臨床血液
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
29 巻, 7 号
選択された号の論文の29件中1~29を表示しています
第29回総会
シンポジウム3.
慢性骨髄増殖性症候群―各疾患相互の関係と位置づけ―
  • 米満 博
    1988 年 29 巻 7 号 p. 950-959
    発行日: 1988年
    公開日: 2009/02/02
    ジャーナル 認証あり
    One hundred and seventeen patients with chronic myeloproliferative syndrome, consisting of 40 patients with chronic myelogenous leukemia (CML), 46 patients with polycythemia vera (PV), 17 patients with primary myelofibrosis (PMF), and 14 patients with essential thrombocythemia (ET) have been observed between 1970 and 1982.
    The results of the repeated hematological investigation were: (1) at diagnosis, significant difference were noted in hematologic and chemical data among these diseases. However, some cases were difficult to diagnosis. (2) Median survival times of the patients with CML, PV, and PMF were 35 months, 135 months, and 70 months, respectively. (3) The incidences of acute leukemic transformation in CML, PV, and PMF were 89.6% (26/29), 23.8% (5/21), and 18.8% (3/16), respectively. Moreover, one patient with ET transformed into acute leukemia. (4) The preleukemic phase, characterized by rapidly evolving spontaneous pancytopenia, was often observed in PV, PMF, and ET. (5) Extensive overlap in hematologic and pathological features was noted between PMF and the terminal phase of PV.
  • 井関 徹, 近藤 春樹
    1988 年 29 巻 7 号 p. 960-968
    発行日: 1988年
    公開日: 2009/02/02
    ジャーナル 認証あり
    Serum vitamin B12 and transcobalamin (TC) were studied in 62 cases with chronic myeloproliferative disorders (CMPD). A newly-established radioimmunoassay for serum R-protein was used in addition to conventional analyses because TC changes in CMPD are mainly attributable to R-protein increase.
    Quantitatively, marked, moderate and mild increases of R-protein were noted in chronic myelocytic leukemia (CML), polycythemia vera (PV) and essential thrombocythemia (ET), respectively. R-protein was also increased in all cases of primary myelofibrosis, chronic neutrophilic leukemia, undifferentiated CMPD and Ph1 negative CML; there have been few detailed reports concerning vitamin B12 and TC in these disorders.
    Because TC elevation in CMPD principally results from R-protein increase, analyses by gel filtration and immunological analyses were not helpful for differential diagnosis among CMPD. On DEAE, increased TC was separated into three types as follows: TC I in CML (both Ph1 positive and negative), PV-binder or TCIII in PV and TC III in the remainder.
    These characteristic TC elevations were also detected in CML cases with myeloid blastic crisis and in PV cases with a normal red cell mass because of venesection or iron deficiency. On the other hand, TC were normal both quantitatively and qualitatively in three out of eleven cases with untreated ET, while constant increases of R-protein were seen in all cases of CMPD except for ET. From these results, it is possible that cases different from true CMPD are included among ET diagnosed by the present criteria.
    As mentioned above, the examination of TC in CMPD is useful for differential diagnosis among the various cases of CMPD and from diseases other than CMPD and may play an important role especially in atypical cases difficult to diagnose.
  • 元井 信
    1988 年 29 巻 7 号 p. 969-976
    発行日: 1988年
    公開日: 2009/02/02
    ジャーナル 認証あり
    In order to clarify the relationship among various types of myeloproliferative disorders (MPD), 58 autopsy cases and 27 autopsy cases of related diseases were examined histologically, immunohistochemically and morphometrically. In chronic type of MPD, chronic myelocytic leukemia (CML), polycythemia vera (PV), myelofibrosis (MF) and essential thrombocythemia (ET), mainly proliferating cells were recognized; granulocyte lineage in CML, erythrocyte lineage in PV, granulocyte lineage and fibroblasts in MF and thrombocyte lineage in ET. However, these differences in proliferating cell type of MPD were not essential but quantative, various type of cell lineages were recognized in each type of MPD. Especially fibrosis was not essential to MF and were found in other MPD. Each transition in MPD were also observed. These findings would indicate that various types of MPD are closely related disorders, having mainly proliferating cell lineage. Immunohistochemial method with various specific antibodies were useful for recognition of various cell lineages.
  • —巨核球コロニー培養法を用いての検討—
    小松 則夫
    1988 年 29 巻 7 号 p. 977-982
    発行日: 1988年
    公開日: 2009/02/02
    ジャーナル 認証あり
    To clarify the mechanism of increased numbers of megakaryocytes and platelets in patients with essential thrombocythemia (ET), we studied in vitro megakaryocytopoiesis in ET, using a megakaryocytic colony assay in methylcellulose containing plasma or serum and medium conditioned by phytohemoagglutinin (PHA) stimulated leukocytes (PHA-LCM). ET plasma supported the megakaryocytic colony formation by normal bone marrow cells better than normal plasmadid. Moreover, in ET bone marrow cells, spontaneous megakaryocytic colonies were formed in the absence of PHA-LCM.
    In conclusion, the mechanisim of increased megakaryocytopoiesis in ET may be ascribed to (I) elevated megakaryocyte-colony supporting activity in plasma and (II) increased sensitivity to Meg-CSF or antonomous proliferation of megakaryocytic progenitor cells.
  • 長尾 忠美, 米倉 修司
    1988 年 29 巻 7 号 p. 983-988
    発行日: 1988年
    公開日: 2009/02/02
    ジャーナル 認証あり
    For the purpose of elucidating the mechanism of myelofibrosis in chronic myeloproliferative disorders (CMD), collagen synthesis of bone marrow fibroblasts (BMF), collagen synthesis stimulated by platelets and collagenase activity were measured in the patients with CMD. The collagen synthesis of BMF from patients with chronic myelogenous leukemia (CML) and polycythemia vera (PV) was significantly greater than that from normal subjects. The platelets of patients with CML, PV and essential thrombocythemia stimulated BMF collagen synthesis significantly compared to the response from normal subjects. The collagenase activity of serum, peripheral leukocytes, bone marrow nucleated cells and BMF from CMD patients was observed to increase significantly greater than that from normals. Moreover, collagenase activity of CML patients with myelofibrosis was significantly higher than that of CML patients without myelofibrosis. From these results, it has been considered that myelofibrosis complicated with CMD occurs due to increased collagen synthesis by BMF and overproduction of collagen stimulated by platelets and resultantly collagenase activity increases.
  • 木村 昭郎, 加藤 修, 蔵本 淳
    1988 年 29 巻 7 号 p. 989-993
    発行日: 1988年
    公開日: 2009/02/02
    ジャーナル 認証あり
    Platelet derived growth factor (PDGF) has been suggested to play some role in the pathogenesis of myelofibrosis frequently encountered in patients with myeloproliferative disorders (MPD). In this study we measured PDGF activity in circulating platelets of patients with MPD, and investigated the growth of marrow fibroblasts from MPD patients using PDGF in the presence of human plasma derived serum.
    PDGF activity of MPD patients was lower than that of normal controls, and the activity in patients with myelofibrosis was slightly lower than in those without fibrosis, supporting the idea that an abnormal release of PDGF from megakaryocytes in the bone marrow environment induce a marrow fibrosis. The PDGF-stimulated proliferation of marrow fibroblasts in MPD patients was increased compared to normal individuals. This increment of proliferation might be due to higher sensitivity of the fibroblasts to plasma derived serum than to PDGF, indicating that MPD fibroblasts are more sensitive to some factor(s) in plasma. These results could explain the pathogenesis of myelofibrosis in MPD patients.
  • (CML marrow stromal cellの由来と機能)
    高橋 益広, 藤原 正博
    1988 年 29 巻 7 号 p. 994-1000
    発行日: 1988年
    公開日: 2009/02/02
    ジャーナル 認証あり
    Chronic myeloproliferative syndromes are disorders originating from multipotent stem cells. Marrow stromal cells, which are defined as the major component of hematopoietic microenvironment, were demonstrated to be different from fibroblasts in the aspects of collagen synthesis, reactivity to the monoclonal antibodies against actin, and the function of stimulating granulopoiesis. Over 95% of marrow stromal cells established from sex mismatched marrow transplanted patients later than 45 days posttransplant were donor origin by the Y-body analysis. Marrow stromal cells established from G6PD heterozygous CML patients were demonstrated to be clonaly involved by the analysis of G6PD isoenzymes. In addition, SV40-transformed cells in marrow adherent cell layer were shown to be able to give rise to both hematopoietic and adherent stromal cells. These findings suggested that chronic myeloproliferative syndromes including CML are clonal hemopathies originating from immature stem cells which can give rise to hematopoietic and stromal cells.
  • —疾患の独立性と相互性の検討—
    谷脇 雅史, 三澤 信一
    1988 年 29 巻 7 号 p. 1001-1009
    発行日: 1988年
    公開日: 2009/02/02
    ジャーナル 認証あり
    Cytogenetic studies were performed on 60 patients with chronic myeloproliferative disorder (MPD). Chromosome analyses were done with G and Q banding on bone marrow or peripheral blood cells incubated for 24 hr. A clonal chromosomal abnormality was observed in 2 of 26 patients with polycythemia vera (PV), in 4 of 23 with essential thrombocythemia (ET), in 4 of 6 with myelofibrosis, and 4 of 5 with UMPD, that could not be classified into any type of the disease. Structural abnormalities of the long arm of no. 20 were found in 4 patients, and were possibly specific for PV and ET. Moreover, the deletion of chromosomal segment was commonly found compared with the chromosomal translocation.
    Chromosomal analysis of a single colony derived from BFU-E or CFU-GM in a patient with ET showed 1q trisomy which was identical to the aberration found in the short term culture of bone marrow cells. This is a cytogenetic evidence for monoclonal origin of the disease.
    Rearrangement of the bcr gene was studied in 6 patients; 2 with PV, 2 with ET, and 2 with UMPD. DNA prepared from the bone marrow or peripheral blood cells was digested with the restriction enzyme Bgl II, Bam HI, and HindIII. Southern blot analysis using a 0.6-kb fragment of the bcr gene showed the presence of a rearranged fragmnet in a UMPD patient with Ph1 translocation, who had not any clinical features of chronic myelocytic leukemia at all. Both cytogenetic and molecular analysis will make it possible not only to elucidate the pathogenesis of UMPD but also to evaluate their clinical features.
  • 大屋敷 純子, 大屋敷 一馬, 外山 圭助
    1988 年 29 巻 7 号 p. 1010-1017
    発行日: 1988年
    公開日: 2009/02/02
    ジャーナル 認証あり
    Molecular biologic study was performed in 30 cases of chronic myeloproliferative disorders (MPD). According to the study dealing with breakpoint cluster region (bcr) rearrangment, 10 of 11 cases of Ph1(+) chronic myelogenous leukemia (CML) had bcr rearrangement, whereas 2 of 5 Ph1(-) CML had the change. On the other hand, other MPD, including 8 cases of polycythemia vera, 2 myelofibrosis, 2 essential thrombocythemia and 1 undifferentiated MPD didn't show bcr rearrangement. These data strongly suggest that bcr rearrangement is a characteristic change for CML in MPD, and a heterogeneity might be present in Ph1(-) CML; some show bcr rearrangement and others do not. This molecular biologic heterogeneity might be related to clinical features in Ph1(-) CML.
  • 関藤 典子, 高橋 功
    1988 年 29 巻 7 号 p. 1018-1024
    発行日: 1988年
    公開日: 2009/02/02
    ジャーナル 認証あり
    The bcr rearrangement in bone marrow and/or peripheral blood cells was examined in followings; 30 standard Philadelphia chromosome-positive chronic myelocytic leukemia (Ph1+CML), 2 variant Ph1+CML [t(9;22;13)(q34;q11;q22), t(9;22)(q21;q11)], 3 Ph1+ acute leukemia, one myelofibrosis (MF), 5 polycythemia vera (PV), 6 essential thrombocythemia (ET), 2 juvenile CML (JCML), 2 chronic neutrophilic leukemia (CNL), 4 chronic myelomonocytic leukemia (CMMoL) and 3 unclassified myeloproliferative disorders (UMPD) patients. By Southern blot analysis using a 3'bcr probe, the bcr rearrangement was positive (bcr+) in 28 standard Ph1+ CML, and 2 variant Ph1+ CML, whereas negative in MF, PV, ET, JCML, CMMoL and UMPD, suggesting that the bcr rearrangement is highly specific for CML and valuable for the diagnosis of this disease. One Ph1+ bcr+ acute leukemia was thought to be blast crisis of CML with a silent chronic phase.
    Further studies on UMPD are necessary in order to clarify the relations of MPD, Ph1- CML and some myelodysplatic syndromes.
臨床研究
  • 白川 晶一, 清水 一之, 新海 晃一, 小島 博嗣, 丸山 文夫, 祖父江 良, 伊藤 直也, 井村 洋, 松井 俊和, 井野 晶夫, 江 ...
    1988 年 29 巻 7 号 p. 1025-1030
    発行日: 1988年
    公開日: 2009/02/02
    ジャーナル 認証あり
    Fifty-five patients with untreated non-Hodgkin's lymphoma received C-MOPP therapy between 1974 and 1987.
    Twenty-six patients failed to achieve complete remission (CR), while the remaining 29 patients achieved CR.
    Comparing non-CR with CR patients, there seemed to be no difference in prognostic factors such as sex, age, initial ESR and LDH level, presence of B-symptoms, histology, presence of advanced stage, and extranodal involvement. According to the pattern of response to C-MOPP, 24 non CR patients except 2 cases which were lost to follow up, could be classified into the following 5 categories.
    Type (1) (relative resistance), 4 patients showed tumor regression of more than 50% of the initial size, but could not achieve CR; Type (2) (intercycle relapse), 8 patients showed regrowth of regressing tumors before nex drug cycle started; Type (3) (resistance), 5 patients showed tumor regression of less than 50% of the initial size; Type (4) (early death), 1 patient who had ATL associated with disseminated strongloidosis; Type (5) (inadequate therapy), 6 patients received inadequate doses of procarbazine because of side effects.
    In order to overcome the unresponsiveness noted in patients categorised as Type (1) through Type (3), more intensive multidrug-therapy should be given as frequently as possible.
  • 大久保 進, 岡山 桂子, 大谷 哲司, 石田 萠子, 安永 幸二郎
    1988 年 29 巻 7 号 p. 1031-1035
    発行日: 1988年
    公開日: 2009/02/02
    ジャーナル 認証あり
    An anti-EDTA antibody-like substance (EDTA Ab.), which interfered with ABO blood type grouping, was detected in a 72-year-old male suffering from liver cell carcinoma. So far, only a few cases of EDTA Ab. have been reported in the literature.
    The panagglutination activity caused by this EDTA Ab. could be detected only in the presence of EDTA at the room temperature, but not in the presence of other mono- or polycarboxylic acids. The activity disappeared at 37°C. Pretreatment of the patient's serum with 2-mercaptoethanol extinguished the activity, suggesting that the activity was attributable to a IgM type antibody. Although the patient showed pancytopenia, that seemed to be due to hypersplenism: Coombs' test was negative, and signs of hemolytic anemia or those of pseudothrombocytopenia could not be found. On the other hand, all of serum samples obtained from six patients with EDTA-dependent pseudothrombocytopenia did not cause the panagglutination. Thus, the correlation between the EDTA-dependent panagglutination and pseudothrombocytopenia could not be found. The agglutination titer of this patient had decreased in three months. Although, we could not definitely identify the intake route of EDTA in our patient, EDTA is now permitted to be added to many kinds of foods and to contrast media for clinical use. Consequently, we should have increasing opportunities of its intake. Attention must be paid to the additives in foods or in medical supplies, whenever we appreciate the results of laboratory examinations.
  • 阿部 総太郎, 石山 泰二郎, 若林 芳久
    1988 年 29 巻 7 号 p. 1036-1040
    発行日: 1988年
    公開日: 2009/02/02
    ジャーナル 認証あり
    We studied the effect of T cells on the growth of B-chronic lymphocytic leukemia (B-CLL) colonies using on in vitro assay B cell colonies were induced by PHA and colony-stimulating factors, which were the culture supernatants conditioned by PHA, activated monocytes and T cells. Colonies of more than 50 cells were counted under a dissecting microscope. Cells in the colonies from B-CLL patients were morphologically similar to the circulating leukemic cells.
    The phenotypic expression of T cells was analyzed in three untreated patients with B-CLL. The result was low Leu3a-Leu2a ratios. T cells from B-CLL patients supressed B-CLL colonies less than to the extent of normal T cells.
  • 土田 昌宏, 小原 明, 月本 一郎, 塙 嘉之, 赤塚 順一, 星 順隆, 植田 穣, 山本 正生, 赤羽 太郎, 小宮山 淳, 伊勢 泰 ...
    1988 年 29 巻 7 号 p. 1041-1049
    発行日: 1988年
    公開日: 2009/02/02
    ジャーナル 認証あり
    It has been shown that P-100 prepared from human urine as colony-stimulating factor enhances granulopoiesis through the stimulation of human monocytes to proliferate and produce human granulocytic colony-stimulating factor (G-CSF).
    A cross over clinical study was performed in order to investigate the protective effect of P-100 on granulocytopenia in 64 pediatric patients during anti-tumor chemotherapy. We also administered P-100 to 28 patients with various types of neutropenias in childhood.
    Objective scoring system was applied to evaluate the effect of P-100 injection. The rates of effect were 28% (7 out of 25 evaluable patients) in case of acute leukemias, and 41% (9 out of 22) in case of solid tumors. The response rate of P-100 in the 22 evaluable patients with various types of neutropenia was 38% (10 out of 22).
    There were no serious side effects during and after P-100 administration.
    We conclude that P-100 may, to certain extent, protect the granulocytopenia induced by anti-tumor chemotherapy. We can also expect P-100 to be a tool in the analysis of pathophysiology of the unique childhood neutropenias.
  • 竹山 英夫, 福谷 久, 竹尾 高明, 山田 博豊, 渡邊 英二
    1988 年 29 巻 7 号 p. 1050-1054
    発行日: 1988年
    公開日: 2009/02/02
    ジャーナル 認証あり
    Twelve cases of leukemia, comprising 6 relapsed cases and another 6 cases refractory to the conventional chemotherapy, were treated with a combination of intermediate-dose cytosine arabinoside (ID Ara-C) and mitoxantrone (MXT). The cases included 8 of AML, 2 of ALL, and 1 each of myeloid crisis of CML and leukemic non-Hodgkin's lymphoma. The schedule was composed of 1 or 2 h infusion of Ara-C in a dose of 700 mg/m2 every 12 h for 5 days in combination with mitoxantrone, 10 mg/body, for 4 days. Of the 12 cases all evaluable, 7 (58.3%) achieved complete remission. The median duration of complete remission was 5 months. Non-hematologic side effects observed were predominantly gastrointestinal, as represented by nausea, vomiting, and diarrhea. No central nervous system toxicity or cardiac damage was observed. These preliminary findings suggest that the regimen (ID Ara-C combined with MXT) can be useful not only for induction of complete remission in refractory leukemia but also as a consolidation therapy for CR cases.
症例
  • 工藤 育男, 副島 薫, 森本 茂男, 沢田 美彦, 相原 守夫, 吉田 豊
    1988 年 29 巻 7 号 p. 1055-1059
    発行日: 1988年
    公開日: 2009/02/02
    ジャーナル 認証あり
    Forty-three-year-old man, complaining of burning pain in his both toes, was admitted to our hospital. Routine laboratory studies showed the following: hemoglobin 15.4 g/dl, red blood cells 6.27×106l with mean corpuscular volume 77.8 μm3, white blood cells 6,000/μl, platelets 66.8×104l, serum Fe 40 μg/dl and TIBC 360 μg/dl. Platelet aggregate ratio, which was measured by the method of Wu & Hoak, was decreased to 0.64 (normal range; 0.88±0.09), and spontaneous platelet aggregation was not observed in vitro. Platelet life span of the patient was shortened to 5 days (normal range; 8.5±0.5 days). The patient was diagnosed as having polycythemia vera with erythromelalgia. Erythromelalgia characterized by burning pain and cyanosis was relieved by the ingestion of Aspirin 600 mg per day. The effect of Aspirin, however, persisted for only 5 days. Neither thrombotic occlusion nor inflammatory process of the toe vessels was histologically observed in the biopsied specimen. These results suggest erythromelalgia of the patient is closely related to either the activation of platelets, especially via cyclooxygenase pathway, or the peripheral circulatory failure caused by increased blood viscosity and decreased deformability of microcytic erythrocytes.
  • 厚井 文一, 頼 敏裕, 高橋 功, 木村 郁郎
    1988 年 29 巻 7 号 p. 1060-1065
    発行日: 1988年
    公開日: 2009/02/02
    ジャーナル 認証あり
    A 50-year-old woman was admitted to our department because of myelodysplastic syndrome (MDS). Bone marrow showed normocellurality and myeloid hyperplasia with 8.8% of myeloblasts. Peripheral blood examination revealed pancytopenia and WBC 2,000/μl with 1% of myeloblast. PMN were deficient not only in granules and staining reactivity for myeloperoxidase and alkaline phosphatase but also in superoxide production.
    These cells inefficiently killed both Streptococcus faecalis and Staphylococcus aureus in vitro.
    She died of pulmonary insufficiency and autopsy revealed disseminated aspergillosis and diffuse pulmonary alveolar proteinosis. Marked functional defects in intracellular killing system of PMN as presented here will be one of the most important prognostic factors in patients with myelodysplastic syndrome.
  • 寺田 有由, 斉藤 毅, 花田 修一, 橋本 修治, 神崎 暁郎, 橋本 正志, 池田 明代, 八幡 義人
    1988 年 29 巻 7 号 p. 1066-1072
    発行日: 1988年
    公開日: 2009/02/02
    ジャーナル 認証あり
    A 60-year-old woman complained of general malaise and visited our hospital in August, 1984. Blood examinations revealed Hb 9.0 g/dl, RBC 287×104/mm3, WBC 2100/mm3, thrombocyte 12.3×104/mm3 and reticulocytes 7.8%. Many poikilocytes especially elliptocytes were seen in a smear of the peripheral blood. Liver function tests showed billirubin of 1.6 mg/dl (indirect bil. 1.1 mg/dl) and LDH of 524 W-U. Serum immunoelectrophoresis disclosed M-protein (IgG-κ type). But she admitted other hospital and splenectomy was performed under the diagnosis of Banti syndrome. Anemia was not improved, so she was referred again to our hospital. From bone marrow and peripheral smear, diagnosis of acute leukemia in elliptocytosis with M-proteinemia was made. So combination chemotherapy was carried out but remission was not obtained. And she died of pneumonia.
  • 村手 隆, 鈴木 隆史, 堀田 知光
    1988 年 29 巻 7 号 p. 1073-1077
    発行日: 1988年
    公開日: 2009/02/02
    ジャーナル 認証あり
    A 58 year-old male was admitted because of severe anemia in March 1987. Peripheral blood showed severe macrocytic anemia and mild leukocytosis. Remarkable megaloblastic changes were seen in bone marrow smears. Laboratory findings revealed a low level of serum vitamin B12, high LDH and the presence of anti-intrinsic factor antibody. Gastrointestinal tract survey showed no abnormality. No chromosomal abnormality was seen on karyotypic analysis. After the treatment with Vit B12, his peripheral blood showed the tendency to polycythemia. Hyperviscosity symptoms such as headache and slow speech appeared. Circulating red blood cell volume analysed at that time was increased to 54 ml/kg. Bone marrow aspiration showed disappearance of megaloblastic changes. The numbers of BFU-E and CFU-E were increased and endogeneous colony formation of CFU-E was detected when cultured without the addition of erythropoietin.
  • 山田 晋一, 江口 春彦, 生野 芳子, 岡村 純, 田坂 英子, 阿南 健一, 榎本 康弘
    1988 年 29 巻 7 号 p. 1078-1082
    発行日: 1988年
    公開日: 2009/02/02
    ジャーナル 認証あり
    A 17-month-old girl was referred to Kyushu Cancer Center because of fever and pain on the right lower leg. The bone marrow examination at the sternum showed only 2% of blasts, whereas percent of blasts was 76% at the right anterior illiac crest, 92.8% at the right posterior, 66% at the left anterior and 0% at the left posterior. Surface marker study demonstrated that the blasts were non-reactive with majority of hematopoietic cells except My9. However, reactivity was found to several antineuroblastoma monoclonal antibodies such as A2B5, UJ13A and PI 153/3. Extensive survey for neuroblastoma failed to reveal any mass lesion. The patient was tentatively diagnosed as metastatic neuroblastoma. The patient responded to the treatment protocol of neuroblastoma and obtained complete remission for 5 months. However, she relapsed in the bone marrow again and was diagnosed as acute megakaryoblastic leukemia when the blasts were found to be reactive with Gp Ib, Gp IIb/IIIa and to have platelet peroxidase. The patient obtained second complete remission for 2 months with the treatment protocol of acute non-lymphocytic leukemia.
    In this report we discussed about cross-reactivity of monoclonal antibodies of neuroblastoma with acute megakaryoblastic leukemia.
  • 乳原 善文, 守永 真一, 山口 潜, 塚田 理康, 松下 央
    1988 年 29 巻 7 号 p. 1083-1087
    発行日: 1988年
    公開日: 2009/02/02
    ジャーナル 認証あり
    A fifty-seven-year-old female was admitted with chief complaints of purpura, a left hypochondrial mass and thrombocytopenia of 19,000/μl. 111In-labeled platelet scintigraphy demonstrated a shorened platelet life span and accumulation of radioisotope in the tumor.
    Heparin administration did not influence the platelet counts which increased from 10,000 to 100,000 following prednisolone administration for twelve days, when a level of PAIgG was high. After extirpation of the tumor the platelet count increased to over 400,000 with normalization of the PAIgG level and shortened platelet life span. Pathological diagnosis of the resected tumor was teratoma of the spleen characterized by many microthrombi and foam cells in the sinus and arteriole of the spleen.
    Thrombocytopenia associated with hamartoma of the spleen is very rare and only eleven cases have hitherto been reported. As to the mechanism of thrombocytpenia, immunological destruction was speculated although local consumption by thrombus formation and/or increased platelet pooling could not be ruled out completely.
    This is the first case report of an effective prednisolone administration in this disease. Prednisolone might prove effective in a similar disease and tumorectomy may be carried out without a bleeding risk.
  • 山根 孝久, 井上 健, 古川 佳央, 佐々木 安津子, 小嶋 和英, 吉川 岳夫, 三宮 祐一, 任 太性, 巽 典之, 奥田 清
    1988 年 29 巻 7 号 p. 1088-1092
    発行日: 1988年
    公開日: 2009/02/02
    ジャーナル 認証あり
    A 43-year-old man who suffered from phlegmone was admitted to our hospital. The peripheral blood showed anemia, thrombocytopenia and leukocytosis; almost all the white cells were hypersegmented neutrophils. A bone marrow aspirate was a hypocellular material and blast cells increased to 4%. A bone marrow biopsy specimen was hypercellular and morphological adnormalities were detected in megakaryocytes. A diagnosis of refractory anemia was made.
    CRP was storongly positive. An administration of antibiotics and γ-globulin for phlegmone was not effective on the high fever. Neutrophil function tests revealed normal phagocytic activity and chemotaxis, but inadequate bactericidal activity. Granulocyte transfusions were given for 5 days. 2 days after the last transfusion, high fever ran down and CRP was negative. Phlegmone subsided gradually.
    He showed leukocytosis, mainly consisting of adnormal neutrophils, during phlegmone. As the cause of phlegmone, inadequate bactericidal activity and adnormal differentiated clone of myeloid by bacterial infection were suggested.
  • 近藤 健司, 若林 芳久, 谷口 修, 江部 司, 広瀬 俊一, 北村 成大
    1988 年 29 巻 7 号 p. 1093-1096
    発行日: 1988年
    公開日: 2009/02/02
    ジャーナル 認証あり
    A 47-year-old male was admitted to our hospital because of leukemic malignant lymphoma. At the time of admission, enlargement of the superficial lymphnodes were minimal, though marked chylothorax in the left costal space and an intraabdominal tumor of a child's head size were recognized. Combination chemotherapy resulted in a marked improvement of clinical symptoms including chylothorax. Clinical manifestations of chylothorax reported are commonly accompanied by malignant lymphoma in the foreign references. However, only three cases have been reported in Japan sofar. Clinical manifestations of chylothorax might be suggestive findings in the diagnosis of malignant lymphoma.
  • 根橋 良雄, 鳥居 泰志, 矢口 誠, 伊藤 良和, 大屋敷 一馬, 外山 圭助
    1988 年 29 巻 7 号 p. 1097-1102
    発行日: 1988年
    公開日: 2009/02/02
    ジャーナル 認証あり
    We report a case of Behçet disease developed in myelodysplastic syndrome (MDS). A 72-year-old male who was first seen in thrombocytopenic state in 1984, presented recurrent ulcers on the oral cavity and genitalia since 1985. On admission, his blood test revealed that a platelet count of 1.5×104l and a bone marrow aspirate showed dysplasia, including hypersegmented neutrophils, micromegakaryocytes and giant platelets. Chromosome study on the bone marrow cells revealed 47, XY, +8, del(20)(q11), indicating the patient was suffering from MDS with monopathic hematological feature. During the course, folliculitis-like eruption was evident and the patient was diagnosed as having incomplete-form Behçet disease. In the literature a small number of cases has been appeared in which Behçet disease developed in MDS or leukemia, and it is difficult to conclude the correlation between these disorders. Accumulation of similar cases presented here might answer the question. We also discuss a monopathic hematological presentation in MDS.
  • 和田 英夫, 玉木 茂久, 桝屋 正浩, 森 美貴, 中瀬 一則, 津田 雅之, 小林 透, 竹内 敏明, 南 信行, 出口 克巳, 白川 ...
    1988 年 29 巻 7 号 p. 1103-1108
    発行日: 1988年
    公開日: 2009/02/02
    ジャーナル 認証あり
    We studied 4 patients with thrombotic thrombocytopenic purpura (TTP) and 4 patients with hemolytic uremic purpura (HUS). One case of TTP occurred during the course of systemic lupus erythematosus (SLE), all cases were positive for anti-nuclear antibody, and two cases were positive for anti-platelet antibody. In one case, the level of platelet-associated IgG was correlated with clinical course and it was speculated that TTP was caused by an autoimmune mechanism. Before the onset of TTP, common cold-like symptoms were seen in 3 patients with HUS. The disseminated intravascular coagulation (DIC) score was high in all patients especially those, with HUS, but typical DIC was not seen in any patients. Microthrombus was detected in 3 patients with TTP, but was not detected in renal biopsy specimens from 2 patients with HUS. All patients showed good response to hemodialysis, steroid, heparin or FOY, but two patients had relapses. TTP patients were mainly treated with a combination of plasma exchange, high-dose steroid and an anti-platelet aggregation agent. Two of them died within 3 weeks but the other two showed improvement after 3 weeks. As the clinical course and symptoms of TTP varied, it should be treated with combination therapies for a long period.
  • 貞森 直樹, 糸山 貴浩, 笹川 一平, 中村 秀男, 早田 央, 池田 柊一, 市丸 道人, 草野 みゆき, 田川 真須子, 重橋 亨
    1988 年 29 巻 7 号 p. 1109-1114
    発行日: 1988年
    公開日: 2009/02/02
    ジャーナル 認証あり
    Chromosomes and surface markers in cells of the lymphnode in two patients with immunoblastic lymphadenopathy (IBL)-like T-cell lymphoma were examined before intensive chemotherapy. In patient A, the cell markers study demonstrated the presence of monoclonality of T-cells and chromosome analysis showed some abnormalities such as trisomy #3 or ring chromosome. In patient B, although the surface marker study failed to demonstrate monoclonality of B- or T-cells, chromosome analysis showed an abnormal clone with trisomy #3 and t(3;7)(q12;q32).
    From the cytogenetic aspects, the significance of chromosomal abnormalities in IBL like T-cell lymphoma was discussed in reference to the literature.
  • 帯刀 亘, 滝沢 慎一郎, 高橋 芳右, 花野 政晴, 水戸 将郎, 和田 研, 服部 晃, 柴田 昭
    1988 年 29 巻 7 号 p. 1115-1119
    発行日: 1988年
    公開日: 2009/02/02
    ジャーナル 認証あり
    A 36-year-old female was admitted to our hospital because of headache and swelling on left leg in April, 1986. She was obese and drowsy due to marked dehydration and hyperglycemia. Thrombosis of inferior vena cava and collateral circulation were demonstrated by RI angiography and venography. Coagulation studies revealed a consumption coagulopathy; namely low platelet count and fibrinogen level, and elevated FDP, D-dimer and fibrinopeptide A. However, the level of antithrombin III, protein C, protein S and plasminogen were normal. Following urokinase and heparin infusion, her general condition and laboratory data improved gradually. Then, warfarin was administered, but she was suffering from pulmonary infarction. In order to prevent fatal pulmonary embolism, inferior vena cava filter was implanted with clinical improvements.
  • 稲葉 俊哉, 柳沼 章弘, 花田 良二, 林 泰秀, 山本 圭子
    1988 年 29 巻 7 号 p. 1120-1124
    発行日: 1988年
    公開日: 2009/02/02
    ジャーナル 認証あり
    An 11-year-old boy was admitted to our hospital because of intermittent high fever, leukopenia, thrombocytopenia, and lymph node enlargements in right neck in May 1987. Lymph node biopsy revealed wide areas of necrosis with nuclear debris. A diagnosis of subacute necrotizing lymphadenitis (SNL) was made. Bone marrow aspiration showed markedly hypocellular marrow with proliferation of mature histiocytes with hemophagocytosis. Serum ferritin was raised to 19,310 ng/ml and SGOT, SGPT, and serum triglycelide were elevated. These findings were compatible with virus-associatted hemophagocytic syndrome (VAHS). He completely recovered from these symptoms 6 weeks later without treatment. Both SNL and VAHS are considered to be associated with infection, and immunological dysregulation of the host. The present case suggests these two disorders may be closely related.
  • 臼杵 憲祐, 湯尾 明, 武藤 良知, 山口 潜, 塚田 理康, 本田 一穂
    1988 年 29 巻 7 号 p. 1125-1128
    発行日: 1988年
    公開日: 2009/02/02
    ジャーナル 認証あり
    A condition of primary brachial nerve plexus lymphoma in a 73-year-old male is described. The tumor presented with solitary brachial nerve mass and treated with surgical excision, which led to a diagnosis of non-Hodgkin lymphoma, diffuse large cell type. The patient was treated with chemotherapy and radiotherapy, but presented unique clinical course that lymphoma relapsed mainly in the nervous system. Finally 20 months after the onset, the patient died. An autopsy showed lymphoma infiltration mainly in the nervous systems, such as left brachial nerve plexus, meninges, spinal ganglions, facial and sciatic nerves.
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