臨床血液
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
31 巻, 7 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
  • 塚田 理康, 上山 淳一, 宮腰 重三郎, 守永 真一, 武藤 良知, 山口 潜, 松谷 章司
    1990 年 31 巻 7 号 p. 903-910
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    In 18 patients with decreased platelet production proved by reduced platelet turnover, the numbers of megakaryocytes in clot section of marrow aspirate were counted. Platelet counts ranged from 19,000 to 128,000/μl. The sternal marrow showed decrease of megakaryocytes only in 3 out of 18 cases while megakaryocytes in the iliac marrow were reduced in 6 out of 11 cases. Only one of 11 cases showed decrease of megakaryocyte in both marrows examined. On the contrary, four cases showed normal number of megakaryocytes at both sites.
    Of 9 cases with normal or increased number of megakaryocytes in sternal and/or iliac marrow, no case showed morphologically abnormal megakaryocytes and only one case had the reduced number of platelet-forming megakaryocytes.
    Results suggested that the number of megakaryocytes in bone marrow especially in the sternum was not reliable for establishing the diagnosis of decreased platelet production, and that about 50% of patients with thrombocytopenia due to hypoproduction of platelets could be diagnosed pertinently only after the study of platelet kinetics.
  • 山田 尚, 遠藤 信也, 甲斐田 さつき, 山村 成子, 藤川 透, 堀口 順子, 稲葉 敏, 長山 泰士, 坂戸 秀吉, 山崎 泰範, 佐 ...
    1990 年 31 巻 7 号 p. 911-916
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    Both the rearrangement and the expression of the bcl-2 gene in Japanese hematopoietic tumor cells were studied by Southern and Northern blot hybridizations. The expression of the bcl-2 gene was studied in seven cultured cell lines and twenty clinical samples, including eleven non-lymphoid tumors and nine lymphoid tumors. All lymphoid tumors except one sample from a patient with ALL expressed the bcl-2 gene. The bcl-2 gene was strongly expressed in B cell tumors. This gene was also expressed in T-ALL samples studied, although the expression was not as marked as in the B cell tumors. Non-lymphoid tumors did not express bcl-2 gene. bcl-2 gene rearrangement was studied in five cultured cell lines and six clinical samples including four follicular lymphomas and two T-ALLs. No abnormal bcl-2 gene configurations were found in any of the clinical samples. Among the cultured cell lines, the BALL-1 line showed two-fold amplification. The frequency of bcl-2 rearrangement in Japanese follicular lymphomas is reported to be lower than that seen in the American follicular lymphomas. Nevertheless, the increased expression of the bcl-2 gene seen in the Japanese B cell tumors studied supports the contention that the bcl-2 gene plays an important role in the pathogenesis of B cell tumors.
  • 天野 正道, 伊東 克郎, 戸川 敦
    1990 年 31 巻 7 号 p. 917-921
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    Twelve patients with resistant or recurrent multiple myeloma were treated with VAD regimen (vincristine, adriamycin, dexamethasone), first reported by Barlogie B et al 1984. Of 11 evaluable patients, 7 responded (partial response [PR] 3, minor response [MR] 4), and the overall response rate was 63.7%. The median interval to achieve remission was 27 days, and the median duration of remission was 125 days. One course seemed sufficient for the assessment of effectiveness of the VAD chemotherapy, because in all responders, over 50% reduction of monoclonal protein was obtained in the first course of the chemotherapy. We also administered verapamil or trifluoperazine in addition to VAD in two patients who had acquired resistance to VAD. Both had MR. Side effects included infections, gastro-intestinal bleeding, congestive heart failure or severe malaise. In conclusion, the VAD chemotherapy is effective but the duration of remission is short and side effects cannot be disregarded. Perspective of VAD and improvement of the management of refractory multiple myeloma is discussed.
  • 長澤 俊郎, 小林 敏貴, 佐藤 祐二, 柳沢 裕美, 中沢 正樹, 阿部 帥
    1990 年 31 巻 7 号 p. 922-928
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    Fifty-four ITP patients were treated by the protocol proposed by Japanese Idiopathic Disease of Hematopoietic Organ Research Committee. Forty-eight patients were treated with corticosteroid (CS). Twenty-eight patients out of 30 patients who were refractory to CS were splenectomized. To study natural courses of splenectomized patients, 25 patients were splenectomized without elevation of platelet counts at the operation and were followed up their platelet counts and PAIgG for one month without treatment. Clinical courses after splenectomy were classified into four types by the platelet increment followed by splenectomy and the platelet counts at one month after splenectomy; type I (markedly over-shooted platelet peak and normalized platelet counts), type II (moderate platelet peak and 50∼100×103l platelets), type III (minimal platelet peak and less than 50×103l platelets), and type IV (elevation of platelet counts at day 7 or later of splenectomy and normalized platelet counts). The results of analyses from 25 patients indicated that the numbers of type I, II, III and IV were 10, 5, 6 and 4, respectively. The clinical course of type IV suggests that platelet production may be impaired by anti-platelet antibodies.
  • 小島 勢二, 松山 孝治, 宮崎 保, 桜田 恵右, 四家 正一郎, 沖野 栄蔵, 赤羽 太郎, 中畑 龍俊, 大国 真彦, 麦島 秀雄, ...
    1990 年 31 巻 7 号 p. 929-936
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    Thirty-nine patients with severe or moderate aplastic anemia received treatment with recombinant human granulocyte colony-stimulating factor (rhG-CSF). The first group of eight patients received rhG-CSF in doses of 100 to 400 μg/m2/d by a daily 30-minute intravenous infusion for one or two weeks. Doses up to 400 μg/m2/d were well tolerated and resulted in increases of neutrophil counts in 5 out of 8 patients. We gave rhG-CSF (400 μg/m2/d) to the second group of 26 patients by a daily 30-minute intravenous infusion for two weeks. The treatment resulted in an increase of neutrophil counts in 15 out of 26 patients (3.1 to 29.5 fold). Further, higher doses (800 or 1,200 μg/m2/d) were administered in 5 patients who did not respond to the dose of 400 μg/m2/d. The treatment increased the neutrophil counts in 3 out of 5 patients. The third group of five patients received rhG-CSF subcutaneously in doses of 20 to 400 μg/m2/d. An increase of neutrophil counts was noted in all five patients. Differential counts of bone marrow aspirate revealed an increase of myeloid: erythroid ratios. However, the responses were transient and neutrophil counts returned to basal levels within 1∼2 weeks after discontinuing treatment. No severe toxicity due to rhG-CSF was observed. These results suggest that rhG-CSF is effective on stimulating granulopoiesis in patients with aplastic anemia. This treatment will be particularly useful for the patient with aplastic anemia suffering from becterial or fungal infections.
  • 外山 圭助, 大屋敷 純子, 高久 史麿, 小林 幸夫, 宮園 浩平, 三浦 恭定, 坂本 忍, 溝口 秀昭, 星野 茂, 浦部 晶夫, 三 ...
    1990 年 31 巻 7 号 p. 937-945
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    A clinical study of rhG-CSF (KRN8601) in patients with myelodysplastic syndrome (MDS) was performed to investigate the hematopoietic effects and the increase of neutrophils. The rhG-CSF was administered daily by intravenous infusion over 30 min. to 21 patients with MDS (PARA=11, RAEB=4, RAEB in T=6).
    The dose was escalated stepwise from 50 to 400 μg/m2 every week. Within one week to 26 days after comencement of rhG-CSF administration, the increases of absolute neutrophil counts in peripheral blood were observed in all patients. Treatment with rhG-CSF enhanced normal marrow myeloid cell differentiation and maturation in 3 of 9 PARA patients and in 3 of 4 RAEB patients.
    None of patients changed to acute leukemia attributabable to rhG-CSF, but one of RAEB patient and two of RAEB in T patients progressed to leukemic phase in 21 days or two months after treatment. Minor side effects or abnormal laboratory findings were observed in 3 patients (14.3%).
    These results suggested that treatment with rhG-CSF was well tolerated and effective for improving the neutropenia between 50 to 400 μg/m2 in patients with MDS.
  • 小林 身哉, 浅野 治彦, 堀田 知光
    1990 年 31 巻 7 号 p. 946-950
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    Peculiar cytoplasmic connection between erythroblasts was observed in the smear preparation of human bone marrow; a pair of erythroblasts in the resting stage was connected by “cytoplasmic bridge” which is very thin and sometimes long compared with the telophase bridge appeared in the usual cytokinesis. Electron microscopy revealed that small amounts of microtubules were running along in the cytoplasmic bridge but mid-body was not seen. Plasma membrane about the middle of the bridge bulged to form “bulging ring”. Erythroblasts in various differentiating stages were connected by the cytoplasmic bridge and the stage of each pair was almost the same.
    The occurrence of the cytoplasmic bridge between erythroblasts was evaluated in the smear preparations of human bone marrow with hematological diseases or normal conditions. In the normal bone marrow, the mean value was 8.1 ± 3.6% (n=33). In patients suffered from autoimmune hemolytic anemia, it was 4.3 ± 2.3% (n=12), aplastic anemia, 3.2 ± 2.1% (n=6) and paroxysmal nocturnal hemoglobinuria, 5.0 ± 1.4% (n=3). While in the erythroleukemia the occurrence was very low showing 0.2 ±0.1% (n=3) of total erythroblasts. In MDS, RAEB (2.5 ± 2.4%) and RA (2.9 ± 2.2%) showed statistically significant lower occurrence. In the normal bone marrow the occurrence was higher, so the formation of cytoplasmic bridge could be essential for a normal proliferation of erythroblasts.
  • 新井 望, 原 明博, 梅田 正法, 白井 達男
    1990 年 31 巻 7 号 p. 951-957
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    COP-BLAM therapy, which has recently been reported to be useful in the treatment of malignant lymphoma, was performed on 36 patients, and the results and adverse effects of treatment were evaluated.
    Complete remission (CR) and partial remission (PR), was obtained in 32 (88.9%) and in 4 (11.1%) out of the 36 patients, respectively. So the effective ratio was 100%. Analyzing results according to staging classification, CR was obtained in all of those in stages I and II, and in 84% of those in stages III and IV. The four patients with recurrent disease after CHOP therapy exhibited a CR ratio of 50%, indicating that therapy is effective in recurrent cases.
    Adverse effects observed among the patients included leukocytopenia under 1,000/μl (5.6%), thrombocytopenia under 5×104l (2.8%), gastrointestinal symptoms (25%), peripheral neuropathy (8.3%) and alopecia (27.3%).
    The efficacy of COP-BLAM therapy appears to be satisfactory with malignant lymphoma, and all adverse effects were of mild degree.
    In addition, it may be effective in the treatment of recurrent cases which remitted after CHOP and other therapies.
症例
  • 佐藤 功, 三浦 明, 横道 弘直, 鈴木 千征, 一迫 玲
    1990 年 31 巻 7 号 p. 958-962
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    We report a 47-year-old man diagnosed as angio-immunoblastic lymphadenopathy with dysproteinemia (AILD) with fibrosis of the bone marrow, lymph node, liver and spleen, and proliferatin of epithelioid cells in lymph node. He was admitted to a hospital in May, 1980 because of general fatigue, cough, fever and systemic lymphadenopathy. The diagnosis of AILD was based on a biopsy of right cervical lymph node. His symptoms were improved but recurred with the addition of icterus and progressive pancytopenia with descrement of prednisolone. He was referred to our hospital in July, 1980 and his physical examination revealed generalized lymphadenopathy, icterus and hepatosplenomegaly. Hemogrm showed pancytopenia, and needle biopsy of the bone marrow disclosed fibrosis. Sections from the lymph node showed AILD with proliferation of epithelioid cells. Administration of 60 mg/day of prednisolone improved the fever, lymphadenopathy and hepatosplenomegaly. However he died suddenly of acute respiratory failure on July 30. Autopsy showed fibrosis of bone marrow, lymph node, liver and spleen with infiltration of abnormal lymphocytes, and pulmonary aspergylosis.
  • 吉田 ナガミ, 堀越 昶, 金丸 峯雄, 島田 一, 竹内 仁, 大島 年照, 堀江 孝至, 土屋 達行, 亀井 喜恵子, 石川 澄江, 林 ...
    1990 年 31 巻 7 号 p. 963-968
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    A 56-year-old male was admitted to the Nihon University Hospital because of general fatigue and anemia on September 21st, 1985. He had mild hepato-splenomegaly. Hematological findings showed RBC 286×104l, Hb 6.0/dl, reticulocyte count 2.5%, pletelet count 9.3×104l and WBC 2,400/μl.
    An erythroblast per 100 leukocytes counted in a blood film was found. Bone marrow was erythroid hyperplasia with megaloblasts. The erythroblasts were PAS positive but not ringed sideroblasts. Other laboratory data including hemolysis were all negative. This case seemed to be diagnosed as refractory anemia (RA) according to the FAB classification. Chromosomal analysis of marrow cells, however, all revealed 46, XY, 20q- at diagnosis and 46, XY, 7q- 20q- after 22 months.
    Furthermore, Hb electrophoresis ahd family study indicated the presence of acquired HbH disease. Neither erythroid bursts (BFU-e) nor late erythroid progenitors (CFU-e) were detected. He has had progressive anemia without proliferation of blasts for over 2 years.
    From these findings, we postulate that the entity of erythremia should be distinguished from RA including many heterogeneous diseases.
  • 山本 雅則, 西成田 進, 古田 勝彦, 竹内 仁, 堀越 昶, 飯塚 芳一, 久保 信彦, 天木 聡, 志田 光正, 絹川 典子, 押味 ...
    1990 年 31 巻 7 号 p. 969-973
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    Abnormal expansion of large granular lymphocytes (LGLs) was observed in peripheral blood and bone marrow in a 28-year-old man. He had general lymphadenopathy and splenomegaly. Surface phenotypical analysis of LGLs showed that these LGLs express CD 2, Ia and NKH-1 but not express CD 3, CD 4, CD 8 and Leu 7. Cytochemical analysis of these LGLs revealed positive acid phoshatase and β-glucronidase reaction but negative α-naphtyl acetate esterase reaction. These LGLs showed very weak NK activity against only MOLT-4 but showed no cytotoxic activity against K 562. An β-receptor gene rearrangement of human T-cell receptor was not found by Southern blot analysis.
    Rapid and fetal clinical course with the results of theses analytical studies showed that this case is highly suggestive of acute leukemia of LGLs which is committed to NK cell lineage.
  • 平澤 晃, 比留間 潔, 脇田 久, 遠藤 伸行, 高林 克日己, 王 伯銘, 吉田 尚, 青墳 信之, 浅井 隆善, 五十嵐 忠彦, 伊藤 ...
    1990 年 31 巻 7 号 p. 974-978
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    A 36-year-old man was diagnosed as having RAEB in 1986, and required blood transfusion regularly because of severe anemia. He received the first bone marrow transplantation following total-body irradiation and etoposide infusion in October 1987. He was found to be relapsed into RAEB on 106th day after BMT. And the second BMT was planned. According to the conditioning regimen of Tutschka, et al, we administrated busulfan and cyclophoshpamide before re-transplantation. On 26th day after BMT, the WBC count exceeded 1,000/μl and anemia was improved, while thrombocytopenia persisted until 50th day. Normal hematopoiesis in the bone marrow was confirmed on the 29th day. No severe side effect except for a little fevering and bleeding was found during the clinical course. Unfortunately he died of pneumonia following graft versus host disease on the 166th day after re-BMT. This new conditioning regimen is considered to be a choice for the high risk patients on re-transplantation.
  • 本橋 百合子, 飯塚 芳一, 竹内 勝啓, 鈴木 新司, 一井 しず子, 竹内 仁, 河村 雅明, 大屋敷 純子, 外山 圭助, 大島 年照 ...
    1990 年 31 巻 7 号 p. 979-983
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    A 38-year-old male admitted to the Internal Medicine of Surugadai Nihon University Hospital, complaing of general fatigue and throat pain. The laboratory examinations revealed leukocytosis (83,900/μl) and an appearance of myeloblasts (90.2%) in the peripheral blood. The nucleated cell count was 56×104l with 85.5% myeloblasts in bone marrow. He was diagnosed as acute myeloblastic leukemia (AML). Though he received two courses of combination chemotherapy with daunorubicine, BH-AC, 6 MP and prednisone, one course of combination with mitoxantrone, etoposide and cytosine arabinoside and one course of combination with aclarubicin cytosine arabinoside and prednisone, he could not achieved remission.
    A chromosome analysis revealed 46, XY del(5)(q22). The amount of DNA fragments hybridized to 4.5 Kb v-fms probe in blasoid cells was approximately a half amount of normal persons. It is not defined the relationship between the decrease of fms and leukemia in this case. He was diagnosed de novo AML, since he had not been received the therapy with potential mutagenic and carcinogenic agents and had not been exposed the irradiation on his works.
  • 山本 克也, 東田 修二, 三木 徹, 鈴木 利哉, 長田 薫, 神山 尚, 三浦 修, 今井 康文, 村上 直巳, 加藤 淳, 奈良 信雄 ...
    1990 年 31 巻 7 号 p. 984-988
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    A 54-year-old man was admitted to our hospital for precise examination of pancytopenia in October 1988. He had been cut off his left femur and irradiated because of osteosarcoma in 1954. After 30 years, he was diagnosed as Waldenström's macroglobulinemia. Melphalan had been given 2 mg daily for 19 months until August 1988, when it was discontinued due to pancytopenia.
    Peripheral blood showed Hb 6.6 g/dl, platelet 40×103l, and WBC 2000/μl with 33% blasts. Bone marrow showed normocellularity with 36% blasts. Although blasts were negative for peroxidase staining, surface marker analysis revealed myeloid (CD 13, CD 33) phenotypes. Chromosome analysis showed 45, XY, -7, inv (3). A CT scan of the liver showed a mass, 10 by 10 cm, compatible with hepatocellular carcinoma. He was treated with very low dose Ara-C without noticeable effect. Hepatic tumor gradually enlarged, and he died of hepatic failure.
    This is a rare case of quadruplicate malignancies. The chromosomal abnormality suggests that AML was secondary leukemia which might be associated with immunosuppression due to macroglobulinemia and/or melphalan therapy.
  • 児玉 和也, 門脇 誠三, 松尾 武文, 酒井 郁也
    1990 年 31 巻 7 号 p. 989-993
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    The patient, an 18-year-old male, was admitted on May 17, 1988, because of high-grade fever, neuralgia and generalized lymphadenopathy. Bone marrow examination revealed a large number of small nests with myeloid blastic cells negative for both peroxidase and TdT activity. Ph1 chromosome and bcr rearranged fragment were positive. On a diagnosis of CML in the accelerated phase, treatment was started with standard BHAC-DMP and vincristine. However, fever still persisted and hematological improvement could not be obtained. From September 20, 1988, mithramycin was given at 25 μg/kg every three days. No fever was noted and the NAP score decreased. However, fever reappeared despite the continuing treatment. Combination use of vincristine (1.0 mg/week) and mithramycin (25 μg/kg/week) was then begun, and the fever immediately disappeared. After mithramycin administration, a transient marked increase of neutrophils appeared in the peripheral blood, suggesting the induction of differentiation. After then, a complete remission was obtained. A transient disappearance of Ph1 chromosome by the chemotherapy was noticed. He has remained in the chronic phase of CML for one year.
    In conclusion, combination use of vincristine and mithramycin may be useful in the treatment of the myeloid blast crisis.
  • 稲葉 俊哉, 林 泰秀, 細谷 静恵, 花田 良二, 山本 圭子, 西田 俊朗, 中村 こずえ, 水谷 修紀, 杉田 完爾, 中澤 真平, ...
    1990 年 31 巻 7 号 p. 994-998
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    A 3-year-old boy was referred to our hospital in September 1985, because of pancytopenia. His bone marrow was normocellular with 18% blasts, which had Auer rod and were positive for peroxidase staining. A diagnosis of refractory anemia with excess blasts in transformation was made according to FAB criteria. Chromosome analysis of bone marrow cells showed normal male karyotype. He attained complete remission with aclarubicin and BH-AC and continued it until August 1987 when pancytopenia and hypoplastic bone marrow developed. Chromosome analysis of bone marrow cells showed normal male karyotype and gene analysis revealed germ-line configuration of breakpoint cluster region (bcr). Overt leukemia developped in May 1988 when his WBC count increased to 60,600/μl with 91% blasts, which were negative for peroxidase staining, positive for anti-Ia and CDw 41 by cell surface analysis, and positive for ultrastructurally demonstrable platelet peroxidase. A diagnosis of megakaryocytic leukemia was made. Chromosome analysis of bone marrow cells showed 46, XY, t(9;22)(q34;q11) and gene analysis revealed rearrangement of bcr. He died in November 1988. Our results and review of literature suggest that late appearing ph1 chromosome and rearrangement of bcr may occur in a variety of hematologic malignancies and influence the course of disease.
  • 柴田 寿彦, 小池 知治, 中村 栄男
    1990 年 31 巻 7 号 p. 999-1003
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    Two young females were admitted one by one because of peculiar skin eruptions suggesting mosquito-allergy and severe pancytopenia with normo∼hypercellular bone marrow.
    The outcome of the first case was fatal on account of severe pancytopenia, immune deficiency and marked jaundice 14 months after the initial pancytopenic episode. In the post-mortem examination, HMR (histiocytic medullary reticulosis) was highly suggestive since hepatosplenomegaly with increase of Kupffer cells and large histiocytes including some atypical cells were detected.
    The second patient was treated with splenectomy because the past treatment were unsatisfactory in the first case and another cases in literatures.
    After the operation, the patient sustained almost normal blood cell level for 11 months without any treatment.
    As for the histopathological findings of resected spleen and lymphnodes, some erythrophagocytic mature histiocytes were found, but no atypical cells was detected.
    The relationship with pancytopenia, mosquito-allergy and HMR was discussed on the two cases and literatures.
  • 佐藤 謙, 牛島 俊和, 大林 由明, 佐藤 宏, 浦部 晶夫
    1990 年 31 巻 7 号 p. 1004-1007
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    A 72-year-old man was admitted to our hospital because of general malaise. The peripheral blood showed pancytopenia (WBC 800/μl, RBC 970,000/μl, Plt 95,000/μl).
    The bone marrow smear revealed morphological abnormalities in three lineage without increase in blasts. He was diagnosed as having myelodysplastic syndrome (MDS). He was treated with recombinant human granulocyte colony-stimulating factor (rhG-CSF) for leukopenia, and with washed RBC transfusions for anemia. Eight months after diagnosis of MDS, blastic cells increased in peripheral blood and bone marrow showing overt leukemic state. He died of pneumonia. An autopsy revealed that atypical megakaryocytes increased in bone marrow and infiltrated into the spleen and liver. This case suggests that not only blasts but also megakaryocytes infiltrate into extramedullar organs in some cases of MDS.
  • 槇島 誠, 山本 光一郎, 元吉 和夫, 永田 直一, 高谷 治, 松浦 喜房, 布施 裕補
    1990 年 31 巻 7 号 p. 1008-1012
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    A 74-year-old man was admitted to the National Defense Medical College Hospital because of purpura on the extremities. The blood hemoglobin level was 11.5 g/dl, the white blood cell count 7.3×109/l with 3% blastic cells, and the platelet count 4.0×109/l. There was leukoerythroblastosis with only mild teardrop appearance of erythrocytes. The blastic cells consisted of two types, megakaryoblast-like cells and myeloblast-like cells. There was a very mild hepatosplenomegaly, but no lymphadenopathies. The bone marrow aspirations yielded dry taps. The marrow biopsy specimen revealed myelofibrosis associated with trilineage hyperplasia, but no apparent infiltration of leukemic cells. Platelet-associated IgG (PAIgG) had a high titer and the response to platelet transfusions was poor. On prednisolone and colchicine, the blastic cells in the peripheral blood disappeared and the platelet count increased. He died of pneumonia. The autopsy showed myelofibrosis with hyperplasia of myeloid and erythroid lineage in the bone marrow. Megakaryocytes were relatively few and there was no infiltration of leukemic cells.
  • 小林 裕, 木村 茂, 木村 晋也, 田中 耕治, 和田 勝也, 小沢 勝, 丸尾 直幸, 近藤 元治
    1990 年 31 巻 7 号 p. 1013-1016
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    A 53-year-old woman was admitted to our hospital on Nov. 16, 1987, because of general fatigue. On admission, she had hepatosplenomegaly and her peripheral blood profile showed a white blood cell count (WBC) of 309×103l with immature neutrophils, a hemoglobin level (Hb) of 7.6 g/dl, platelet count (PLT) of 536×103l, neutrophilic alkaline phosphatase (NAP) score of 44. Both Vitamin B12 and LDH levels were high. The bone marrow showed marked myeloid hyperplasia. In a cytogenetic study, Ph1 was found in 3 of 8 metaphases and Ph1 with an additional abnormality of 8 trisomy was noted in 5 of 8 metaphases. She was diagnosed as having chronic myelogenous leukemia (CML) and treated by i.m. injection of interferon (IFN)-α at a daily dose of 6×106 U. Administration of IFN-α induced fever for a few days. WBC, PLT count and LDH level gradually decreased, and the NAP score and hepatosplenomegaly improved. She achieved remission in February, 1988. Administration of IFN-α was stopped in April, 1988, when the bone marrow showed hypocellularity and normal karyotype. She was treated with 20 mg of prednisolone daily from May until August, because of progressive pancytopenia. She had received no treatment until July, 1989. In May, 1989, the bone marrow again showed myeloid hyperplasia and Ph1 was found in all cells analyzed. Therefore, we resumed IFN-α treatment. It is interesting that remission of CML continues for more than one year after discontinuation of IFN-α in this case.
  • 宇都宮 洋子, 岩政 喜久恵, 安川 正貴, 玉井 伴範, 塩坂 孝彦, 坂東 史郎
    1990 年 31 巻 7 号 p. 1017-1021
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    A 43-year-old woman with Ph1-positive chronic myelogenous leukemia (CML) was diagnosed as havihg blastic crisis. The phenotype of blasts was CD9+, CD10+, CD19+, CD11b+ and CD33+, suggesting the B Lymphoid and myeloid mixed lineage. Two color analysis of CD10 and CD33 revealed that 50% of blast cells had both B lymphocyte- and myelomonocyte-associated surface markers. Rearrangement of the immunoglobulin heavy chain gene was detected. After culturing blasts with 12-o-tetradecanoyl-phorbol 13 acetate (TPA), basophilic granules appeared in cytoplasm of the cells. These granules were positive for toluidine blue staining. This finding that the biphenotypic blasts expressing both B lymphoid and myelomonocytoid features differentiated into basophils suggests that blasts of this case are derived from a common progenitor of B lymphoid and myeloid lineages including basophil.
  • 和田 秀穂, 大槻 剛巳, 杉原 尚, 阿多 雄之, 橋本 正志, 山田 治, 八幡 義人, 真鍋 俊明, 宮本 寛治
    1990 年 31 巻 7 号 p. 1022-1027
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    The patient was a 64-year-old woman who was admitted to our hospital because of lumbago. A diagnosis of multiple myeloma (non-producing type) was made, based on (1) the presence of multiple osteolytic lesions, (2) hypercellular marrow with 64.2% plasmocytoid malignant cells, (3) no monoclonal gamma-globulin was detected in the serum and urine, and (4) abnormal monoclonal gamma-globulin was also not detected in the cytoplasm and membranes of these malignant cells.
    After several courses of chemotherapy, a pleural effusion infiltrated by myeloma cells developed and the patient's serum contained a markedly increased amylase activity of salivary-type. Amylase activity was also detected in vitro in the supernatant of cultured myeloma cells established from the patient's pleural effusion. The presence of α-amylase in the myeloma cells, which were derived from pleural effusion, was demonstrated immuno-histochemically. These observations indicates that amylase was ectopically produced by these myeloma cells. Interestingly, 14 out of 20 metaphases in the cells derived from pleural effusion showed translocation of lp22 near the region of lp21, where the amylase gene was assigned.
  • 沢田 美彦, 工藤 育男, 高見 秀樹, 相原 守夫, 吉田 豊, 木村 あさの, 中原 一彦
    1990 年 31 巻 7 号 p. 1028-1031
    発行日: 1990年
    公開日: 2009/03/12
    ジャーナル 認証あり
    A 23 year old female, born in 1957, was diagnosed as having idiopathic thrombocytopenic purpura at the age of 3 and treated with prednisolone during her childhood with no response. On her regular check-up in 1978, facial edema and proteinuria suggested renal disease. The family history was negative for bleeding diathesis or renal disease. Close examination revealed the following: WBC 4,200/μl without leukocyte inclusions, RBC 3.42×106l, Hb 11.7 g/dl. PT 10.6 sec, APTT 28.9 sec. Platelet count 4,500/μl by HEMATRAK 360, and 40×103l measured by microscopic method. Giant platelets were noted on peripheral blood smear with an average diameter of 6.1 μm. Bleeding time (Duke) was 12.0 min. Number of megakaryocytes was increased although platelet production was remarkably decreased. Results of platelet aggregation and retention tests were normal. Platelet life span (T1/2) was 2.3 days. Senosory neural hearing loss, congenital cataract, double ureter and short small intestine were also found. Chromosome analysis showed 46XX.
    She underwent splenectomy resulting in increase of the platelet count to 226×103l. The increased platelet count, however, gradually decreased to the initial count in 2 years although the bleeding tendency was improved. In 1987, renal function deteriorated, causing intractable hypertension. The serum creatinine was 4.8 mg/dl. The following year she developed cerebral bleeding and died 4 days after the episode. The serum creatinine was 8.6 mg/dl.
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