Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
Volume 41, Issue 11
Displaying 1-14 of 14 articles from this issue
  • Ken TOBA, Ichiro FUSE, Tadashi KOIKE, Junjiro TSUCHIYAMA, Wataru HIGUC ...
    2000 Volume 41 Issue 11 Pages 1151-1157
    Published: 2000
    Released on J-STAGE: July 28, 2009
    JOURNAL RESTRICTED ACCESS
    Reticulated platelet (RP) analysis using thiazole orange (TO) and a flow cytometer is a convenient and promising method for estimating platelet kinetics in patients with thrombocytopenia. Among many different modifications of the TO method, a novel protocol reported by Matic in 1998 seems to be superior because it allows RP analysis using a sample of whole blood very quickly and accurately. Therefore, we used this method to analyze platelet kinetics in patients with idiopathic thrombocytopenic purpura (ITP) and hemophagocytic syndrome (HPS), as well as patients with hematopoietic malignancies (HM) after intensive chemotherapy. The proportion of RP was increased in the patients with ITP and HPS, and furthermore showed a negative linear correlation with the platelet count. The change in the proportion of RP occurred about one week before the change in the circulating platelet count in the patients with HM after chemotherapy. This modification of the TO method by Matic is expected to become a standard protocol for RP analysis.
    Download PDF (505K)
  • Naoki TAKAHASHI, Atsuo MARUTA, Chizuko HASHIMOTO, Kazunobu KATO, Juich ...
    2000 Volume 41 Issue 11 Pages 1158-1163
    Published: 2000
    Released on J-STAGE: July 28, 2009
    JOURNAL RESTRICTED ACCESS
    A 45-year-old man was diagnosed as having Ph1+ acute lymphocytic leukemia (ALL) in February 1997. Complete remission was achieved by chemotherapy. Allogeneic BMT from his HLA-identical sister was performed on June 11, 1997. Engraftment was relativly quick, but acute GVHD (grade I) developed. The patient was discharged on day 113. Seven months after BMT, in January 1998, exertional dyspnea developed gradually. Chest X-ray examination showed diffuse interstitial pneumonia, for which corticosteroid was started immediately. The symptoms and signs gradually improved. However, on the 20th hospital day (February 23), bilateral subcutaneous emphysema developed in the neck and supraclavicular region. Chest X-ray and CT examinations showed pneumomediastinum without pneumothorax. The pneumomediastinum and subcutaneous emphysema gradually subsided after 3 weeks of bed rest. Subcutaneous emphysema and pneumomediastinum are relatively rare complications of allogeneic BMT.
    Download PDF (568K)
  • Kazuko KUDO, Keizo HORIBE, Katsuhiko IWASE, Masaru KONDO, Seiji KOJIMA
    2000 Volume 41 Issue 11 Pages 1164-1170
    Published: 2000
    Released on J-STAGE: July 28, 2009
    JOURNAL RESTRICTED ACCESS
    Essential thrombocythemia (ET) is one of the quite rare myeloproliferative disorders in children. The natural course and outcome of this disease have been reported to vary. We report three children (two boys and one girl, mean age at diagnosis 12 yr) with ET who showed different clinical courses. The girl was asymptomatic, but the boys had ankle pain and priapism, respectively. The platelet count ranged between 2300 and 2900×109/L, and the diagnoses were made according to the criteria of the Polycythemia Vera Study Group. The serum thrombopoietin level reached 0.33 and 0.47 fmol/ml in two patients. All three children were administered aspirin or dipyridamole orally. Normalization of the platelet count was observed in two patients, and stable disease persisted in one. The 12 pediatric patients with ET reported previously in Japan demonstrated a low incidence of serious thrombohemorrhagic complications and a favorable outcome, none developing acute leukemia. Careful continuous observation and conservative treatment may be preferable in pediatric patients who do not have cardiovascular symptoms, avoiding the use of potential leukemogens such as alkylating agents and hydroxyurea.
    Download PDF (559K)
  • Masaaki NOGUCHI, Seiki ARUGA, Atsushi KATO, Kazuo OSHIMI
    2000 Volume 41 Issue 11 Pages 1171-1177
    Published: 2000
    Released on J-STAGE: July 28, 2009
    JOURNAL RESTRICTED ACCESS
    In October 1998, a 37-year-old man was admitted because of petechiae and thrombocytopenia following flu-like symptoms. On admission, there were numerous petechiae on the back and extremities. The WBC count was 3,700/μl with 3% atypical lymphocytes, the lymphocyte CD4/CD8 ratio 0.37, and the platelet count 1,000/μl. Mild splenomegaly was detected by CT. Results of blood chemistry and coagulation tests were normal. A bone marrow aspirate was normocellular with no signs of dysplasia, and the megakaryocyte count was normal. The patient was initially diagnosed as having virus infection-associated thrombocytopenia. Steroid therapy was started immediately, and the platelet count rose to 15×104l on day 21. The PA-IgG level was 652 ng/107 platelets. Mild liver dysfunction developed, and atypical lymphocytes increased in number thereafter. Because IgM anti-cytomegalovirus (CMV) antibody (Ab) was positive and IgG CMV Ab was negative, a diagnosis of CMV mononucleosis was made. Gancyclovir was therefore started. Four months later, steroid therapy was discontinued, and partial remission has since been maintained. In healthy individuals, CMV infection is usually latent, and the development of mononucleosis is rare. So far, 11 cases of CMV-induced mononucleosis associated with severe thrombocytopenia have been reported in immunocompetent adults.
    Download PDF (441K)
  • Sumio SAKAMAKI, Yasuo HIRAYAMA, YASUHIRO Nagaoka, Hiroyuki KURODA, Ken ...
    2000 Volume 41 Issue 11 Pages 1178-1182
    Published: 2000
    Released on J-STAGE: July 28, 2009
    JOURNAL RESTRICTED ACCESS
    A 16-year-old boy was diagnosed as having acute lymphoblastic leukemia and underwent autologous stem cell transplantation at the time of his second complete remission. Retardation of platelet recovery was evident at day 90 (<5×104l), and at day 660 after transplantation the platelet count was 8.5×104l. Neutrophils and RBCs showed only slightly retarded recovery. Bone marrow stromal cells, which are thought to play an integral role in megakaryopoiesis, were examined. Although TPO mRNA expression per cell was normal, CFU-F was significantly decreased, resulting in a decrease of total TPO mRNA expression. In contrast, expression of G-CSF mRNA per cell was increased. It was thought that chemotherapy before bone marrow transplantation may have reduced the number of stromal cells, leading to retardation of platelet recovery because of low TPO expression.
    Download PDF (367K)
  • Satsuki IWASE, Shinobu TAKAHARA, Tetsuaki SEKIKAWA, Kiyoshi ITO, Shuji ...
    2000 Volume 41 Issue 11 Pages 1183-1188
    Published: 2000
    Released on J-STAGE: July 28, 2009
    JOURNAL RESTRICTED ACCESS
    We report a case of disseminated MALT lymphoma with macroglobulinemia in an 80-year-old man who presented with a persistent fever. A radiograph of the chest showed infiltration of the left lung and pleural effusion of the right lung. The fluid contained numerous atypical lymphoid cells, which were positive for CD19, CD20, and HLA-DR, and negative for CD5 and CD10. Analysis of a pleural biopsy sample demonstrated no abnormality. A CT scan of the abdomen showed extensive thickening of the wall of the stomach adjacent to a peritoneal mass. Endoscopic examination disclosed antral ulceration. Histopathological examination of gastric samples revealed infiltration by centrocyte-like cells and lymphoepithelial lesions. Serum electrophoresis detected a macroglobulin peak at 34.5 g/l, and immunoelectrophoresis revealed an IgM κ component. A bone marrow aspirate showed infiltration by the same lymphoid cells as those in the pleural fluid. A chromosome study of the lymphoid cells from both the bone marrow and pleural fluid showed a normal karyotype. The final diagnosis was MALT lymphoma involving the stomach, lungs and bone marrow. The patient did not consent to chemotherapy, and instead was given oral prednisolone. He died of respiratory distress one year and four months after diagnosis. Autopsy revealed wide dissemination of the tumor cells.
    Download PDF (502K)
  • Masato YAGITA, Rie TABATA, Yoshiteru KONAKA, Tatsuharu OHNO, Kiyoshi T ...
    2000 Volume 41 Issue 11 Pages 1189-1194
    Published: 2000
    Released on J-STAGE: July 28, 2009
    JOURNAL RESTRICTED ACCESS
    A 67-year-old man was referred to our hospital for treatment of hemophagocytic syndrome. Hypotension, hypoxemia, pleural effusion, severe anasarca, and splenomegaly were noticed at the time of admission. Laboratory findings showed anemia (7.7 g/dl), thrombocytopenia (4.5×104l), an increase of serum LDH (1,466 IU/L) and severe hypoalbuminemia (1.9 g/dl). Bone marrow aspiration revealed an increase of reticulum cells with active hemophagocytosis and the presence of immature lymphocytes (6.0%). Lymphoma was suspected, but effective chemotherapy could not be performed because of progressive hypoxemia and severe hypoalbuminemia, and the patient died of the disease 2 weeks after admission. Autopsy revealed large lymphoid cells packed within systemic vessels as well as invasion into organs such as the liver, lungs, and spleen. The postmortem diagnosis was intravascular large B-cell lymphoma. Hypoalbuminemia and hypoxemia appear to be important clinical features of intravascular large B-cell lymphoma.
    Download PDF (609K)
  • Toru KIGUCHI, Kenji NIIYA, Yasuhide YAMAWAKI, Midori SHIMA, Mine HARAD ...
    2000 Volume 41 Issue 11 Pages 1195-1200
    Published: 2000
    Released on J-STAGE: July 28, 2009
    JOURNAL RESTRICTED ACCESS
    Infrequently, inhibitors against factor VIII can develop in non-hemophiliac patients and cause serious bleeding. In the last year, we have experienced 3 non-hemophiliac patients who developed factor VIII inhibitors. Here, we describe the clinical courses of the three patients and the characteristics of the inhibitors. Case 1: A 69-year-old man underwent a partial gastrectomy because of early gastric cancer, and one month later developed signs of a bleeding tendency such as hematemesis, tarry stools and intramuscular hemorrhage. Blood coagulation tests revealed prolongation of the activated partial thromboplastin time (aPTT), which had been normal on admission. Case 2: A 78-year-old woman with no previous disease history developed generalized subcutaneous purpura. Blood coagulation tests performed on admission revealed a prolonged aPTT. Case 3: A 30-year-old man was admitted to an emergency hospital because of left intrapleural hemorrhage and liver injury caused by a traffic accident. Two months later, a hematoma developed at the site of drainage in the left chest, and blood coagulation tests revealed prolongation of the aPTT, which had been normal on admission. Plasma factor VIII procoagulant activities in cases 1, 2 and 3 were 3%, 1% and 5%, respectively. The respective factor VIII inhibitor titers were 78, 870 and 0.5 Bethesda units/ml. The immunoglobulin class and subclass of the inhibitors examined by an ELISA method were: case 1, IgG1 and 4; case 2, IgG2 and 4 (dominant); case 3, IgG4. In cases 1 and 3, the patients recovered after glucocorticoid therapy, but in case 2 the patient died of intraperitoneal hemorrhage despite receiving two courses of methylprednisolone pulse therapy. The above clinical experience suggests that patients who develop high titers of inhibitors may be refractory to ordinary immunosuppressive therapy, and therefore more aggressive therapy such as plasma exchange and/or bypass therapy using activated prothrombin complex concentrates or an activated factor VII preparation should be considered.
    Download PDF (499K)
  • Noriko IZUMI, Hiroaki KUMAGAI, Tetsuro SHINDO
    2000 Volume 41 Issue 11 Pages 1201-1207
    Published: 2000
    Released on J-STAGE: July 28, 2009
    JOURNAL RESTRICTED ACCESS
    A 16-year-old girl was hospitalized because of anemia and thrombocytopenia in April 1998, and was diagnosed as having AML (FAB: M2). After failure of initial remission induction therapy, she was successfully treated with the MEC regimen as a second-line chemotherapy. On June 22, the first consolidation therapy was started. One week later, the patient developed a high fever with backache. Chest computed tomography (CT) on July 8 showed a 3-cm mass lesion adjacent to the thoracic descending aorta in the left upper lobe. She was given fluconazole and antibiotics, and remained in remission. On July 24, the mass lesion changed to a cavitary lesion on chest CT, suggesting a fungal infection, probably aspergillosis. With recovery from neutropenia, the patient became asymptomatic, and fluconazole was changed to itraconazole. On July 27, she suffered sudden, massive hemoptysis and died. Autopsy revealed a localized adhesion between the cavitary lesion and the thoracic descending aorta, and the aortic wall was ruptured at this site. Microscopic examination revealed invasion of mucormycotic hyphae into the wall of the aorta with infiltration of inflammatory cells. The vasa vasorum were occluded by thrombi, in which mucormycotic hyphae were detected.
    Download PDF (711K)
  • Tetsuhiro FUKUYAMA, Kenichi KOIKE, Masaaki SHIOHARA, Yumi KUROKAWA, Ka ...
    2000 Volume 41 Issue 11 Pages 1208-1213
    Published: 2000
    Released on J-STAGE: July 28, 2009
    JOURNAL RESTRICTED ACCESS
    A 15-year-old girl developed acute lymphoblastic leukemia (ALL). The patient was treated according to the 13th protocol of the Tokyo Children's Cancer Study Group, and thereafter remained free of disease. However, at the age of 20, she complained of polyuria, polydipsia and amenorrhea. Hematological or meningeal relapse was ruled out on the basis of clinical and laboratory findings. The plasma concentrations of GH, TSH, LH, FSH, ACTH and ADH were low or below the detectable limits. There was no increase in urine osmolarity after water deprivation. Arginine, LH-RH, TRH and CRH tolerance tests revealed no or low responses of GH, LH/FSH, TSH, and ACTH/cortisol, respectively. Magnetic resonance imaging demonstrated thickening of the pituitary stalk, which was homogeneously enhanced by gadolinium administration. A biopsy specimen showed fibrosis and infiltration of CD8-positive T lymphocytes in a portion of the pituitary stalk, whereas the adenohypophysis was normal. In addition, no leukemic cells were observed in the samples. Thus, a diagnosis of lymphocytic infun-dibuloneurohypophysitis (LIN) was establishied. All the symptoms were improved by treatment with hydrocortisone, L-thyroxine, desamino-8-d-arginine vasopressin, estrogen and gestagen. This is the first reported case of ALL complicated by LIN.
    Download PDF (777K)
  • Kazutaka TAKAGI, Toru NAKAMURA, Hirofumi MISAKI, Takanori UEDA
    2000 Volume 41 Issue 11 Pages 1214-1219
    Published: 2000
    Released on J-STAGE: July 28, 2009
    JOURNAL RESTRICTED ACCESS
    A 62-year-old man was diagnosed as being in the chronic phase of chronic myelogenous leukemia (CML) in 1990, and subsequently treated with hydroxyurea (HU). The total HU dose administered reached 2,929 g (average, 1.44 g/day). In December 1995, the patient was admitted to our hospital for control of the CML. Following HU therapy, he often experienced high fever (38∼39°C) due to infection or blastosis, and at that time his skin showed marked pigmentation, dryness and scaling with itching and anhidrosis. A skin biopsy sample from the left scapula showed atrophic change of the skin and epidermal tissues with fibrotic changes and damage to the subcutaneous glands. This was strongly suspected to have been caused by the continuous HU administration, and the anhidrosis and dryness was considered to have contributed to the patient's high body temperature. Frequent cooling of the patient's body was effective.
    Download PDF (651K)
  • Teruhiko KOZUKA, Shunichi FUKUDA, Toshi IMAI, Tatsuo SEZAKI
    2000 Volume 41 Issue 11 Pages 1220-1225
    Published: 2000
    Released on J-STAGE: July 28, 2009
    JOURNAL RESTRICTED ACCESS
    Recently, there have been several reports describing patients with multiple myeloma complicated by consciousness disturbance due to hyperammonemia. Here we report a patient with multiple myeloma and hyperammonemia, who died after rapid progression of the disease. A 71-year-old man who had been diagnosed as having Bence Jones protein (κ)-type multiple myeloma in 1996 was readmitted to our hospital in February 1997 because of worsening bone pain, renal dysfunction, and hypercalcemia. Bone marrow aspiration yielded an almost dry tap, and the bone marrow was found to be completely occupied by immature plasma cells. Although liver dysfunction was slight, the serum ammonia level was high and increased gradually. Despite treatment, the patient died due to cerebral embolism and progression of plasmacytic leukemia in October 1997. Peripheral blood sampled at the time of death showed a serum ammonia level of 204 μg/dl, and the myeloma calls were cultured using monolayered bone marrow stromal cells as feeder cells. This led to the successful establishment of a cell line. The level of ammonia in the supernatant was high, indicating that the cultured myeloma cells produced and released ammonia.
    Download PDF (469K)
  • Hidekazu MASUOKA, Koji SANO, Hiroko OHTSUBO, Kaichi NISHIWAKI, Toshio ...
    2000 Volume 41 Issue 11 Pages 1226-1230
    Published: 2000
    Released on J-STAGE: July 28, 2009
    JOURNAL RESTRICTED ACCESS
    A 45-year-old woman was referred to our hospital because of hyperthyroidism complicated by atrial fibrillation and heart failure. Laboratory data revealed pancytopenia, with a white blood cell count of 2,600/μl, red blood cell count of 330×104l, and platelet count of 6.2×104l. The patient had normal transaminase levels, but tests for hepaplastin and cholinesterase showed values of 34% and 1.4 U/ml, respectively, indicating liver dysfunction. There was also decreased excretion of indocyanine green. After initiation of treatment with 30 mg thiamazole and 20mg propranolol daily, the patient's thyroid function normalized and the other abnormal laboratory findings such as pancytopenia and liver dysfunction also disappeared. Pancytopenia is a rare complication of hyperthyroidism. In this case, various laboratory abnormalities were normalized by antithyroid therapy alone, indicating that the hyperthyroidism itself was closely related to the pathogenesis of pancytopenia and liver dysfunction.
    Download PDF (478K)
  • Yoshiki NISHIHORI, Naofumi YAMAUCHI, Kageaki KURIBAYASHI, Yasushi SATO ...
    2000 Volume 41 Issue 11 Pages 1231-1237
    Published: 2000
    Released on J-STAGE: July 28, 2009
    JOURNAL RESTRICTED ACCESS
    An 11-year-old boy was diagnosed as having acute lymphoblastic leukemia (ALL, L1) in 1987 and underwent treatment with an ALL high-risk protocol (prednisolone, vincristine (VCR), daunorubicin, l-asparaginase), which resulted in complete remission. In 1990 he developed chronic hepatitis C and received interferon therapy. In December 1994, ALL recurred, and the patient was treated with VCR. He subsequently developed severe hemolysis (Hb 12.5 g/dl→6.8 g/dl) with increases of indirect bilirubin, AST, and LDH. Furthermore, symptoms resembling a syndrome of inappropriate secretion of ADH (SIADH) and DIC developed. Upon incubation of the patient's red blood cells with VCR in vitro, extreme deformity of the cells was observed. These findings suggested that splenomegaly, due to liver cirrhosis which had developed rapidly from chronic hepatitis C while the patient was in an immunosuppressed state induced by anticancer drugs, had trapped the deformed red blood cells and resulted in severe hemolysis. The patient died on the 165th day after admission due to liver failure.
    Download PDF (658K)
feedback
Top