1 Chromosome Detected by means of a Large Number of Karyotype Analysis—Kimio TANAKA, Nanao KAMADA, Nobuo OGUMA, Yasuo TAKIMOTO, Atsushi KURAM ...1984 Volume 25 Issue 11 Pages 1778-1787
Published: 1984
Released on J-STAGE: January 26, 2009
JOURNAL RESTRICTED ACCESSA large amount of karyotype analysis was performed on 16 cases with chronic myelocytic leukemia at the time of diagnosis and of those 6 cases at blastic phase, in order to investigate pathophysiological conditions of CML from cytogenetic point of view.
All cases examined in chronic phase, even in the early phase of the disease, were found to have 1.3 to 12.4 percent of additional abnormal cells, showing numerical changes in chromosomes 8, 9, 15, 18 and 21, and structural changes in chromosomes 1, 2, 3, 4, 5, 8, 17 and 18, including common karyotypic abnormalities found in blastic phase, such as +9, +18 and +22q-. Of the six cases examined at both phases, chronic and blastic, only one case (#1) showed gradual increase of abnormal clone found in chronic phase and finally terminated in blastic phase with additional chromosome aberrations of i(17q). Four cases showed new additional abnormal clones during the chronic phase (#3 and 6) or at the time of blastic phase (#2 and 4). The last case (#5) did not show any additional clone during the coruse of the disease.
These findings indicate that Ph1 positive cells are essentially labile and acquisite easily additional chromosome aberrations in any stage of the disease.View full abstractDownload PDF (1056K) -
Teruo INO, Yoshihisa MORISHITA, Toshikazu MATSUI, Suzuaki SHIMIZU, Har ...1984 Volume 25 Issue 11 Pages 1788-1795
Published: 1984
Released on J-STAGE: January 26, 2009
JOURNAL RESTRICTED ACCESSThe French, American and British cooperative group (FAB) proposed in 1976 a classification of acute leukemia based on morphological and cytochemical criteria. In order to test the applicability and clinical usefulness of the FAB classification, an evaluation was performed on 104 cases of acute leukemias and related disorders treated in our institute. We followed the criteria of the original proposal with a clearer definition of promyelocytes as immature cells with 20 or more azurophilic granules. Those with less than 20 granules were considered to be blasts.
The number of cases of each category as determined by consensus of the 3 observers was 16 L1, 9 L2, 0 L3, 20 M1, 15 M2, 8 M3, 11 M4, 7 M5, 2 M6 and 16 myelodysplastic syndrome (MDS). The rate of complete coincidence of the classification among the 3 observers was 81% in ranged from 56% in L2 to 100% in M3. Eight cases of “ALL” of the FAB classification had been classified as peroxidase negative AML in the former conventional classification, and 4 cases of MDS as erythroleukemia. “ALL” might include in its category peroxidase negative AML and other undefinable forms, and the distinction between L1 and L2 was not always clear even after scoring system was applied. We found that all M2 cases had more than 10% promyelocytes, or more than 25% granulocytes beyond the promyelocytic stage. In the classification of M4 and M5, non-specific esterase staining with α-naphthyl acetate used as substrate was very useful.
The FAB classification was found to be more objective in its criteria than previously employed conventional one, and could be used as a method of standard classification among different institutes. However, it should need further refinement supplemented with electronmicroscopic and immunologic examinations.View full abstractDownload PDF (657K) -
—Changes in Absolute Counts and in Staining to α-Naphthyl Butyrate Esterase—Eijiro OHMOTO, Isao TAKAHASHI, Fumikazu KOHI, Shigeo AOYAMA, Noritoshi ...1984 Volume 25 Issue 11 Pages 1796-1802
Published: 1984
Released on J-STAGE: January 26, 2009
JOURNAL RESTRICTED ACCESSUsing α-naphthyl butyrate esterase (α-NBE) staining, the absolute monocyte count wes examined in 30 patients with acute leukemia, including 20 with acute nonlymphocytic (ANLL) and 10 with acute lymphocytic leukemia (ALL). The absolute monocyte count in the peripheral blood was significantly decreased to 33.5±30.5/cmm in untreated cases and increased up to 268.0±88.5/cmm in remission (normal subjects: 291.4±109.0/cmm). There was the inverse correlation (r=-0.63, p<0.01) between the percentage of leukemic cells in the bone marrow and peripheral monocyte counts. Furthermore, peripheral monocytes were cytochemically classified into 3 types according to α-NBE staining; namely, monocytes slightly, moderately and heavily staind were classified into Type I, Type II and Type III, respectively. In normal subjects the percentage of monocytes of Type I was 9.3±4.8%, Type II 29.6±9.0%, Type III 61.1±11.5%. On the other hand, Type I monocytes were predominant in untreated acute leukemia, while the populations of monocytes returned to those in normal subjects in remission. Some of relapsed cases showed not only the decrease in the absolute monocyte count but also the increase of Type I and Type II monocytes before the clinical features of the relapse were apparent.
The changes of absolute monocyte count and also its staining behavior to α-NBE are thought to reflect the clinical course of acute leukemia and to be useful for early diagnosis of the relapse.View full abstractDownload PDF (995K)
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Hiromi YASHIGE, Hitoshi IMANISHI, Tatsuro TAKINO, Toshiaki NAKASHIMA, ...1984 Volume 25 Issue 11 Pages 1803-1808
Published: 1984
Released on J-STAGE: January 26, 2009
JOURNAL RESTRICTED ACCESSA 65-year-old female was admitted to Kyoto Yosanoumi Hospital on 12th March, 1982. She complained of palpitation and general fatigue. Physical examination demonstrated anemia, hepatosplenomegaly and no lymphadenopathy. In peripheral blood the red blood cell count was 309×104/mm3. Hemoglobin was 7.3 g/dl. Hematocrit was 24.5%. The white blood cell count was 3300/mm3. The platelet count was 15.2×104/mm3. Bone marrow aspiration revealed that the frequencies of plasmacytoid cells was 18.4%. In laboratory findings total protein was 8.7 g/dl with γ-gl 33.5%. IgM was 8,320 mg/dl. Serum protein study revealed monoclonal gammopathy with IgM-κ type and positive Sia test. Incubation of serum at 56°C for 30 min. resulted in formation of opapue gelatinous materials. All these findings are compatible with the diagnosis of macroglobulinemia with pyroglobulin. But cryoglobulin was not demonstrated. Melphalan 4 mg/day and predonin 30 mg/day were effective in reducing IgM and nomalizing total protein.View full abstractDownload PDF (498K) -
Taijiro ISHIYAMA, Naoki SUGAYA, Tatsuo MIYAYAMA, Masakuni SUGIMOTO, Yo ...1984 Volume 25 Issue 11 Pages 1809-1813
Published: 1984
Released on J-STAGE: January 26, 2009
JOURNAL RESTRICTED ACCESSA 69 year-old man was examined in June 1982, complicaining of tingling pain and numbness in the palms and first 3 fingers of each hand. A diagnosis of bilateral carpal tunnel syndrome was made. Surgical decompression of the median nerves was done with good symptomatic relief.
In August 1983, he was admitted to Juntendo University hospital with swelling of both his hands and feet. Laboratory findings revealed a monoclonal IgG of lambda type in the serum, with positine urinary Bence Jones protein. An examination of the bone marrow reaveled a proliferation of plasma cells supporting multiple myeloma. An X-ray-film survey of both the humerus showed punched out lesion. Amyloid depositions in his stomach and rectum was proved by biopsy.
He was treated with melphalan and discharged.View full abstractDownload PDF (919K) -
Takemi OHTSUKI, Nobumasa INOUE, Takashi SUGIHARA, Masakiyo MANNOJI, Yo ...1984 Volume 25 Issue 11 Pages 1814-1818
Published: 1984
Released on J-STAGE: January 26, 2009
JOURNAL RESTRICTED ACCESSA 24 year old female of (Evans' syndrome) with 8 weeks pregnancy was treated for thrombocytopenia with prednisolone, high-dose γ-globulin, and artificial abortion. Thrombocytopenia was first noted just before the first delivery in 1980. No treatment was given until May 20, 1983, when the patient was consulated to the second pregnancy with marked bleeding tendencies (platelet count: 900/mm3). Bone marrow findings were identical to those of (Evans' syndrome). Laboratory examinations revealed a positive direct Coombs test, increased indirect bilirubin (0.8 mg/dl) with low haptoglobin level (40 mg/dl). Prednisolone (60 mg/day×14 days) was first challenged with minimal response. The patient responded to a high dose γ-globulin (400 mg/kg/day×5 days) and platelet count was increased to 10×104/mm3. Thus, artificial abortion was carried out. After the procedure, she has been well without any treatment. From these observations, the pathogenesis of thrombocytopenia in this patient appears to be related to pregnancy, and the removal of the unknown factor by the artificial abortion could be one of the choice of the treatment for severe cases with the pregnancy-related idiopathic thrombocytopenic purpura. Temporary administration of high-dose γ-globulin is beneficial to increase platelet counts sufficient for the artificial abortion. The clinical consideration for the treatment of the ITP patients with early stage of pregnancy is discussed.View full abstractDownload PDF (359K) -
Fumihiro FUJIWARA, Seizaburo KONISHI, Noriko SATO, Shinsaku IMASHUKU, ...1984 Volume 25 Issue 11 Pages 1819-1824
Published: 1984
Released on J-STAGE: January 26, 2009
JOURNAL RESTRICTED ACCESSA 7 year-old Japanese male of congenital high red cell membrane phosphatidyl choline hemolytic anemia with stomatocytosis and with increased sodium transport is reported. At his age of 3, moderate amneia (RBC 158×104/μl with marked reticulocytosis (22.9%) and mild jaundice (indirect bilirubin 1.2mg/dl) were first noted with normal hepatic function tests. Red cell morphology demonstrated marked stomatocyosis with erythroid hyperplasia in the bone marrow. Red cell life span (51Cr T1/2) was 15.5 days. Osmotic fragility of the patient's red cells was decreased with the Dacie I pattern on autohemolysis tests. Plasma lipids were: total cholesterol 124mg/dl, triglycerides 143mg/dl, phospholipids 158mg/dl, β-lipoproteins 314mg/dl, HDL-cholesterol 26mg/dl. Red cell membrane lipids (μg/1010 RBC) were: total phospholipids 3,102 (N: 2,604±241), phosphatidyl choline 1,042 (733±64), Phosphatidyl ethanolamine 862 (806±86) sphingomyelin 676 (663±73), and phosphatidyl serine 481 (366±38), associated with increased free cholesterol (1,415; N: 1,202±103). Sodium influx (3.72mmoles/l RBC/hour) and sodium efflux (rate constant hr-1; 0.40) were markedly increased compared to normal control (1.42±0.16 and 0.24±0.05) respectively. Red cell Na and K concentrations were maintained normally. No other case was detected in the patient's pedigree. No abnormality was detected on hemoglobin metabolism or on glycolytic activities.View full abstractDownload PDF (505K) -
—Identified by Platelet Peroxidase—Kunishige KATO, Mitsuoki EGUCHI, Kenichi SUGITA, Mutsuko ISHII, Hirosh ...1984 Volume 25 Issue 11 Pages 1825-1830
Published: 1984
Released on J-STAGE: January 26, 2009
JOURNAL RESTRICTED ACCESSA case of acute myelogenous leukemia with a prodominant megakaryocytic cell line is reported.
A 10-year-old boy was admitted to Dokkyo University Hospital because of anemia and bleeding tendency in May 1982. A diagnosis of acute myelogenous leukemia was made and chemotherapy (ACMVP protocol) was initiated. Complete remission was obtained after 3 months, however, he relapsed in Feb. 1983. Bone marrow after chemotherapy showed 81% blast cells and 5% undifferentiated cells. The number of megaryocytes was increased to 1.665/cmm in the bone marrow but the number of platelets was only 2.2×104/cmm in the peripheral blood.
Electron microscopic examinations including platelet peroxidase (ppo) and myeloperoxidase (MPO) were performed on bone marrow specimens. A megakaryocytic cell line, which was characteristically PPO positive and MPO negative, was observed in 15% of the total nucleated cells. Megakaryoblasts were difficult to differentiate from myeloblasts morphologically, because both of them were 7∼8μ in diameter, with a round nucleus and scanty endoplasmic reticulum. However, PPO was positive in the nuclear envelope and endoplasmic reticulum of megakaryoblasts. Micromegakaryocytes, which were 8∼10μ in diameter with a round or oval nucleus and numerous endoplasmic reticulum, were also PPO positive in nuclear envelope and endoplasmic reticulum but negative in Golgi apparatus. The megakaryocytes, smaller (10∼20μ in diameter) and less mature than normal megakaryocytes exhibited an undeveloped demarcation membrane and α granules. These small young megakaryocytes were hardly differentiated on light microscopic examination.
The case with acute myelogenous leukemia with a predominance of megakaryocytes without thrombocytosis is very rare and a combination of electron microscopic techniques with MPO and PPO was useful in making the diagnosis.View full abstractDownload PDF (1579K) -
Yoshitaka HISHITANI, Keiichi SHINODA, Takio YASUI, Toshiji MOZAI, Tada ...1984 Volume 25 Issue 11 Pages 1831-1836
Published: 1984
Released on J-STAGE: January 26, 2009
JOURNAL RESTRICTED ACCESSA case of multiple myeloma of a 58 year old male is reported. The patient was admitted because of low back pain. Physical examinations at admission revealed mild anemia. The liver and spleen were not palpable, and no lymphoadenopathy was found. The hematocrit was 32.0% and the white cell count was 3,500 with 24% neutrophils and 61% matured lymphocytes. Plasma cell was not found in the peripheral blood. Bone marrow study revealed hypocellularity with 76.7% myeloma cells. Myeloma cells resembled rather lymphoid cells especially seen in patient with acute lymphoblastic leukemia. The plasma protein concentration was 6.2 gram per 100ml (the albumin 69.0% and the γ-globulin 7.7%) with slight M spike in the fast γ region. The M component was identified IgG-κ type by immunoelectrophoresis. The urine containd Bence Jones protein κ type in large quantities. The skeletal rentgenograms revealed multiple punched out lesions with generalized osteoporosis.
This case may be considered as unusual case of myeloma because in spite of a large amount of urinally Bence Jones protein, slight M component was detected in the serum. Therefore, this case is considered as an intermediate type between Bence Jones myeloma and IgG+Bence Jones myeloma.View full abstractDownload PDF (2081K) -
Tsuyoshi NAKAMAKI, Tsuyoshi SAITO, Umeki INOUE, Shigeru TOMOYASU, Nobu ...1984 Volume 25 Issue 11 Pages 1837-1842
Published: 1984
Released on J-STAGE: January 26, 2009
JOURNAL RESTRICTED ACCESSA 26-year-old woman with a past history of 3 spontaneous abortions was admitted because of neurological symptoms due to multiple cerebral infarctions. Pancytopenia with reticulocytosis was observed in the blood. Bone marrow examination showed erythroid hyperplasia. The direct Coombs' test was positive using anti-C3c and C3d antisera. The indirect Coombs' test was also positive. The DNA antibodies were positive and serological tests for Syphillis were false positive biologically. The LE cell phenomenon was negative. A diagnosis of Evans' syndrome was made.
The PT, aPTT and recalcification time were all prolonged. The thrombin time and platelet factor 3 activity were normal. In spite of these coagulation abnormalities, thrombelastogram showed hypercoagulability and plasma β-thromboglobulin was elevated. The antithrombin III and α2-macroglobulin were normal. A lupus anticoagulant was detected using the kaolin clotting time with mixture of patient's and normal plasma by the method of Exner et al. The “lupus cofactor” phenomenon was also detected. The anticoagulant was neutralized by anti-human IgA serum.
The paradoxical hypercoagulability despite of lupus anticoagulant in vivo might be an important factor on the occurrence of cerebral thrombosis in this case.View full abstractDownload PDF (363K) -
Yoshiro MURAI, Noriko NAKAMURA, Kenji IKEBUCHI, Makiko FUKAYAMA, Akiyo ...1984 Volume 25 Issue 11 Pages 1843-1847
Published: 1984
Released on J-STAGE: January 26, 2009
JOURNAL RESTRICTED ACCESSA brother and a sister with combined congenital deficiency of factor V and VIII were reported.
The propositus was 70 year-old woman who gave a long history of excessive bleeding following minor lacerations and who had been transfused because of prolonged and severe bleeding following a tooth extraction. The activated thromboplastin time and the prothrombin time were prolonged. Both factor V and VIII coagulation activities were depressed, but assays of factor VIII related antigen were normal. The protein C activity was 43% and the activity of protein C inhibitor was 46%. The slight decrease in these activities were considered to be due to associated liver cirrhosis. The patient's platelet showed normal aggregation patterns in response to various agents including ristocetin. Infusion of cryoprecipitate in the propositus gave no elevtion of factor VIII activity more than the expected increase.
The parents of these patients were first cousins. Therefore, inheritance pateren of combined deficiency of factor V and VIII was considered to be autosomal recessive. This conclusion is shared by many reports. Our finding concerning protein C inhibitor activities was not consistent with the report of Marlar and Griffin who suggested that deficiency of protein C inhibitor was likely to be essential to this disorder.View full abstractDownload PDF (378K) -
Hiroyuki KANBAYASHI, Kyoko IWAYA, Hiroshi YOSHIDA, Toshiyuki ISHIBASHI ...1984 Volume 25 Issue 11 Pages 1848-1853
Published: 1984
Released on J-STAGE: January 26, 2009
JOURNAL RESTRICTED ACCESSAn autopsy case of chronic myelogenous leukemia (CML) terminating megakaryoblastic transformation was reported. The patient was a 75-year-old male who had been diagnosed as CML and treated with busulfan since June, 1980. He admitted again to our hospital on August, 1982 because of low grade fever and general fatigue.
The white cell count in the peripheral blood was 11.5×104/cmm, and bone marrow was dry tap. Electron microscopy of the blast cells revealed micromegakaryocytes with typical granules, demarcation membrans, and surface blebs. Platelet-peroxidase (PPO) was examined, showing positive reaction in rough endoplasmic reticulum and nuclear envelope of the blast cells, though it was not positive in granules and Golgi apparatus. In addition, the monoclonal antibody to platelet glycoprotein IIb IIIa, TP80, reacted positive in about 20% of the blast cells.
Chromosomal analysis from peripheral blood revealed double clones of 46, XY, t (9:22) (q34:q11) and 49, XY, +8+19, +21, t (9:22) (q34:q11).
From these findings, this patient was diagnosed as megakaryoblastic transformation. He did not respond to chemotherapy and died 4 months after transformation.View full abstractDownload PDF (1033K) -
Shiro NAKAYAMA, Teruo YOSHIDA, Hiroki YABE, Kenichi NAGAI1984 Volume 25 Issue 11 Pages 1854-1859
Published: 1984
Released on J-STAGE: January 26, 2009
JOURNAL RESTRICTED ACCESSThe treatment of pernicious anemia is usually straight-forward. However, anaphylactic reactions after injection of vitamin B12 preparation have been described in the past. This is a case report of pernicious anemia in which adverse reactions occurred soon after vitamin B12 injection as manifested by marked neutropenia and severe impairment of liver functions terminating in death.
An 79-year-old man with no significant past illness or history of allergy to drugs was admitted on November 26, 1982 for investigation of anemia. Peripheral blood on admission showed RBC 213×104/mm3, Hb 8.7g/dl, WBC 4,700/mm3 with almost normal differential, and platelet 5.5×10/mm. Bone marrow aspiration revealed erythriod hyperplasia with megaloblastic changes. The serum B12 was 220pg/ml. The diagnosis of pernicious anemia was confirmed by the Schilling test and the presence of intrinsic factor antibodies. About 2 hours after receiving an intramuscular injection of cobamide (Calomide S) 1,000 μg for the first time, severe allergic reactions with a high fever and general skin eruptions developed followed by elevation of transaminases, hyperbilirubinemia and marked granulocytopenia 3 days later. Subsequently, prednisolone 60mg per day was started, and over the next 2 days WBC count gradually returned to normal. However, because of long-lasting high fever despite discontinuation of vitamin B12 therapy, antibiotics were given with a suspicion of complicating sepsis, with which return of a marked granulocytopenia and worsening of hepatic dysfunction occurred in 2 days. His condition continued to deteriorate with disturbances of consciousness, and he died due to respiratory distress 11 days after injection of vitamin B12.
This patient was thought to have sensitivity not only to vitamin B12 but also to antibiotics. At present, it is not clear whether allergic reactions were due to substances added to the drugs as a preservative or to vitamin B12 itself. It is important, however, to be aware of such rare but potentially lethal side effects.View full abstractDownload PDF (449K) -
Ryuichi NOHNO, Hiroshi HARADA, Kimihiro SHIRAISHI, Naomi MASHIKO, Misa ...1984 Volume 25 Issue 11 Pages 1860-1866
Published: 1984
Released on J-STAGE: January 26, 2009
JOURNAL RESTRICTED ACCESSA 78 year-old man was admitted to Kurume University Hospital because of fever and generalized lymphadenopathies in April 1983. He diagnosed as malignant lymphoma 1 year before and treated with chemotherapy for four months. On admission the white blood cell count was 126,000/cmm with 86% abnormal lymphocytes.
A lymphnode biopsy revealed follicular lymphoma (medium-sized cell type by LSG classification). The morphological findings of abnormal lymphocytes in peripheral blood smear showed 51% small cleaved cells, 26% large non-cleaved cells and 9% ATL like lymphocytes. By immunological studies these cells were all mature B cells with IgG, κ monoclonality but expressed common ALL antigen. Chromosome study of our patient revealed abnormalities [53, XY, +7, +10, +12, +17, +18, +18, +20, t (4; 18)].
A diagnosis of leukemic lymphoma was established because of abnormal lymphocytes probably deriving from follicular center cells. Though he received chemotherapy with VEMP, CHOP regimens, he died of pneumonia about two months after diagnosis of leukemic conversion.View full abstractDownload PDF (1438K) -
Shigeo HORIIKE, Katsuhito YOSHITOKU, Taira MAEKAWA, Jun-ichi EDAGAWA, ...1984 Volume 25 Issue 11 Pages 1867-1872
Published: 1984
Released on J-STAGE: January 26, 2009
JOURNAL RESTRICTED ACCESSWe report a case of adult T-cell leukemia (ATL) with disseminated vesicle formation by herpes simplex virus infection, to which Acyclovir, a new anti-viral drug, was very effective.
A 33 year-old housewife was admitted on January 13, 1982 because of productive cough and easy fatigability. Physical examination on admission revealed marked hepatosplenomegaly and generalized lymphadenopathy. An abnormal shadow indicative of pneumonia as observed on chest X-ray. The WBC count was 169,000/cmm, consisting of 86% atypical lymphocytes with lobulated nucleus. These atypical cells had a monoclonal helper/inducer (OKT-4 positive) T-cell phenotype, and the anti-ATLA antibody was positive (×80). The diagnosis of ATL was made, and she received the combination therapy of vincristine, 6-MP, cyclophosphamide and prednisolone. Although partial remmion was achieved after chemotherapy, frank relapse occurred one month later, and she died of pneumonia six months after admission.
During her clinical course, repeated vesicle formation in the skin was seen in addition to the specific skin lesions resulting from leukemic cell invasion. The vesicles were positive for Tzanck test, and the electron microscopic examination of biopsied lesion revealed herpes virus infection. In spite of treatment with gamma-globulin and interferon, hemorrhagic vesicle formation took place over her whole body. After the parenteral administration of Acyclovir at a dose of 15mg/kg/day, vesicles had become dry and formed crust without any side-effect.View full abstractDownload PDF (945K)
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1984 Volume 25 Issue 11 Pages 1873-1877
Published: 1984
Released on J-STAGE: January 26, 2009
JOURNAL RESTRICTED ACCESSDownload PDF (448K) -
1984 Volume 25 Issue 11 Pages 1878-1881
Published: 1984
Released on J-STAGE: January 26, 2009
JOURNAL RESTRICTED ACCESSDownload PDF (392K)
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1984 Volume 25 Issue 11 Pages 1882-1892
Published: 1984
Released on J-STAGE: January 26, 2009
JOURNAL RESTRICTED ACCESSDownload PDF (1012K)
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