臨床血液
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
26 巻, 7 号
選択された号の論文の19件中1~19を表示しています
第26回総会
シンポジウムIII Hemophilia―1984
  • 大森 啓造, 須永 俊明, 斎藤 英彦
    1985 年 26 巻 7 号 p. 1035-1041
    発行日: 1985年
    公開日: 2009/02/02
    ジャーナル 認証あり
    The structure of human factor VIII/von Willebrand factor (F VIII) was examined by electron microscopy. F VIII was purified from intermediate-purity F VIII concentrates, normal plasma or hemophilia A plasma. These F VIIIs were rotary shadowed with carbon and platinum, then examined by transmission electron microscope. There were no differences in shape between these three types of F VIIIs. The structure of rotary shadowed F VIII resembles very flexible filaments with irregularly spaced small nodules and hetrogeneous length. To evaluate the size distribution of F VIII, the contour length of 100 molecules were measured. They ranged from 150 to 1,080 nm with a mean length of 520 nm. The corresponding molecular weights were determined by comparison to the similar strands of fibronectin. The molecular weight of the F VIII ranged from 600,000 to 4,500,000 daltons with a mean molecular weight of 2,140,000 daltons.
  • 緒方 完治, 南 三郎, 神谷 忠
    1985 年 26 巻 7 号 p. 1042-1049
    発行日: 1985年
    公開日: 2009/02/02
    ジャーナル 認証あり
    We have studied the role of factor VIII/vWF in platelet retention by glass beads column and ristocetin-induced platelet aggulutinationu sing five mouse monoclonal antibodies, polyclonal rabbit antiserum and homologous antibodies to human F VIII/vWF.
    Four mouse monoclonal antibodies to human VIII R: WF were derived from BALB/C mouse spleen cells fused with NS1 mouse plasmacytoma cells. These antibodies, harvested from culture medium or ascites fluid, reactcd with partially purified F VIII/vWF and with plasmas from normal subjects, as measured by ELISA, but not with plasma from patient with severe von Willebrand's disease. Ooe of the four monoclonal antibodies, P-AHF 110, inhibited both VIII R; RCo and platelet glass beads column retention (PL-GBR), and three of the four monoclonal antibodies, NOM1-3, inhibited PL-GBR only. Anti-VIII: C monoclonal antibody (Hybritech Inb) blocked VIII: C only.
    An amout of rabbit antiserum to F VIII/vWF that provided equivalent inhibition of VIII R: RCo blocked PL-GBR much more effectively than the mouse monoclonal antibody, P-AHF 110.
    Nonprecipitating antibodies to VIII R: Ag were found in a patient with severe von Willebrand's disease during replacement therapy with F VIII concentrate and cryoprecipitate. The antibodies blocked PL-GBR but did not inhibited VIII R: RCo at all.
    These data suggested that the site or sites on VIII R: WF that are associated with VIII R: RCo differ quantitatively or qualitatively from those associated with PL-GBR.
  • 松田 重三
    1985 年 26 巻 7 号 p. 1050-1058
    発行日: 1985年
    公開日: 2009/02/02
    ジャーナル 認証あり
    Cellular as well as humoral immunological functions of 53 hemophiliacs who were under the replacement therapy with anti-hemophilic preparations were evaluated. T4 lymphocyte count of 25 cases out of 53 hemophiliacs was revealed to be decreased moderately to markedly and T8 lymphocyte count in 26 cases out of them was increased moderately to markedly. Accordingly the ratio of T4/T8 lymphocytes was less than 0.7 in 27 cases. HLA-DR positivities of T4 and T8 lymphocytes were also proved to be increased in 5 and 16 respectively in 26 cases. It was also revealed that activity of NK cell and blastogenesis of lymphocytes were depressed. KLH skin test was proved positive in 91% of hemophiliacs in contrast to low positivity of PPD skin test (58%). In addition phagocytic functions of neutrophiles, tested using opsonized zymosan was deteriorated in 58% of the patients. Serum concentrations of immunoglobulins, especially in IgG, and beta 2-microglobulin (beta 2-M) were increased moderately to markedly in 37% and 40% respectively.
    Consequently it could be concluded that cellular and humoral immunological functions of hemophiliacs were deteriorated to a considerable degree. Although it was suggested that those immunological abnormalities in hemophiliacs might be induced by repeated infusions of immunosuppressive acidic protein, beta 2-M and ATLV/HTLV (adult/human T cell leukemia virus) that might be present in currently used anti-hemophilic preparations, further investigations would be requested.
  • 藤巻 道男
    1985 年 26 巻 7 号 p. 1059-1068
    発行日: 1985年
    公開日: 2009/02/02
    ジャーナル 認証あり
    One of the major complications of transfusion therapy in hemophilia A and B is the elicitation of an antibody to the infused coagulation factor. Observation on inhibitor patients suggest that there are two type of antibody response. In most patients, high titer antibodies occur (>10 Bethesda U/ml), making further replacement therapy with factor VIII impractical. On the other hand, only low levels of antibodies arise (1∼5 Bethesda U/ml), levels which tend not to increase after exposure to factor VIII.
    As for the treatment of hemophiliacs with inhibitors, general therapies under present conditions of Japan are as follows.
    1. In the case of less than 10 Bethesda Units/ml:
    Factor VIII preparations for the patients with hemophilia A, or factor IX preparations (PCC) for the patients with hemophilia B are infused much. Consequently the inhibitors are neutralized. As for the bypassing therapy, 50∼100 units (factor IX activity)/kg of prothrombin complex concentrates (PCC) are infused 1∼3 times a day.
    2. In the case of more than 10 Bethesda Units/ml:
    50∼100 units (factor IX activity)/kg of PCC are infused 1∼3 times a day. If PCC are not effective, 50∼100 units (FEIBA unit or Autoplex·FECU)/kg of Activated PCC are infused 1∼3 times (at an interval of 8∼12 hours) a day. During these treatments, plasmapheresis are performed at need and 100∼300 units/kg of factor VIII preparations for the patients with hemophilia A or the same value of factor IX preparations (PCC) for the patients with hemophilia B are infused 1∼3 times a day.
    Concerning replacement therapies, the therapeutic option is important. In addition, the appropriate should be performed after the conditions of patients and their hemorrhagic conditions are made clear.
  • 福井 弘, 高瀬 俊夫
    1985 年 26 巻 7 号 p. 1069-1079
    発行日: 1985年
    公開日: 2009/02/02
    ジャーナル 認証あり
    The effect of DDAVP was studied in normal subjects and patients with mild or moderate hemophilia A and various types except homozygous form of von Willebrand's disease (vWD).
    In 10 normal subjects, 0.4 μg/kg of DDAVP infusion resulted in a rapid 2-4 fold increase in factor VIII coagulant activity, factor VIII coagulant antigen, factor VIII related antigen and ristocetin cofactor activity. After DDAVP infusion, all multimers of factor VIII related protein (VIII R) in the plasma were increased in concentration and larger forms than 20×106 daltons appeared newly for several hours. On the other hand, multimers in the platelets were decreased in their concentration. It was considered that factor VIII R is released from platelets as well as endothelial cells following DDAVP infusion.
    In 8 patients with mild or moderate hemophilia A, four activities also increased 2-4 fold, respectively. The multimeric composition of plasma VIII R before and after DDAVP infusion were similar to those in normal subjects. In 5 patients with type I vWD, the increase in 4 activities was 3-9, 3-8, 5-12 and 4-6 fold. All multimers were present in the plasma, though they were decreased in concentration. DDAVP infusion resulted in an increased concentration of these forms as well as the appearance of larger forms. In 4 patients with type IIA, a similar rise in 4 activities was observed. This type showed only 3-4 smaller multimer bands in the plasma, and one more band appeared for several hours following DDAVP infusion. One patient with type IIB showed also a similar increase in 4 activities, but there was a moderate decrease in platelet count. In this type, the larger multimers were absent from plasma, but present in platelets. After DDAVP infusion, larger multimers appeared in the plasma and disappeared after 2 hours.
    These results led to the conclusion that DDAVP is useful for the hemostatic management of patients with mild or moderate hemophilia A and type I and type IIA vWD.
  • 長尾 大
    1985 年 26 巻 7 号 p. 1080-1085
    発行日: 1985年
    公開日: 2009/02/02
    ジャーナル 認証あり
    In Japan, home therapy of hemophilia was officially approved on February 1, 1983. A nation-wide survey was performed in August, 1984, and assessment of this type of treatment was tried through analysis of the results, from various points of view.
    Questionaires were sent to hemophiliacenters covering 1,690 hemophiliacs. Out of the 1,690 patients, 639 (37.8%) were on home therapy programmes. Through analysis of 386 answers (60% of the 639 patients), it was revealed that mean age was 22 years, and duration of home therapy was within a year in 35.2% of the patients and above 5 years in 12.7%.
    As for the amount of blood products consumed, after the start of home therapy programmes, it was “increased” in 19.0% of the patients by more than 20%, “decreased” in 43.5% of the patients by more than 20%, and “unchanged” in 34.7%. In 66 patients, actual annual amount used was studied. In average, 26,206±22,540 U/pt/year were used 2 years before starting home therapy, and 52,305±38,877 U/pt/year were used 4 years after starting the programme. However, if the amount of blood products consumed is divided by each patient's body weight, it was always around 900 U/kg/year, and did not show any significant changes through out the 7 years.
    Benefits and problems of home therapy programmes were listed up by both the patients and hemophilia centers, and it was pointed out that proper teaching programmes were the most important, in order to solve the problems.
  • 櫻川 信男
    1985 年 26 巻 7 号 p. 1086-1089
    発行日: 1985年
    公開日: 2009/02/02
    ジャーナル 認証あり
    The fundamental treatment for the patients of hemophillia and von Willebrand's disease is the replacement of the coagulation factors deficient to each patients. Recently the home infusion therapy has become widespread, but the patients are still suffering the fear of the accident accompanied by the venous injection. Therefore the oral administration of the coagulation factors is the most desirable form of therapy for these patients.
    Based upon the findings of the animal experiment (using beagle dogs) that at least some part of the liposome encapsulated Factor IX concentrates administered orally is absorbed from the intestine, we performed the clinical trial of the oral administration of liposome encapsulated Factor VIII concentrate to the volunteer patients of mild haemophilia A and type 1 von Willebrand's disease.
    In the patients of haemophillia A, Factor VIII: C increased by several percent and Factor VIII R: Ag increased by 40∼100%. In the patients of von Willebrand's disease shortening in bleeding time, aPTT, and increase in ristocetin induced platelet aggregation was observed, and the recovery rates of Factor VIII: C was 20∼40%.
臨床研究
  • 島崎 千尋, 西尾 晃, 春山 春枝, 伊勢村 卓司, 中川 雅夫, 伊地知 浜夫
    1985 年 26 巻 7 号 p. 1090-1097
    発行日: 1985年
    公開日: 2009/02/02
    ジャーナル 認証あり
    Cytofluorometric analysis of DNA content on leukemic cells in 12 patients with blastic phase of chronic myelogenous leukemia was performed referring to morphological and cytogenetic findings.
    DNA distribution patterns were classified in the following three types: I) Leukemic cells consisting of 2C stem line with scattering up to 4C DNA values, II) Leukemic cells consisting of 2C stem line with that containing abnormal large DNA contents, and III) Leukemic cells consisting of hyper-2C stem line. Based on this classification, three patients were grouped in type I, three in type II, and six in type III. Morphologically, abnormally large and bizarreshaped cells were encountered in type II and III. Distribution patterns of DNA contents corresponded well to cytogenetic findings.
    Through these observations, the deviation of DNA content, suggesting the appearance of aneuploid cells, was considered to be due to abnormal cell division and was supposed to correspond to the development of blastic crisis.
  • 垣下 榮三, 小山 哲司, 永井 清保, 朱 明義, 岡本 英三
    1985 年 26 巻 7 号 p. 1098-1103
    発行日: 1985年
    公開日: 2009/02/02
    ジャーナル 認証あり
    Transient hypotension, shock and DIC occurred following injection of about 20000 units of thrombin into esophageal varices in endoscopic embolization. Hypotension was seen within a few minutes after thrombin injection and improved without sequela. DIC became marked 30 min. after injection and showed signs of improvement 1 day later. Similar incidents were also seen in patients who received purified thrombin, indicating that these are due to thrombin itself. Furthermore, hypotension and shock seemed to be associated with the formation of microthrombi because their onset coincided with that of decreases in fibrinogen and platelets at a very early stage of DIC. The use of aprotinin, FOY and methyl prednisolone for prevention of hypotension and shock was a failure. It seems that an effective preventive treatment must confine thrombin in the varices.
  • 吉川 敏, 田原 徹, 佐尾 浩, 山内 辰也, 吉川 治哉, 松山 孝治, 平岩 明和, 赤尾 幸博, 森島 泰雄, 小寺 良尚
    1985 年 26 巻 7 号 p. 1104-1112
    発行日: 1985年
    公開日: 2009/02/02
    ジャーナル 認証あり
    Twenty patients, 16 with acute leukemia (AL) and four with chronic myelogenous leukemia (CML), were treated by allogeneic bone marrow transplantation. All six patients with AL transplanted in the first remission are alive without recurrence 1 1/4∼2 5/6 yrs after transplantation. Karnofsky's performance acores in five patients are more than 90%. All seven patients with AL transplanted in relapse died 36∼495 days after grafting. Of four patients with CML, one transplanted in chronic phase is alive 387 days after transplantation.
    Acute graft-versus-host disease (GVHD) developed in 13 patients (63%), of whom only two had severe GVHD (grade III or higher). Ten (71%) of the 14 patients who survived for more than 100 days after transplantation developed chronic GVHD. Interstitial pneumonitis (IP) occurred in seven (35%) patients, of whom five were associated with cytomegalovirus infection. Four patients (20%) had relapse of leukemia.
    Of the 12 deaths, six (50%) were due to IP and four (33%) to relapse of leukemia.
  • 大野 章, 宮崎 修一, 五島 瑳智子, 金子 晴生, 白井 達男
    1985 年 26 巻 7 号 p. 1113-1119
    発行日: 1985年
    公開日: 2009/02/02
    ジャーナル 認証あり
    It is documented that patients with multiple myeloma (M.M.) have a high incidence of bacterial infection, suggesting a defect in their defense against bacteria. In order to assess the possibility that the killing activity of neutrophils in M.M. patients is impaired, the bactericidal activity and superoxide productivity of neutrophils were studied in normal individuals and M.M. patients.
    The O2- productivity and bactericidal activity were significantly lower in M.M. patients than in the normal. A high correlation between the O2- productivity and bactericidal activity was found in M.M. patients. Moreover, the decreasing levels of O2- productivity was closely related to the progression of stages in M.M. patients, i.e. it showed a positive correlation with Hb levels and a negative correlation with serum calcium levels based on the criteria of each stage. These results indicate that the function of impaired neutrophils might be due to bone marrow injured by tumor cells, and that one of the factors causing frequent high infections in M.M. patients is related to decreased O2- productivity.
症例
  • 大塚 実, 越野 健, 藤村 政樹, 金森 一紀, 上田 幹夫, 舟田 久, 原田 実根, 中村 忍, 松田 保
    1985 年 26 巻 7 号 p. 1120-1125
    発行日: 1985年
    公開日: 2009/02/02
    ジャーナル 認証あり
    A 73-year-old woman who suffered from bronchial asthma from Mar. 1981, was admitted to our clinic because of dyspnea on Feb. 7th, 1984. Laboratory examinations revealed WBC 22,100/mm3, eosinophils 87% and IgE 4,875 U/ml. There was no shadow on chest X-rays. Thereafter, the left optic nerve atrophy, paresthesia al left lower leg and bilateral feet, transient pulmonary infiltration, pericardial effusion and hepatomegaly were observed. She was injected dexamethasone 4 mg/day into the left orbita from Feb. 21st, 1984. But the defect of visual field only slightly improved. She was administerd prednisolone 50 mg/day from Mar. 23rd, 1984, and laboratory examinations revealed WBC 4,500/mm3 and eosinophils 2% three days after. Paresthesia improved but three was no improvement in left visual defect, pericardial effusion and hepatomegaly. Therefore the diagnosis of hypereosinophilic syndrome (HES) was made. But bronchial asthma, aspergillus fumigatus and parasite are considered as the etiology of eosinophilia.
    Report of the case of HES complicated with optic nerve atrophy like this has been scanty. So we have reported this case here.
  • 田中 信子, 小林 正之, 落合 成正, 片山 俊夫, 菊池 明夫, 吉田 真弓, 小幡 和恵, 海渡 健, 宮原 正
    1985 年 26 巻 7 号 p. 1126-1130
    発行日: 1985年
    公開日: 2009/02/02
    ジャーナル 認証あり
    A nineteen years old female with a platelet releasing disorder was presented. Her pathological condition was characterized by the defects of both secondary platelet aggregation and ATP release in response to ADP, collagen and epinephrine respectively. The storage pool of her platelet seemed to be intact, because the supernatant of her destroyed platelets was sufficient to induce the aggregation of normal platelets. Either addition of arachidonate or calcim ionophore A 23187 resulted in normal aggregation of her platelets.
    On an electronmicroscopic study, over 50% of her platelets revealed the membrane complex originated from dense tubular system and open canalicular system.
    From above results, it may be suggested that her platelet has a normal cyclo-oxygenase activity, but possible impaired arachidonate liberation from phospholipid. It is presumed that her defective platelet function may be due to an impaired calcium mobilization.
  • 森内 幸美, 上平 憲
    1985 年 26 巻 7 号 p. 1131-1134
    発行日: 1985年
    公開日: 2009/02/02
    ジャーナル 認証あり
    A 75-year-old man was admitted to Nagasaki Municipal Hospital because of severe anemia and thrombocytopenia following to the upper respiratory tract infection. On physical examination, neither lymphadenopathy nor splenomegaly was observed except mild hepatomegaly. The bone marrow specimen showed erythroid and megakaryocytic hyperplasia. Laboratory examination revealed autoimmune hemolytic anemia with thrombocytopenia (Evans' syndrome). Donath-Landsteiner test was also positive. An anti-platelet antibody was detected using mixed passive haemagglutination method. Serological tests for syphilis were negative. Serum IgM level was increased and complement levels were decreased.
    Further characterization of the D-L antibody was as follows; Direct Coombs' test at 4°C were positive for IgM and strongly positive for complements. The serum of patient showed strong biphasic hemolysis of the p panel red blood cells. It reacted only weakly with i cord blood samples, on the other hand, it reacted strongly with I blood samples.
    These results suggested that this unique antibody was of IgM and was lacking in anti-P specificity. Anti-I specificity was also suspected.
  • 豊田 恭徳, 殿内 力, 三間屋 純一, 山本 美智子, 坂田 薫代, 中尾 仁一
    1985 年 26 巻 7 号 p. 1135-1139
    発行日: 1985年
    公開日: 2009/02/02
    ジャーナル 認証あり
    A ten-year-old boy was admitted to the Shizuoka Children's Hospital because of pallor and palpitation May 4 1984. On admission, his hematological data showed: RBC 108×104l, WBC 2,500/μl with 700/μl neutrophils, platelet 19,000/μl, and corrected reticulicyte 0.5%. An aspirated bone marrow specimen revealed 24,000/μl nucleated cells with 60% nonhematopoietic elements. A diagnosis of severe aplattic anemia was made.
    He was conditioned by cyclophosphamide and 3 Gy total body irradiation. His anti group A antibody in the blood was removed by immunoadsorbent column consisting of human blood group A antigen bound to crystalline silica for major ABO incompatibility (AB→B). He received 4.9×108/kg bone marrow cells from an HLA-identical eight-year-old brother. No significant hemolytic reaction occurred. A transient rise of hemagglutinin titer occurred on day 15.
    Evidence of engraftment was presented on day 21 with moderate cellular bone marrow.
    Despite intravenous methtrexate for preventing of graft-versus-host disease, he developed grade III acute GVHD in the skin and the liver. Oral cyclosporine A administration induced a marked improvement of skin erythema and jaundice, but chronic GVHD in the liver and oral mucosa persisted. He is in satisfactory hematological remission.
  • 能登 紳之, 菅谷 直樹, 若林 芳久, 広瀬 俊一
    1985 年 26 巻 7 号 p. 1140-1144
    発行日: 1985年
    公開日: 2009/02/02
    ジャーナル 認証あり
    A 42-year-old male was admitted to our hospital with the complains of high grade fever and diffuse superficial lymphadenopathy.
    He was diagnosed as T cell type, non-Hodgikin's lymphoma (diffuse mixed type) by a cervical lymphonode biopsy. Clinical examinations revealed that his clinical stage was IV. He was treated with a conbination of vincristine, cyclophosphamide, 6-mercaptopurine and prednisolone (VEMP therapy).
    Wet cough and high grade fever occurred 16 days after the start of the VEMP therapy. X-ray examination revealed the pneumonic shadow and small cavities in the right lower pulmonary area. Nocardia asteroides were isolated from his sputum a few days later. Broncho-alveolar lavage examination was performed and Nocardia asteroides were isolated from the right S10 area. Therefore the VEMP therapy was stopped, and sulfamethoxazole-trimethoprim therapy was performed. The patient responded to the treatment very well.
  • —急性巨核芽球性白血病と急性骨髄性白血病の2症例—
    石橋 敏幸, 内田 立身, 松田 信, 佐藤 正, 木村 秀夫, 七島 勉, 三田 正行, 鈴木 彰, 刈米 重夫
    1985 年 26 巻 7 号 p. 1145-1150
    発行日: 1985年
    公開日: 2009/02/02
    ジャーナル 認証あり
    Two patients with acute myelofibrosis are reported. All the patients showed pancytopenia, appearance of blast cells in peripheral blood, absence of prominent splenomegaly and tear-drop erythrocytes, and fibrosis on bone marrow biopsy.
    Blast cells in Case I showed a high nucleocytoplasm ratio, cytoplasmic projections and vacuoles. Electron microscopic myeloperoxidase (EM-MPO) was negative, but platelet factor 4 was domonstrated by immunofluorescent method. An autopsy revealed a generalized marrow fibrosis accompanied with the infiltration of megakaryoblasts and megakaryocytes which were also identified by the presence of platelet factor 4 and Factor VIII on bone marrow and other organs.
    Hematological investigation on Case 2 indicated that blast cells were negative in peroxidase, α-naphthyl butyrate esterase, naphthol AS-D chloroacetate esterase, PAS, but positive in EM-MPO. The proliferation of megakaryocytic lineage was not observed.
    Case 1 died rapidly before chemotherapy. In Case 2, an intensive chemotherapy resulted in the transient decrease of blast cells and disappearance of marrow fibrosis by biopsy.
    Case 1 was associated with the proliferation of megakaryoblasts, and a diagnosis is very likely to be acute megakaryoblastic leukemia with secondary fibrosis. In Case 2, the relationship between the blast cells and fibrosis was not clear. Our experiences on these patients suggest that the clinical entity “acute myelofibrosis” is heterogenous in origin, and that detailed analysis of the blast cells is important.
  • 吉川 治哉, 岩崎 泰正, 田原 徹, 佐尾 浩, 山内 辰也, 吉川 敏, 仁田 正和, 社本 幹博, 高本 滋, 上田 龍三, 赤塚 弘 ...
    1985 年 26 巻 7 号 p. 1151-1158
    発行日: 1985年
    公開日: 2009/02/02
    ジャーナル 認証あり
    A 22-year-old woman who had been diagnosed as CML 7 years ago was admitted to our hospital on March 23, 1984, because of lumbago and abdominal distention. She was found to have marked splenomegaly and diagnosed as chronic phase of Ph1-positive CML on admission. Bilateral cervical and submandibular lymphadenopathy appeared suddenly on 12th hospital day and increased in size and number. Biopsy specimens of the cervical lymphnodes were diagnostic of blastic crisis of CML. Despite of vigorous combination chemotherapies, blastic crisis of the bone marrow occurred on 97th day and she died by cerebral bleeding on 120th day.
    Cytochemical studies of the blasts showed focal acid phosphatase reactivity and negative peroxidase. An electron-microscopic study of the blasts from the lymphnodes showed clustered dense bodies in the cytoplasm, which seemed to be one of the most reliable distinct features of the T cells. As karyotype of lymphnode cells, 73% metaphases examined had the Ph1-chromosomes, confirming their origin from CML. With flow cytometric analysis, DNA aneuploidy was detected in the lymphnodes and the bone marrow respectively at the time of involvement of each tissue by blast cells. That indicated the clonal change of leukemic cells cytogenetically along the blastic crisis in this case. Surface marker analyses by monoclonal antibodies showed that blasts of the lymphnodes and the bone marrow at blastic crisis expressed no T-, B- nor myelomono-related antigens, but only 4A and Tp 40 antigens, which exist on pan T cells including prothymocyte.
    Thus, it was strongly suggested the blasts in this CML case were derived from the pre-T cell origin.
  • 松井 俊和, 井野 晶夫, 小野 芳孝, 森川 歌知子, 江崎 幸治, 平野 正美, 長瀬 啓三, 戸谷 徹造, 荒井 昌之
    1985 年 26 巻 7 号 p. 1159-1164
    発行日: 1985年
    公開日: 2009/02/02
    ジャーナル 認証あり
    A case of adult T-cell leukemia (ATL) which developed dissminated strongyloidosis was described. A 43 year old female, a native of the Amamioshima island, with presenting symptoms of lymphadenopathy and unconsciousness was diagnosed as having ATL and hypercalcemia on the basis of abnormal lymphocytosis with convoluted nuclei which had surface characteristics reacting positively with OKT4, lymphnode histology of diffuse pleomorphic infiltration, positive (×320) anti ATLV antibody and high serum calcium level. Daily hydrocortisone and eel calcitonine were administered for hypercalcemia, and 2 week'y doses of cyclophosphamide (800 mg each) and vincristine (2 mg each) for ATL, giving a significant relief of the symptoms. Three weeks after hospitalization, however, she developed fever, severe abdominal pain, headache and pulmonary infiltration followed by unconciousness and respiratory failure; she died one week thereafter. Examination of tracheal aspirates at the terminal stage revealed filariform larvae of strongyloides stercoralis. Autopsy confirmed ATL and disseminated strongyloidosis with abundant filariform larvae being found in intestinal wall, trachea, lungs, pleura and lymphnodes. Brain was not examined. The endemic area of intestinal infestation by strongyloides stercoralis partially overlaps that of increased incidence of ATL. Hyperinfection may occur when filariform larvae penetrate the intestinal wall in the host with decreased defense mechanism as in the case described.
feedback
Top