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Volume 19, Issue 2
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[in Japanese]1978 Volume 19 Issue 2 Pages 75-83
Published: 1978
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSDownload PDF (676K)
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Hisaichi FUJII, Koji NAKASHIMA, Noboru MATSUMOTO, Shiro MIWA1978 Volume 19 Issue 2 Pages 84-92
Published: 1978
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSIn Japan, 75 cases with the red cell enzyme deficiency which could cause hemolysis have so far been reported and 63 cases of them were examined in our laboratory. These include pyruvate kinase (PK) deficiency [29 cases], G6PD deficiency [21 cases], glucosephosphate isomerase deficiency [7 cases], phosphofructokinase deficiency [4 cases], glutathione reductase deficiency (heterozygous) [4 cases], pyrimidine 5'-nucleotidase deficiency [3 cases], glutathione peroxidase deficiency (heterozygous) [3 cases], phosphoglycerate kinase deficiency [2 cases], LDH deficiency [1 case] and diphosphoglyceromutase deficiency [1 case].
The frequency of G6PD deficiency is strikingly low in Japan, which might be caused by the fact that Japanese have been isolated in island country without positive selective pressure of malaria. Consanguineous marriage was noted in 11 families from 25 PK deficient families. We could characterize the pure homozygous PK variant with identical variant allele, and identified 7 different variants. The PK variants with high Km for phosphoenolpyruvate and heat instability result in severe hemolysis. Clinical and biochemical studies revealed that the clinical manifestation depends on the kind of the deficient enzyme (or isozyme) and the biochemical characteristics of the variant enzyme.View full abstractDownload PDF (659K) -
Part 1 Establishment of a Japanese Burkitt Lymphoma Cell LineHirofumi KISHIMOTO1978 Volume 19 Issue 2 Pages 93-99
Published: 1978
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSA new cell line (JBL) was established in vitro from pleural effusion of a 29-year-old Japanese female with Burkitt lymphoma. She was Epstein-Barr virus (EBV)-seropositive but JBL cells as well as her lymphoma cells were EBV genome-free on repeated testing. A majority of cells from both sources bore monoclonal surface IgM with lambda light chains. The JBL line grew in suspension with a doubling time of 30 hours and consisted of primitive lymphoid cells with large nuclei, prominent nucleoli and vacuolated cytoplasm. Chromosome analysis of JBL cells revealed a pseudodiploid karyotype with a large subtelocentric marker involving chromosome 11 but no chromosome 14 translocation characteristic of African Burkitt lymphoma.View full abstractDownload PDF (964K) -
Fumihiko NISHIOKA, Akira YACHI, Takeo WADA1978 Volume 19 Issue 2 Pages 100-106
Published: 1978
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSThe levels of serum β2-microglobulin (β2μ) in multiple myeloma and other related disease were studied by the double antibody Mancini's method which offered an unique combination of single radial immunodiffusion and the double antibody method for the microdetermination of β2μ.
Serum β2μ levels in multiple myeloma were elevated to a considerable extent, and the same results were obtained in leukemia, malignant lymphoma, SLE and/or solid tumors. This suggests the possibility that in these cases relatively higher levels of serum β2μ may be presumably related to the active production of β2μ in vivo. However, since serum β2μ levels show a tendency to increase significantly in parallel with the serum creatinine levels in the cases of multiple myeloma and solid tumors as well as in the cases of renal disease, changes of serum β2μ levels should be carefully evaluated in comparison with the renal function in cancer patients.
In the cases of CML or gastric cancer serum β2μ levels appeared to be changeable from stage to stage, and a discussion was made in relation to its possible mechanism.View full abstractDownload PDF (530K) -
Akira ONUMA, Kazuie IINUMA, Tadashi HAYASHI1978 Volume 19 Issue 2 Pages 107-115
Published: 1978
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSIn order to investigate the disturbances of central nervous system (CNS) in acute childhood leukemias, EEG records were performed in 41 children suffering from acute leukemias. They were divided into non-CNS-involving (CNS (-) group: 25 cases) and CNS-involving group (CNS ⊕ group: 24 cases). In 24 cases of CNS ⊕ group, increased cell counts in CSF were observed in 13 cases, clear CSF were observed in 6 cases and lumbar punctures were not performed in 5 cases.
The results were as follows:
1) EEG abnormalities were seen in 56.0% of CNS (-) group, and in 83.3% of CNS ⊕ group.
2) Focal signs in EEGs were seen in 14 of 24 cases of CNS ⊕ group, and in 4 of 25 cases of CNS (-) group. Thrce of the 4 cases who revealed focal signs in EEGs in CNS (-) group, developed CNS-leukemias later. (Two cases developed within 1 month, and 1 case developed 12 months later.)
3) The diffuse low voltages in EEGs were commonly seen in the cases treated with vincristine and prednisolone.
4) EEG records were performed in 5 cases treated with Methotrexate (MTX) intrathecal injections and in 3 cases treated with 60Co whole skull irradiations, before and after treatments for CNS-involvements. EEGs, as well as clinical symptomes, showed marked improvements in 6 cases but a slight aggravation was observed in 1 case treated with MTX.View full abstractDownload PDF (657K) -
Toshiyuki TAKAGI, Masao OGURO, Hisashi MAJIMA1978 Volume 19 Issue 2 Pages 116-123
Published: 1978
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSFourteen patients with “leukemic” non-Hodgkin's lymphoma were studied on clinical manifestations and pathologic findings.
A term “leukemic” was defined as histologically proved lymphoma with more than 5% neoplastic cells in bone marrow and positive neoplastic cells in peripheral blood.
Since lymphosarcoma in childhood and youngster with mediastinal mass becomes very frequently “leukemic” at the time when lymphadenopathy is still localized, those patients should be separated as “leukemic type” from stage IV of Ann Abor staging classification.
Autopsy findings showed huge profound lymphadenopathy and massive infiltration of neoplastic cells in the liver, spleen, bone marrow, kidney, lung, and other organs, despite of regressed superficial lymphadenopathy and improved hematology data. Major cause of death was severe systemic hemorrhage.
Eight patients with “leukemic” non-Hodgkin's lymphoma were treated with intensive combination chemotherapy with Adriamycin, Cyclophosphamide, Vincristine, and Prednisolone (ACVP Therapy). Three complete and three partial remissions were observed for a duration of 20∼90 days with repeated ACVP Therapy. Of major side effects including alopetia, numbness, GI disorders, leucopenia and thrombocytopenia, severe leucopenia could be a limiting factor of ACVP Therapy.
Serum LDH level correlated well to therapeutic effects.View full abstractDownload PDF (604K)
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Masanao OKADA, Jun HANAI, Hidekazu KAMITSUZI, Toyohiko ADATCHI, Yosuke ...1978 Volume 19 Issue 2 Pages 124-132
Published: 1978
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSCongenital leukemia is a very rare condition. This report deals with two autopsied cases of congenital leukemia without Down's syndrome.
Case 1. A premature newborn female of 38 weeks gestational period was born to healthy parents. Her weight was 2,400 gm. The pregnancy was uneventfull, except the correction of the breech presentation under the fluoroscopy. Numerous hemorrhagic skin nodules were disseminated over the entire body. Her symptoms were suspected to be due to congenital leukemia, but the hematological examination was not done. The infant died six hours after delivery. The autopsy revealed hepatomegaly (395 gm), splenomegaly (27 gm) and lymphadenopathy. Any anomaly was not observed. Microscopically the extramedullary infiltration of leukemia cells was massive, and few organs were spared from the infiltration. The leukemia cells were composed of myeloblasts, promyelocytes and myelocytes.
Case 2. The female baby was born at full term. Her weight was 3,350 gm. At birth there was purpura at the abdominal wall and the right cheek. At 4 weeks of age skin nodules were observed. Hepatosplenomegaly was apparent. Laboratory examinations were as follows: RBC 239×104/cmm, Plt 3×104/cmm, WBC 14,400/cmm (unclassified immature cells 91%). Antileukemic agents brought a hematological remission, but she died at six months and three weeks of age because of the cytomegalovirus infection. The autopsy revealed the scarcity of the leukemia cells which were observed only in the bone marrow and the pleura. The leukemia cells in the section were positive to Sudan Black B stain and diagnosed as myelogenous.
It seems likely that “congenital leukemia” is a condition different from leukemia of infancy and childhood.View full abstractDownload PDF (1759K) -
Takeo TAKEDA, Isamu HAMADA, Akira YOKOTA, Sohichiro MACHIDA1978 Volume 19 Issue 2 Pages 133-138
Published: 1978
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSA 12-year-old Japanese girl with traumatic cardiac hemolytic anemia is reported.
At 6 years of age heart murmur was detected when she was operated for a left inguinal hernia. Subsequently, she had aortoplasty, ligation and division of patent ductus arteriosus. After a five years' period of healthy life she experienced three attacks of unconsciousness while cycling. With the diagnosis of congenital aortic and supraaortic stenosis, aortic valve commissurotomy and widening of supravalvular stenosis with woven Dacron patch (4×2cm) were carried out on Jan. 21, 1976. After the second operation, anemia developed with increasing severity.
Laboratory data were as follows: RBC 250×104/mm3, Hb 7.3g/dl, reticulocytes 9.0%, elevated serum bilirubin level (especially of indirect form), increased serum free hemoglobin value, and decreased serum haptoglobin level. Peripheral blood film showed many schistocytes. Urine hemosiderin was positive by Prussian Blue staining. With oral administration of iron and folic acid preparation, she recovered almost completely after one year. From such findings, it was thought that the main cause of hemolysis was possibly due to the woven Dacron patch implantation.
A scanning electronmicroscopic picture of the fragmented red blood cells has also been shown.View full abstractDownload PDF (577K) -
Motoko MIKAMI, Toshio SOFUNI1978 Volume 19 Issue 2 Pages 139-146
Published: 1978
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSA 60 year-old male was exposed to the atomic bomb approximately 3 km from the hypocenter at age 30. He has 7 children.
Leukocytosis and the appearance of immature granulocytes were noted at the time of his cylce examination at the Radiation Effects Research Foundation in August 1975. In November 1975, the diagnosis of chronic granulocytic leukemia was made from the bone marrow aspiration which revealed granulocytic hyperplasia (G/E=6/1), and chromosome analysis of the bone marrow cells showed 45, X, -Y, Ph1 karyotype in 100% of the cells examined. At that time, the spleen was palpable 2 finger breadths below the left costal margin. Peripheral blood findings were as follows: RBC, 348×104; Hgb, 10.8 g/dl; Hct, 33.5%; platelets, 295,000; and WBC, 83,500 (differential; Pro 1.5, Myelo 12.5, Meta 11.0, Stab 9.5, Seg 44.5, Ly 10.5, Mono 7.0, Eos 1.5, Baso 2.0). The neutrophile alkaline phosphatase was normal (rate 60, score 260).
The leukocyte count decreased markedly after busulfan administration. 19 months after the diagnosis, his clinical course is satisfactory and there are no findings suggestive of blastic crisis.View full abstractDownload PDF (562K) -
Shigeru YANAGISAWA, Iwao EMURA, Yoshihisa OHNISHI1978 Volume 19 Issue 2 Pages 147-153
Published: 1978
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSAn autopsy case of 74-year-old male with immunoblastic lymphadenopathy is reported. The patient had fever, cough, rapid enlargement of generalized lymph nodes, splenomegaly and hypergammaglobulinemia (IgG, IgA, IgM). A lymph node biopsy was performed and he was treated with vincristine and predonisolone. Though temporary favorable progress, his course was one of progressive deterioration, with death occurring six months after the onset of symptoms.
A biopsy specimen of lymph node showed a morphologic triad: proliferation of arborizing small vessels; prominent immunoblastic proliferations; and amorphous acidophilic interstitial material. At autopsy, generalized lymph nodes showed cellular depletion and a prominent deposit of acidophilic interstitial material. A very small amount of immunoblast were found here and there only in lymph nodes and spleen. Electron microscopic study demonstrated that there were much fibrous long spacing type of collagen fibril in the acidophilic interstitial material.View full abstractDownload PDF (1228K) -
A Case of Preleukemia in Childhood Characterized by a Transient Response to the Oxymetholone TherapyShin-ichiro KURIYA, Kazuo DAN, Yosiki MATSUI, Hideki KUDO, Ken-ichi KA ...1978 Volume 19 Issue 2 Pages 154-159
Published: 1978
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSA 12-year-old boy of preleukemia is reported. He visited to our outpatient clinic with complaints of exertional dyspnea and ecchymoses in August, 1974. On physical examination frank anemia and ecchymoses were found. Initial blood study revealed a Hb of 5.5g/dl, hematocrit of 18%, erythrocytes of 161×104/mm3, reticulocytes of 1.1%, platelets of 2.7×104/mm3, and leukocytes of 3,700/mm3 with neutropenia. Moderate anisopoikilocytosis was shown on the blood smear. The bone marrow was hyperplastic and characterized by maturation arrest of the myeloid series and bizarre nuclei of erythroblasts. The oxymetholone therapy, 60mg daily, was given to result in nearly normal hematologic findings in five months, and was discontinued in August, 1975. A mild anemia, moderate to severe leukopenia and thrombocytopenia again developed in Feburary, 1976, which showed only an inadequate response to the oxymetholone therapy. In October of the same year, myeloblasts began to increase in the bone marrow and were occasionally detected in the peripheral blood. A cytogenetic study at this stage revealed hyperdiploidy in 94% of mitotic figures examined, the mode of chromosome number being 50. Subsequently, the patient took a rapid downhill course, and died of sepsis due to E. coli in December, 1976.View full abstractDownload PDF (661K) -
Yoshikiyo YAMAZAKI, Haruo TAKESHITA, Hyonosuke IZUMI, Fumio KONISHI1978 Volume 19 Issue 2 Pages 160-165
Published: 1978
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSA man aged 52, was first investigated in February 1975, when he was given a two-months history of exertional dyspnea and pallor. He had pancytopenia, and a diagnosis of aplastic anemia was made by aspirated bone marrow; this was assumed to be idiopathic in the absence of any history of exposure to toxic drugs or chemicals. He had a negative Ham's test. Six months later, Ham's test became positive, but the urine contained neither hemosiderin nor hemoglobin; in this stage paroxysmal nocturnal hemoglobinuria was only a laboratory phenomenon. In February 1977, he passed dark urine containing hemosiderin and hemoglobin, and the diagnosis of paroxysmal nocturnal hemoglobinuria was established. The change in neutrophil alkaline phosphatase score was from high values in the stage of aplastic anemia to low ones in the PNH stage.View full abstractDownload PDF (589K) -
Ryuzo OHNO, Ryuzo UEDA, Hisamitsu SUZUKI, Masashi TANAKA, Kohji EZAKI, ...1978 Volume 19 Issue 2 Pages 166-171
Published: 1978
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSThirty eight years old male with hematologically typical CML with low LAP but without Ph1 chromosome developed blast crisis 4 years after the diagnosis. The blasts were regarded as lymphoid by May-Giemsa stain, phase-contrast microscopy, electron microscopy, supravital stain for nucleoli with brilliant cresyl blue, and histochemical stains including peroxidase, Sudan black B, β-glucuronidase, PAS, naphthol-AS-esterase and acid phosphatase.
EAC rosett was positive in 85% of blasts and surface Ig was positive in 31%. The activity of terminal deoxyribonucleotidyl transferase was very high (11.67 nmole/108 cells).
Therefore this seems to be a case of blast crisis of CML with lymphoid cells possessing B-cell markers.View full abstractDownload PDF (633K) -
Tadashi KOIKE, Sohji MIURA, Yoshio TAKEMOTO, Michiyoshi SUGAWARA, Tets ...1978 Volume 19 Issue 2 Pages 172-179
Published: 1978
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSTwo cases of angioimmunoblastic lymphadenopathy with dysproteinemia are reported.
The first case, 68-year-old man, presented with fever and generalized lymphadenopathy. The second case, 66-year-old man, presented with fever and dry cough. Both cases showed generalized lymphadenopathy, hapatosplenomegaly, plasmacytoid cells in peripheral blood and polyclonal hypergamma-globulinemia. Lymph nodes biopsies revealed loss of normal architecture, a prominent proliferation of immunoblast, abundunce of arborizing small vessels, and the deposit of an amorphous acidophilic interstitial material. The second case showed hypersensitivity to penicillin and suffered with fatal interstitial pneumonitis, which was thought to be pulmonary manifestation of this disease.View full abstractDownload PDF (1040K) -
Fumihiro SAWA, Noriaki SHINOMIYA, Sakiko MARUYAMA, Toshiro NAKAGAWA, I ...1978 Volume 19 Issue 2 Pages 180-186
Published: 1978
Released on J-STAGE: January 19, 2009
JOURNAL RESTRICTED ACCESSLeukemia and aplastic anemia are each distinguishable clinical entities, but a small percent of leukemic patients shows the same clinical and laboratory findings as aplastic anemia at the beginning of the illness, so it is very important to discuss the relationship of these two diseases.
We observed patients with aplastic anemia and acute leukemia in two of the three siblings.
The elder sister was diagnosed as having idiopathic aplastic anemia at the age of 1 year and 11 months. The patient died of an intracranial hemorrhage due to a traffic accident 2 years and 7 months after the diagnosis. No sign suggesting a preleukemic stage was found. The patient had three episodes of intussusceptions during her disease, but they were treated successfully with high pressure enema.
The younger brother was diagnosed as having acute leukoblastic leukemia at the age of 7 months. Complete remission was induced in the patient with VAMP therapy, but the patient died of bone marrow relapse, meningeal infiltration, intracranial hemorrhage and sepsis 1 year after the diagnosis.
Neither blood diseases nor malignant neoplasmas were found in the family of these siblings. The family members had normal karyotype according to chromosome studies. Their mother had been exposed to the atomic bomb radiation in Hiroshima 2,000 meters from the hypocenter at 4 years of age.View full abstractDownload PDF (566K)
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