臨床血液
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
14 巻, Suppl. 号
選択された号の論文の26件中1~26を表示しています
臨床研究
  • 河北 靖夫, 片山 則孝, 牧野 卓麿, 荒木 弘一
    1973 年 14 巻 Suppl. 号 p. S1247-S1254
    発行日: 1973年
    公開日: 2008/10/31
    ジャーナル 認証あり
  • 市丸 道人
    1973 年 14 巻 Suppl. 号 p. S1255-S1263
    発行日: 1973年
    公開日: 2008/10/31
    ジャーナル 認証あり
    Several cases of myeloproliferative disorders in atomic bomb survivors were presented and the disturbances of hematopoietic organs, especially bone marrow, due to atomic bomb irradiation were discussed.
    1. One of the acute radiation effects was severe hypoplasia of bone marrow, which seemed to have either recovered soon or resulted in death within few months.
    2. Various myeloproliferative disorders were recognized as late effect of atomic bomb irradiation after some latent period. The occurrence of leukemia was most remarkable. Besides leukemia, myeloproliferative disorders having proliferative disturbances of erythropoietic series or megakaryopoietic series were also seen in proximal survivors in Hiroshima and Nagasaki. Myelofibrosis was also seen.
    3. Following the recovery from hypoplastic marrow as acute radiation effect, there may possibly be no increase in the risk of aplastic anemia in atomic bomb survivors.
    Myeloproliferative disorders sometimes showed peripheral blood picture like aplastic anemia.
  • —特にsinocapillaropathyを中心に—
    梅原 千治, 八木 奎司, 山内 保人, 黒羽根 生自, 上山 隆也, 伊藤 久雄, 酒井 信彦, 平嶺 信郎
    1973 年 14 巻 Suppl. 号 p. S1264-S1280
    発行日: 1973年
    公開日: 2008/10/31
    ジャーナル 認証あり
    From the results of the studies on the histological findings of the bone marrow which was obtained by biopsy from patients with aplastic anemia, it was clarified that the most characteristic change of the bone marrow of the patients was exudation which was supposed to be induced by a serous inflammation.
    In order to prove whether aplastic bone marrow could be induced by serous inflammation or not, an experiment using microwave radiation was made on the rabbits. By this experiment, it was proved that after radiating the femur of rabbits, inflammatory changes with remarkable exudation occured and thereafter completely aplastic bone marrow, which had quite a resemblance to that of human aplastic anemia, developed.
    From the results of the clinical and experimental studies, it was concluded that aplastic anemia is caused by a sinocapillaropathy or inflammation of the bone marrow. It was also our considered opinion that by inducing the thought of sinocapillaropathy, which caused disorders of the transport unit between the sinus and the hemocytoblasts, almosc all of the unsolved questions about aplastic anemia could be solved without thinking of stem cell failure.
  • 石川 昭, 山田 耕一郎, 新井 洋子, 坂本 正子, 富田 純正
    1973 年 14 巻 Suppl. 号 p. S1281-S1287
    発行日: 1973年
    公開日: 2008/10/31
    ジャーナル 認証あり
    Studies were performed on the erythropoietin in patients with aplastic anemia and acute leukemia in childhood, and following results were obtained.
    1. The reciprocal relationship was shown between the level of urinary erythropoietin and hemoglobin concentration in peripheral blood showing elevated activity as hemoglobin decreased below 10 gm. per 100 ml. No increased activity of erythropoietin was seen with hemoglobin of above 10 gm. per 100 ml. except cases treated with testosterone.
    2. The level of erythropoietin revealed to be lowered as the erythroblastic distribution in bone marrow increased. However, no increased erythropoietin was seen in some cases with decreased erythroblastic compartment.
    3 In aplastic anemia with hemoglobin of below 10 gm per 100 ml, more elevated urinary erythropoietin activity was observed by adding normal sera of healthy human adult than that of non-treated anemic urine. No such a finding was seen in patients with aplastic anemia of hemoglobin above 10 gm. per 100 ml and also in leukemic patients regardless the hemoglobin concentration.
  • 長谷川 弥人, 外山 圭助, 安藤 泰彦, 小川 哲平, 陳 田柏, 渡辺 清明, 藤山 順豊, 中沢 堅次, 高橋 隆一
    1973 年 14 巻 Suppl. 号 p. S1288-S1293
    発行日: 1973年
    公開日: 2008/10/31
    ジャーナル 認証あり
    It has been still controversial that idiopathic acquired aplastic anemia is a definite clinical entity.
    Analysis of 51 cases with idiopathic aplastic anemia for the period of 12 years between 1962 and 1972 in the Keio University Hospital led us to conclude that aplastic anemia consists of two types: one is genuine (typical) aplastic anemia, the other aplastic anemia syndrome (atypical aplastic anemia). The diagnosis of genuine aplastic anemia should be made at the presence of pancytopenia with relative lymphocytosis, a-(hypo-)plastic bone marrow picture and elevated serum iron, One should make a diagnosis of aplastic anemia syndrome when found any two of following itemes: 1) reticulocytosis. 2) absence of relative lymphocytosis 3) monocytosis 4) erythroblasts in peripheral blood. 5) erythroid hyperplasia of bone marrow prior to treatment 6) left shift of myeloid series in bone marrow. 7) normal level of serum iron 8) splenomegaly.
    Aplastic anemia syndrome can terminate in acute myelogenous leukemia, paroxysmal nocturnal hemoglobinemia (PNH) or aplastic anemia PNH syndrome. A part of the syndrome may be curable.
    Thus genuine aplastic anemia is a definite clinical entity and should be strictly separated from aplastic anemia syndrome.
綜説
臨床研究
  • —特にSideroblastic anemia, 鉛中毒,Erythropoietic protoporphyriaを中心にして—
    高久 史麿, 青木 洋祐, 浦田 郡平
    1973 年 14 巻 Suppl. 号 p. S1303-S1310
    発行日: 1973年
    公開日: 2008/10/31
    ジャーナル 認証あり
    A new specific and simple method for the determination of δ-aminolevulinic acid (ALA) synthetase activity in human bone marrow erythroblasts has been developed in our laboratory. In this method, ALA synthetase of erythroblasts was partially purified by sonicating the mitochondria fraction and extracting into deoxycholate. By this extraction procedure, almost all of the activities of succinyl-CoA hydrolase, α-ketoglutarate dehydrogenase, succinyl-CoA synthetase have been eliminated. It has then become possible to use the 14C-succinyl CoA-a direct pecursor of ALA formation-as the substrate for the assay of ALA synthetase. ALA formation from the 14C-succinyl CoA and glycine in the partially purified enzyme has been isolated by column chromatography and its radioactivity was counted with a liquid scintillation counter. By this method, the whole procedures for the assay have been completed within 4 hours.
    Moderate to marked decrease in the ALA synthetase activity has been demonstrated in all 19 cases of primary sideroblastic anemia. In the sideroblastic anemia patients with markedly decreased ALA synthetase activity were included a patient of congenital type and a patient who responded to pyridoxine treatment. In another case responsive to pyridoxine treatment, the enzyme activity was decreased moderately to one half of normal. In these patients who responded hematologically to pyridoxine treatment, the ALA synthetase activity was increased after the treatment with pyridoxine. Among the patients who had been exposed to lead, 5 patients showed an increase in the ALA synthetase activity in bone marrow erythroblasts. Although 2 patients showed normal activity, these patients had been supposed to be isolated from the exposure to lead for a certain period of time. The ALA synthetase activity was also studied in 4 cases of erythropoietic protoporphyria. In 2 cases occurring in one family, the enzyme activity was normal, while in other 2 cases, the enzyme activity was apparently increased. In one patient with increased enzyme activity, however, the activity became normal at the stage of clinical remission.
    Significance of these findings of the ALA synthetase activity in erythroblasts of these patients was discussed in relation to the pathophysiology of these hematological disorders.
  • 原 宏, 金丸 昭久, 浜野 照明, 永井 清保
    1973 年 14 巻 Suppl. 号 p. S1311-S1324
    発行日: 1973年
    公開日: 2008/10/31
    ジャーナル 認証あり
    In an attempt to clarify the pathogenesis of pancytopenia, the erythroblast kinetics and the colony formation were studied both experimentally and clinically.
    First, erythroblast kinetics in controls and bled rabbits were investigated by using autoradiography and stathmokinetics on each erythroblast compartment which had been classified by the measurement of nuclear diameter. These results showed that the generation times of mitotable cell compartments I, II and III were invariable in both groups but the compartment transit time (CTT) of non mitotable cell compartment IV was shortened in the bled group.
    This conclusion was also confirmed from the in vitro autoradiography of erythroblasts in the patients with hemolytic anemias.
    It has been generally accepted that erythropoietin is over-produced in bleeding and hemolytic anemias in response to the decrease in erythrocyte count in peripheral blood. Though erythropoietin has been believed to differentiate the stem cell into proerythroblast, our data-indicated that it showed an additional action i.e. the shortening of CTT of the compartment IV. It has been reported that erythropoietin activity was high in aplastic anemia. However, the present work revealed the increase in relative size of the compartment IV, namely, the prolongation of its CTT. From this discrepancy drove us it seemed to be speculate of that erythropoietin might not act on the bone marrow in aplastic anemia.
    In order to solve this problem, the colony stimulating activity (CSA) which is one of serum hemopoietic factors and acts in promoting the granulocytic colony formation in vitro was measured in sera from healthy controls and aplastic patients, using the marrow cells of C3H mice. The results showed the high level of CSA in the majority of aplastic anemia.
    From these findings the following hypothesis on the pathogenesis of pancytopenia may be postulated.
    In aplastic anemia, the humoral hemopoietic factors such as erythropoietin, CSA and others, although increased in the serum, are precluded from entering bone marrow tissues, possibly due to disturbances in the permeability of vessel wall or sinusoid endothelium.
  • 三輪 史朗
    1973 年 14 巻 Suppl. 号 p. S1325-S1330
    発行日: 1973年
    公開日: 2008/10/31
    ジャーナル 認証あり
    Results of clinical, hematological and erythrocyte enzyme studies on 19 cases in 15 families with pyruvate kinase deficiency are presented. The study revealed that there are several different PK variants. In two cases, marked reticulocytosis associated with decreased red cell life span became apparent after splenectomy. The implication of this together with mechanisms of hemolysis in PK deficiency is discussed.
  • —その精度に関する検討—
    桝屋 富一, 宮城 研, 坂田 貞利
    1973 年 14 巻 Suppl. 号 p. S1331-S1340
    発行日: 1973年
    公開日: 2008/10/31
    ジャーナル 認証あり
    The acute attacks of porphyrias (especially in cases of acute intermittent porphyria, variegate porphyria and hepatic coproporphyria) are very often iatrogenic. The porphyrias should be suspected as one of the differential diagnosis before the unnecessary laparotomy or the administration of barbiturates for the patients with neuropathy of unknown etiology.
    In the diagnosis of porphyrias, the quantitation of porphyrins and their precursors is one of the most important diagnostic procedures. This is, however, often laborious and time consuming. Fortunately, there is an increased excretion of porphobilinogen in the urine during the acute attack and this compound can be identified by a simple screening test of Watson-Schwartz. The problem is how to find out the other patients while they are still in the latent stage. The laborious quantitative methods are not appropriate for the epidemiological and genetic investigations which deal with large number of materials, and thus it becomes very important to establish a simple and convenient screening test for porphyrias. For these reasons, the new simple filter paper method has been designed.
    Methods are as follows. For the urine, one drop of the urine is applied at the center of two pieces of filter paper, one drop on each. The papers are examined under the ultra-violet light for the pink fluorescence of porphyrins. If this is present, the papers are dried and the area of pink or red fluorescence is traced by using a soft pencil. Then, another filter paper is placed under each sample-bearing paper closely. One drop of 0.5% HCl is added in the fluorescing circle on the one piece and 5% HCl is added in the same manner on the other. If the pink or red fluorescence exceeds the circle marked with pencil and penetrates to the second paper placed below, the urine is positive for the excessive porphyrin content.
    For the fecal analysis, a small amount of feces (less than a grain of rice size or about 0.04 g wet weight) is applied to the center of two pieces of filter paper. One drop of ether: acetic acid mixture (ethyl ether 5 volumes and glacial acetic acid 1 volume) is added and the papers are examined under the ultra-violet light for the fluorescence. If this is detected, the papers are dried and traced with pencil along the edge of the fluorescent area. Then, one drop of 0.5% HCl is added on the one and 5% HCl is added on the other in the fluorescent circle. The definition for the positive results is the same as mentioned for the urine.
    All the porphyrias can be detected with this method, except acute intermittent porphyria. In this instance, the excretion of porphyrins is not so much, especially in remission, and the test is often negative. However, Watson-Schwartz test is positive in most instances, even in remission. The filter paper method proves its importance in cases of variegate porphyria and hereditary coproporphyria. Watson-Schwartz test shows negative reaction during their remission, while our filterpaper method shows positive results because of the increased copro-and protoporphyrin in their excreta.
    As for the false positive reaction, a pink fluorescence is sometimes seen before the addition of HCl in the feces of a person taking a large amount of green vegetables or chlorophyll derivatives and cases with occult bleeding. However, these false positive reactions are excluded by the addition of 0.5% and 5% HCl. With the addition of HCl, these compounds are not extracted and thus do not move across the circle marked by a pencil on the filter paper, nor do they penetrate to the paper placed below.
  • —芽球の電顕的観察—
    赤羽 太郎, 江口 光興, 小宮山 淳, 花村 潔, 塚田 昌滋
    1973 年 14 巻 Suppl. 号 p. S1341-S1346
    発行日: 1973年
    公開日: 2008/10/31
    ジャーナル 認証あり
    The electron microscopic and ultvastructural studies on the blasts of the bone marrow were performed in 11 children with acute blastic leukemia, and the morphological comparison of the blasts in children with prolonged first remission and early relapse was investigated. The fine structure of the blasts at admission and at early relapse was also compared.
    1) The patients with prolonged first remission consisted of 4 children, whose durations of first remission were more than 4 years and 3 months, more than 3 years and 9 months, 3 years and 3 months, and 2 years and 2 months, while the patients with early relapse consisted of 7 children, whose durations prior to early relapse were 4 to 9 months, 6 months in average.
    2) The fine structure and cytochemistry of the blasts in patients with prolonged first remission and early relapse suggested the different tendency. Also it was suggested that more matured blasts electron microscopically appeared at early relapse.
  • 内野 治人, 田中 信夫, 大前 清甫
    1973 年 14 巻 Suppl. 号 p. S1347-S1353
    発行日: 1973年
    公開日: 2008/10/31
    ジャーナル 認証あり
    Since the early stage of vitamin B12 investigations in clinical field arround 1950, it has been well known that hypercobalaminemia was one of the characteristics in human leukemias, especially in chronic granulocytic leukemia without any reasonable explanations as to its mechanism. This study was undertaken to elucidate the mechanism of hypercobalaminemia in leukemia, from the view-point of B12 values in sera, leukocytes and urine as well as the identification of four B12 forms of derivatives, that is, Cyanocobalamin (CN-B12), Hydroxocobalamin (OH-B12), DBCC and Methylcobalamin (CH3-B12). Hypercobalaminemia in plasma was found to be mostly significant in cases of chronic granulocytic leukemia, but high B12 contents in leukocytes were found to be significant in cases of acute granulocytic leukemia. High serum B12 contents were corrected following the decrease of leukocytes counts after the administration of the antileukemic agents, but were not influenced by the spontaneous increase or decrease of leukocytes counts without any drugs administration. Following the drug administration, high urinary excretion of B12 which occurred intermittently was found in course of leukemia, especially of chronic granulocytic leukemia. As to the percent distribution of CN-B12, OH-B12, DBCC and CH3-B12, it was found that approximately a half of B12 derivatives was CH-B12 in sera, leukocytes and urine of chronic granulocytic leukemia as well as of normals and it was concluded that the increased B12 contents in blood and urine of leukemia were not due to the increase of one specialized form of B12 derivatives. The percent distribution of four B12 derivatives which bound to the increased Transcobalamin I in chronic granulocytic leukemia were also found to be similar to the patterns of whole plasma, demonstrating predominantly in CH3-B12 contents, while the B12 derivatives pattern in Transcobalamin II were found to be the same percent distribution in three derivatives of CH3-B12, DBCC and OH-B12 except CN-B12 fraction. From the obtained results which are described above, the discussion is extended to the possible mechanism of hypercobalaminemia and hypercobalaminuria in leukemic patients in terms of the granulocytes turnover and destruction.
  • 栗田 宗次, 小川 一誠, 亀井 良孝, 村上 稔, 尾山 淳, 太田 和雄, 須知 泰山
    1973 年 14 巻 Suppl. 号 p. S1354-S1358
    発行日: 1973年
    公開日: 2008/10/31
    ジャーナル 認証あり
    Out of 58 patients with reticulum cell sarcoma leukemic manifestation, leukemic reticulosarcomatosis, developed in 6 cases (10.3%), following the criteria for leukemic manifestation consisted of differential counts of white blood cells with 30 per cent or more sarcoma cells. Six patients developed to luekemic manifestation had advanced stages at first visit to hospital, had small sarcoma cells, and had the lymphadenogram of less than 60 per cent of sarcoma cells in lymph glands at initial diagnosis. At the leukemic phase sarcoma cells were found in aspirated smears of bone marrows in all cases, and hepato-splenomegalies were found frequently. Their survivals from initial symptoms were less than 6 months in 3 patients and less than 16 months in all patients. Therefore, it was suggested that leukemic reticulosarcomatosis might be a subgroup of reticulum cell sarcoma prone to leukemic manifestation and with the rapid course of disease.
  • 芳賀 圭五
    1973 年 14 巻 Suppl. 号 p. S1359-S1364
    発行日: 1973年
    公開日: 2008/10/31
    ジャーナル 認証あり
    Essential thrombocythemia is a rare blood disorder characterized by the persistent increase in blood thrombocytes without any demonstrable cause and frequently accompanied by bleeding symptoms.
    The present report deals with two cases, both 40 years old male, with prominent increase in thrombocytes, namely, from 600,000 to 3,700,000/cmm., with slight leukocytosis and scattered immature myeloid cells. Platelet adhesiveness and ADP aggregation were impaired. In one of them serum potassium was remarkably high. Its etiology was discussed.
  • 山崎 博男, 高橋 武男, 間下 信昭
    1973 年 14 巻 Suppl. 号 p. S1365-S1371
    発行日: 1973年
    公開日: 2008/10/31
    ジャーナル 認証あり
    Platelet aggregability by ADP and adrenaline was measured in 142 clinical cases including 48 healthy volunteers and 25 patients in acute stage of thrombosis. We measured not only appearance rate of primary and secondary aggregation but also intensity of aggregation which was expressed as the percentage of the maximum deflection of optical density of platelet rich plasma against optical density of platelet free plasma obtained from the sample. In healthy group, there was no correlation between age and platelet aggregability. Females showed higher aggregability than males, however the difference was not significant. There was no difference in appearance rate of primary and secondary aggregation and intensity of primary aggregation between the diseased and healthy group. On the one hand, the intensity of secondary aggregation of the patients in acute stage of thrombosis was higher than that of the healthy group with a statistical significance (P<0.01∼0.05). Especially the difference was marked in the response induced by adrenaline. Such enhancement was not observed in the groups of recovery stage of thrombosis, hypertension and other miscellaneous diseases. Nine cases of angina pectoris showed a higher secondary platelet aggregation induced by 1 μg/ml of adrenaline than the healthy group with a statistical significance (P<0.05).
  • 東 音高
    1973 年 14 巻 Suppl. 号 p. S1372-S1374
    発行日: 1973年
    公開日: 2008/10/31
    ジャーナル 認証あり
    The normal human platelets can be aggregated by hydrogen peroxide (H2O2) in vitro.
    This H2O2-induced platelet aggregation was inhibited by adenosine, suggesting that the final pathway was ADP.
    There are certain differences, however, between ADP-induced and H2O2-induced platelet aggregation with respect to the effects of temperature and metabolic inhibitors, such as KCN, NaN3, antimycin and oligomycin.
    The H2O2-induced platelet aggregation could be inhibited by vitamin E derivatives, indicating the potential value of vitamin E derivatives for the prevention of occlusive vascular disease.
  • —自験例を中心とした考察—
    森 和夫, 平塚 巖, 酒井 秀章, 樋渡 克英, 高橋 恒男, 丸浜 喜亮, 我妻 和夫, 山形 敞一
    1973 年 14 巻 Suppl. 号 p. S1375-S1384
    発行日: 1973年
    公開日: 2008/10/31
    ジャーナル 認証あり
    A 21 year old girl of systemic Weber-Christian disease associated with consumption coagulopathy was reported.
    Hemorrhagic episodes of this case began after 3 month of the onset of Weber-Christian disease complicating with petechie, ecchymosis, nosebleeding, hematuria and melena.
    General laboratory examinations revealed hyperlipemia, abnormal liver function tests and delayed blood sedimentation rate during severe clinical stage. Examination on clotting factors showed a decreased level of fibrinogen and other clotting factors which were due to consumption coagulopathy and secondary liver function disturbance.
    Complex pathophysiology concerning the hemorrhagic diathesis associated with Weber-Christian disease were discussed from various aspects of blood coagulation mechanisms involving abnormal fat metabolism and vascular lesion.
  • 椙江 勇, 菱川 要子
    1973 年 14 巻 Suppl. 号 p. S1385-S1392
    発行日: 1973年
    公開日: 2008/10/31
    ジャーナル 認証あり
    The effects of t-AMCHA (t-4-aminomethylcyclohexane-1-carboxylic acid) on fibrinogen turnover rate were studied in rabbit. After administration of rabbit fibrinogen-131I, radioactivity of fibrinogen and plasma were measured by well-type scintillation counter for 1 week and during the same period, hemostatic activity by thrombelastography, fibrinogen level in plasma and fibrinolytic activity by euglobulin lysis time and fibrin plate were measured.
    The results obtained are as follows:
    1) In the control group of 8 rabbits the half time of fibrinogen turnover measured by fibrinogen was 48 hours (2.0 days). But half time measured by plasma was 72 hrs. (3.0 days). Hemostatic and fibrinolytic activity and fibrinogen level were not changed significantly for a week.
    2) In the t-AMCHA group of 6 rabbits after administration of t-AMCHA, fibrinolytic activity were dominantly decreased and elasticity of clot were slightly increased but fibrinogen level were not changed. The half time of t-AMCHA group was 2.3 days by fibrinogen and 3.7 days by plasma and these results were not significantly changed compared with 2.8 days and 4.3 days obtained from the results before administration in the same rabbits.
    3) However, in the two cases of t-AMCHA group which showed rapid half time before administration (1.42 days and 1.50 days), half time were almost twice (2.57 days and 2.85 days) after administration.
    From these results, the catabolic pathway of fibrinogen concerned with fibrinolysis were discussed in this paper.
  • 福武 勝博, 藤巻 道男, 浮田 実, 北原 武
    1973 年 14 巻 Suppl. 号 p. S1393-S1399
    発行日: 1973年
    公開日: 2008/10/31
    ジャーナル 認証あり
    One of five patients in a family with hemophilia A developed an inhibitor to factor VIII, being of IgG-antibody in nature. And it has been demonstrated that this inhibitor might have an inhibitory effect on platelet aggregation observed by recalcification method. Patientd eveloped an inhibitor has been successfully managed with Reptilase treatment.
症例
  • 浅井 一太郎, 山口 潜, 木本 元治, 武藤 良知, 塚田 理康
    1973 年 14 巻 Suppl. 号 p. S1400-S1403
    発行日: 1973年
    公開日: 2008/10/31
    ジャーナル 認証あり
    Four cases of acquired primary sideroblastic anemia reported in this paper had in common a typical manifestation of many ringed sideroblasts in the bone marrow, ranging between 56 and 80 per cent of all erythroblasts. Many iron granules were noted not only in the mature erythroblasts, but in the perinuclear area of the early erythroblasts.
    Ferrokinetic study assayed by 59Fe revealed no uniform pattern. Evidence of slight hemolysis was noted in two of the four patients. No remarkable change in the porphyrin metabolism was detected. Examinations for abnormal hemoglobin were within normal limits.
    All patients have received pyridoxin by mouth for at least four months without considerable effect.
  • 岩崎 一郎, 長谷川 真, 岩藤 孫六, 春藤 哲正
    1973 年 14 巻 Suppl. 号 p. S1404-S1408
    発行日: 1973年
    公開日: 2008/10/31
    ジャーナル 認証あり
    A 24 year-old female having a history of anemia for a year without obvious symptom of SLE was admitted for exact examination of anemia. Urine showed urobilinogen (++) and bilirubin (−). Blood examination showed RBC 234×104, Hb 60%, reticulocytes 12‰ and erythroid hyperplasia of the bone marrow. Serum indirect bilirubin was 1.33 mg/dl. ANF, LE cell and Wasserman reaction were positive. Red cell life span was slightly shortened (51Cr—23.4 days DF32P—88 days). Ferrokinetic study showed high serum iron level 198 μg/dl, short plasma iron disappearance time (PIDT) (27 min.), increased plasma iron turnover rate (PIT) (38.4 mg/kg/day) and low red cell utilization (26.5%). Bone marrow uptake was high but was kept high level throughout 3 weeks. The rise of spleen and liver uptake was not seen. This case was diagnosed SLE with anemia due to ineffective erythropoiesis from these findings.
    Several factors including red cell destruction and suppression of erythropoiesis is assumed to take part in the genesis of anemia of SLE but the genesis is not yet elucidated in detail. This case of SLE whose anemia was proved to be due to ineffective erythropoiesis was the first report.
  • 西村 昂三, 五十嵐 正宣, 渡辺 昭彦, 弘岡 順子, 南 秀樹, 設楽 利二, 吉田 滋彦, 細谷 亮太
    1973 年 14 巻 Suppl. 号 p. S1409-S1414
    発行日: 1973年
    公開日: 2008/10/31
    ジャーナル 認証あり
    Three cases of Down's syndrome with congenital leukemia or dysgranulopoiesis are reported.
    The first case died at 34 hours of age probably because of widespread intestinal stenosis. Autopsy revealed clusters of abnormal cells only in the bone marrow, but no leukemic infiltration in other organs. The second case is still alive showing variable numbers of blasts in the peripheral blood. The third case died at the age of 6 years 7 months because of pneumonia. Autopsy could not reveal any leukemic change.
    It is not certain, at present, whether these conditions are true leukemia or non-leukemic dysgranulopoiesis. Collection, close observation, recording and analysis of such cases are mandatory to clarify the nature of this condition and may help to solve the etiology of childhood leukemia.
  • —自験例と本邦報告例の検討—
    重田 英夫, 山口 覚太郎, 米満 博, 武田 従信, 奥田 邦雄
    1973 年 14 巻 Suppl. 号 p. S1415-S1425
    発行日: 1973年
    公開日: 2008/10/31
    ジャーナル 認証あり
    A case of the “juvenile” type of chronic myelogenous leukemia seen in a 6 months old male has been described. A review of 21 cases of this condition including the present case in the Japanese literature has been made together with analyses of the clinical features, therapeutic effects and the changes in basophils in peripheral blood, serum vitamin B12, and immunoglobulins in chronic myelogenous leukemia with negative Ph1. The following summary may be made:
    The ages of the reported cases range from 2 months to 9 years, and the survival time is longer in patients with earlier onset. The effect of therapy on the survival time is less conspicuous compared with the adult type. The chronic myelogenous leukemia with negative Ph1 lacks the characteristic features of that with positive Ph1 such as basophila and high serum B12, and exhibits a different immunoglobulin pattern. In this respect, the “juvenile” type of chronic myelogenous leukemia is in a position closer to acute myelogenous leukemia. Whether or not juvenile chronic myelogenous leukemia is a separate disease entity, depends on the extent of increase in HbF and on whether or not it is of congenital metabolic derangement.
  • 日野 志郎, 福井 圭子
    1973 年 14 巻 Suppl. 号 p. S1426-S1433
    発行日: 1973年
    公開日: 2008/10/31
    ジャーナル 認証あり
    A 59-year-old female office-clerk was admitted to the hospital in March, 1968, for evaluation of a moderate splenomegaly accidentally recognized in an X-ray mass examination for gastric cancer. The final clinical diagnosis was myelofibrosis with myeloid metaplasia because of progressive hepatosplenomegaly and weight loss, leukoerythroblastosis, teardrop poikilocytosis, abnormal platelets, dry taps on the bone marrow aspiration, and later X-ray changes of bone. The Philadelphia chromosome was negative and the leukocyte alkaline phosphatase was normal.
    On admission the patient had no respiratory distress, and physical and roentgenologic examinations of the chest were normal. Eight months later nonproductive cough developed and the chest X-ray film taken in March, 1969, revealed a right hilar fullness and diffuse mottled densities in the right middle lung field, which made a slow and steady progression to the right upper lobe and left lung (Fig. 1-4). She died three years and nine months after the first recognition of splenomegaly.
    Autopsy confirmed the clinical diagnosis. The pulmonary changes, most prominent in the right upper and middle lobes (Fig. 7), consisted of marked fibrosis and hematopoiesis including a small amount of megakaryocytes and erythroid series (Fig. 10), which had presumably progressed along the blood vessels from the hili (Fig. 8 and 9). The extramedullary hematopoiesis with fibrosis also affected the pleurae, pericardium and peritoneum. In addition, lymph nodes and subcutaneous tissues of the anterior chest and abdomen were involved, though trivially.
    In the literature there are a few cases with perivascular, peribronchial or interstitial involvement of the lung, 3, 4, 7, 8, 15, 17, 20) which, however, were described only anatomically and assumed to have revealed no such marked clinical manifestations as in this case.
  • 栗栖 明
    1973 年 14 巻 Suppl. 号 p. S1434-S1439
    発行日: 1973年
    公開日: 2008/10/31
    ジャーナル 認証あり
    A 50-year-old man, shop-keeper, who had had myocardial infarction 10 years since, was hospitalized with complaint of slight intermittent cerebellar symptoms several years long. The clinical pathological examinations identified a hen's egg large hemangioblastoma of cerebellum with erythrocytosis.
    The tumor was inoperable, then 60Co radiation therapy of 5480 rads per 47 days for the treatment of the tumor was performed after setting of ventriculo-sinus shunt for the intracranial depressing.
    Erythrocytosis was improved soon after the setting of the shunt and was getting better within normal range. No relapse were observed for 20 months long untill today with the exception of a transit erythrocytosis in the 3rd week after the completion of radiation therapy.
    It suggested that certain dose of irradiation to the tumor at an earlier time during radiation therapy might induce some erythropoietin active substances from the tumor tissue, although no erythropoietin assay of the tumor tissue or the patient's plasma were examined. Increased intracranial pressure and characteristic angiographic finding of the tumor similar to the appearance of arterio-venous shunt might be also one of the causative factors of erythrocytosis as well as erythropoietin active substances, respectively.
    The patient was discharged after about 5 months of hospitalization and rehabilitated. It was recommended that cross-examination of papilledema and short-life radioisotope scitigramm of the brain may be useful as a screening method for the tumors of cerebellum in the cases with erythrocytosis.
  • —本邦第一例の報告—
    外山 圭助, 藤山 順豊, 菊池 正夫, 陳 田柏, 長谷川 弥人, 植草 実, 三村 孝, 熊井 浩一郎
    1973 年 14 巻 Suppl. 号 p. S1440-S1446
    発行日: 1973年
    公開日: 2008/10/31
    ジャーナル 認証あり
    A 56-year old female complaining of abdominal distention was first seen on Dec. 1971. She had an abdominal tumor the size of a 28-week pregnant uterus. There was no splenomegaly. Plain film of the abdomen disclosed gall stones. Intravenous pyelogram showed displacement of the ureters and absence of hydronephrosis. The hemoglobin was 21.3 g/dl, Ht 65%, RBC 630×104/mm3; WBC, differential count and platelet count were normal. Blood gas analysis was within normal limits. Red cell mass was 58 ml/kg. On Jan. 24, 1972, total hysterectomy and cholecystectomy were performed, resulting in disappearance of erythrocytosis. The histology of extirpated uterus was leiomyoma. On follow-up examinations for over 19 months, the hematologic values remained normal. RBC mass was found to be normal (24 ml/kg) 5 months after operation. The erythropoietin (ESF) activity estimated by the starved rat assay was significantly elevated in the tumor extract of this patient in comparison with the tumor extract of the patient unassociated with erythrocytosis. Slightly increased ESF activity in the serum was inclined to decrease after operation. It is suggested that erythrocytosis in this case was caused by the production of ESF or ESF like substance in the tumor.
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