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T Shichishima

Publications and source records attributed to T Shichishima.

At least 37 records · Page 2Linked to original sources
PubMed1998

[Myositis caused by hydroxyurea in a patient with chronic myelogenous leukemia].

A 59-year-old man with chronic myelogenous leukemia (CML) had a white-blood-cell (WBC) count of 55,400/microliter when admitted in July 1997, and was placed on oral hydroxyurea (HU) of 1,500 mg/day. Treatment with 600 MU/day of interferon alpha (IFN alpha) was started on August 5. HU was discontinued when the patient's WBC count dropped to 8,100/microliter on August 18. However, HU was resumed about a month later, after his WBC count increased to 10,100/microliter, but discontinued when the patient started to complain of chills, high fever, and bilateral femoral pain. HU treatment was initiated again one week later, after the patient's WBC count had begun rising but ceased again after he experienced chills, high fever, and bilateral femoral pain. The patient's myogenetic enzymes were found to be increasing the following day, and a lymphocyte stimulation test (LST) with HU showed a high stimulation index of 41.7%. The elevation of myogenetic enzymes and the results of the LST suggested that myositis due to HU was the cause of the patient's clinical manifestations. His myositis spontaneously disappeared after HU was discontinued. Although the patient is no longer receiving HU, IFN alpha has brought his CML under control. To our knowledge, this is the first reported case of myositis caused by HU.

Antineoplastic Agents↗
PubMed1997

Relationship between the phenotypes of circulating erythrocytes and cultured erythroblasts in paroxysmal nocturnal hemoglobinuria.

To investigate erythropoiesis in paroxysmal nocturnal hemoglobinuria (PNH), we studied the expression of glycosylphosphatidylinositol (GPI)-anchored membrane proteins on circulating erythrocytes and erythroblasts obtained by erythropoietic cell culture in nine patients with this disease. One-color and two-color flow cytometric analyses were performed using monoclonal antibodies for decay-accelerating factor (DAF) and/or CD59/membrane attack complex-inhibitory factor (MACIF). In addition, terminal deoxynucleotidyl transferase-mediated dUTP-biotin nick end-labeling (TUNEL) analysis was performed to assess apoptosis of erythroblasts from six patients. On flow cytometric analysis, cases 1 to 6 had positive and negative erythrocyte populations, case 7 intermediate and negative populations, case 8 positive, intermediate, and negative populations, and case 9 a single double-negative population. In addition, cases 1 to 6 and 8 had positive, intermediate, and negative erythroblast populations, while cases 7 and 9 had intermediate and negative populations. The percentage of double-negative erythrocytes showed a significant correlation with that of double-negative erythroblasts (r = .741, P < .05). In seven of nine patients, more erythroblasts than erythrocytes were negative for the two membrane proteins. Also, some patients with an intermediate population of erythrocytes did not necessarily show an increase of PNH II erythroblasts. Apoptosis of PNH erythroblasts was also detected, but the percentage of apoptotic cells in PNH patients showed no difference from that in healthy volunteers. These findings suggest that the final phenotype of mature erythrocytes in PNH is determined during maturation from erythroblasts to erythrocytes by the disappearance or persistence of PNH II erythroblasts. In addition, PNH erythroblasts in vitro may be partly lost by apoptosis, but apoptosis does not play an important role in determining GPI-linked protein expression.

Adult↗
PubMed1997

Chronic myelogenous leukemia with a hypooctoploid cell population.

A patient with chronic myelogenous leukemia who underwent transformation from the accelerated phase to the chronic phase with interferon-alpha treatment is reported. On admission, the numbers of myeloblasts and promyelocytes in the peripheral blood and bone marrow were 7.0% and 12.8%, respectively. The platelet count was 633.6 x 10(4)/microliter, and megakaryocytes were frequent in the bone marrow. In the accelerated phase, cytogenetic analyses revealed a hypotetraploid and a hypooctoploid cell population with two and four Philadelphia chromosomes, respectively, in the bone marrow specimen. The hypooctoploid clone disappeared with administration of interferon-alpha and the hypotetraploid clone also subsequently disappeared.

Aged↗
PubMed1997

Report on a patient with paroxysmal cold hemoglobinuria.

Antibodies against the human blood group P antigen (anti-P alloantibodies) agglutinate phenotype P1 and P2 erythrocytes treated with papain at 4 degrees C but not phenotype PK and p erythrocytes. This condition is referred to as an autoimmune disease of paroxysmal cold hemoglobinuria (PCH), and the anti-P specificity is found in the cold hemagglutinins. Serum from a patient suspected to be suffering from PCH by the cold auto-agglutination properties was tested for anti-P specificity, using papain-treated O blood group erythrocytes. The serum-mediated hemagglutination and the serum and complement-mediated immunohemolysis were inhibited by globoside (P antigen GalNAc beta 1-3Gal alpha 1-4Gal beta 1-4Glc-ceramide) and Forssman glycosphingolipid (GalNAc alpha 1-3GalNAc beta 1-3Gal alpha 1-4Gal beta 1-4Glc-ceramide). Therefore, we concluded that she had PCH. She was completely cured 6 months later.

Female↗
PubMed1996

In vivo effects of various therapies on complement-sensitive erythrocytes in paroxysmal nocturnal hemoglobinuria.

The percentage of complement-sensitive erythrocytes varies among patients with paroxysmal nocturnal hemoglobinuria (PNH) and is related to disease severity. We examined the changes of complement-sensitive erythrocytes following administration of androgens, prednisolone, dextran, and iron to 12 PNH patients using the complement lysis sensitivity test or flow cytometric analysis of decay accelerating factor and CD59/membrane attack complex-inhibitory factor expression for 11 years. Five untreated PNH patients were also studied as a control group. The complement-sensitive erythrocyte count remained almost constant in the control group, while it increased in four out of five patients receiving androgens. In addition, it decreased in two out of three patients receiving prednisolone, increased in both patients treated with dextran, and increased slightly in two of the three patients receiving iron therapy. Episodes of hemoglobinuria increased in three of the nine patients showing an increase of complement-sensitive erythrocytes, and decreased in four patients receiving prednisolone or dextran. A good response to treatment was clinically observed in four patients receiving androgens, in one patient treated with prednisolone, and in one patient receiving dextran according to the scoring system. These findings suggest that PNH remains stable when the number of complement-sensitive erythrocytes remains fairly constant, and that the PNH III erythrocyte count is especially related to the frequency of hemoglobinuria. Thus, it seems to be important to determine the long-term effect of drug therapy on complement-sensitive erythrocytes to select the most appropriate treatment.

Adolescent↗
PubMed1996

[Myelodysplastic syndrome with multiple chromosome aberrations in a patient with Werner's syndrome].

An unusual case of Werner's syndrome with myelodysplastic syndrome (MDS) in a 63-year-old male is reported. He was transferred to our hospital for evaluation of pancytopenia in August 1994. He had surgery for cataracts at age 47. On admission, his weight was 39 kg and his height was 148 cm. He looked pale, had thin limbs, a bird-like face, loss of hair, a hoarse voice, and atrophic skin with ulcers. A complete blood count showed: hemoglobin 8.7 g/dl; platelet count 1.5 x 10(4)/microliters; and white blood cell count 2,900/microliters with 3% blasts. A bone marrow aspiration revealed hypercellularity with 11.8% blasts and trilineage myelodysplasia. A diagnosis of RAEB was made according to the FAB classification. Cytogenetic analysis of the bone marrow cells revealed multiple aberrations such as 44, XY, -3, -5, add (4) (q?31), add(6) (p2?), del(7) (q22), add(10) (q24), del(12) (q?), add(14) (q32), -15, -16, -17, + mar1, +mar2, +mar3. He died of pneumonia on day 11. Although approximately 700 cases (about 300 cases in Japan) of Werner's syndrome have been reported, the occurrence of hematopoietic malignancies in Werner's syndrome is rare, and the literature includes 8 cases of leukemia and 4 cases of MDS.

Bone Marrow Cells↗
PubMed1995

[Sweet's syndrome in a patient with chronic myeloproliferative disorder during recombinant human granulocyte colony stimulating factor therapy].

A patient with chronic myeloproliferative disorder (CMPD) developed Sweet's syndrome during granulocyte colony-stimulating factor (G-CSF) therapy. A 61-year-old man with essential thrombocythemia was treated with busulfan intermittently since April, 1991. In February, 1993, hepatosplenomegaly with leukoerythroblastosis arose and a diagnosis of myelofibrosis with extramedullary hematopoiesis in the spleen was established. For alleviation of left hypochondralgia due to splenomegaly, he received splenic irradiation in September, 1993. Soon after the irradiation, his peripheral blood revealed pancytopenia and then administration of rhG-CSF was begun on the 9th of October, 1993. One week after G-CSF therapy, he became feverish and painful eruptions on the face and the upper extremities appeared and enlarged. Skin biopsy resulted in a diagnosis of Sweet's syndrome. Treatment with oral prednisone, 30 mg daily, was begun, and rapid and significant improvement of the skin lesions was obtained. The pathogenesis of Sweet's syndrome remains obscure, but careful follow up is necessary for patients during G-CSF therapy with respect to development of Sweet's syndrome.

Chronic Disease↗
PubMed1995

Glycosylphosphatidylinositol (GPI)-anchored membrane proteins in clinical pathophysiology of paroxysmal nocturnal hemoglobinuria (PNH).

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic anemia in which abnormal red blood cells with enhanced susceptibility to complement undergo intravascular hemolysis when complement is activated in vivo, resulting in hemoglobinuria. This enhancement of complement susceptibility appears to involve multipotent stem cells and, in this respect, is thought to closely resemble myelodysplastic syndrome. Recently, it has been reported that the increased susceptibility of PNH cells to complement-mediated lysis is related to a deficiency of complement regulatory membrane proteins, especially CD55 and CD59. Both proteins are glycosylphosphatidylinositol (GPI)-anchored membrane proteins. It was found that a deficiency of GPI-anchored membrane proteins in PNH cells is due to the faulty synthesis of the GPI-anchor which may occur during early synthesis. Moreover, it is suggested that an abnormality of the PIG-A (phosphatidylinositol glycan-class A) gene, which is related to the early stage of GPI-anchor synthesis, is responsible for the pathogenesis of PNH. In conclusion, the clinical expression of PNH is dependent on two factors, complement susceptibility of PNH blood cells and changes in bone marrow function. The search for the ideal treatment of this disease may be aided by resolving the relationship between the two.

Complement Activation↗
PubMed1994

[Negative Ham's test and sugar water test on admission in paroxysmal nocturnal hemoglobinuria].

A 55-year-old man was admitted to our department because of shortness of breath and pancytopenia on March 27, 1992. On admission, palpebral conjuctiva were anemic. Laboratory findings showed pancytopenia, a reticulocyte count of 44,835/microliters, hemosiderinuria, LDH of 710 IU/l, haptoglobin of 6 mg/dl, NAP score of 301, red cell acetylcholinesterase of 1.5 U, negative Ham's test and negative sugar water test. Bone marrow was hypoplastic (2.4 x 10(4)/microliters). The erythrocytes of this patient showed complement-sensitive cells by the complement lysis sensitivity test, and a negative population consisting of double negative erythrocytes by flow cytometric two-color analysis using monoclonal antibodies to CD55 and CD59. A diagnosis of PNH with hypoplastic bone marrow was thus made. Therefore, flow cytometric two-color analysis appears to be superior to the standard tests currently used.

Antibodies, Monoclonal↗
PubMed1994

[A case of acute nonlymphocytic leukemia with leukemic cell infiltration to the uterus followed by bone marrow relapse].

A 65-year old female was diagnosed to have acute nonlymphocytic leukemia (M2) in July, 1990. Complete remission was achieved by BHAC-DMP (enocitabine, daunorubicin, 6-mercaptopurine, prednisolone) therapy. Complete remission had continued without symptoms for about two years. Uterial infiltration of leukemic cells occurred in May, 1992, followed by bone marrow relapse after two months. The second hematological complete remission was obtained after one course of the chemotherapy, but uterial infiltration of leukemic cells had still remained.

Aged↗
PubMed1993

Discordant and heterogeneous expression of GPI-anchored membrane proteins on leukemic cells in a patient with paroxysmal nocturnal hemoglobinuria.

We performed a flow cytometric analysis using monoclonal antibodies to decay accelerating factor (DAF) and CD59/membrane attack complex inhibitory factor (CD59/MACIF) in order to investigate the leukemic cells and erythrocytes from a patient with paroxysmal nocturnal hemoglobinuria (PNH) who developed acute myelocytic leukemia. In May 1990, the leukemic cells comprised 70% of the mononuclear cells in the bone marrow and 76% of those in the peripheral blood. They consisted of a mixture of positive and negative populations, including single DAF-positive cells. In August 1990, almost 100% of the peripheral mononuclear cells were leukemic blasts, and these consisted of a single population with reduced DAF expression. Single-color flow cytometric analysis showed that the leukemic cells lacked CD59/MACIF, while control leukemic cells (n = 3) expressed both DAF and CD59/MACIF. Leukemic blasts from this patient and six control patients expressed lymphocyte function-associated antigen 3 and FcIII receptors (CD 16) both before and after treatment with phosphatidylinositol-specific phospholipase C. The patient's erythrocytes lacking DAF and CD59/MACIF expression corresponded to the proportion of complement-sensitive cells at the onset of acute leukemia. These DAF- and CD59/MACIF-deficient erythrocytes disappeared almost completely with progression of the leukemia. In conclusion, it appears that the expression of glycosylphosphatidylinositol-linked membrane proteins by leukemic cells was heterogeneous and discordant in our patient, and that the leukemic cells were derived from the PNH clone because of their deficiency of CD59/MACIF. It is also suggested that DAF could compete more effectively than CD59/MACIF for a limited number of anchor molecules available on the proliferating leukemic cells.

Antigens, CD↗
PubMed1993

Diagnosis of paroxysmal nocturnal haemoglobinuria by phenotypic analysis of erythrocytes using two-colour flow cytometry with monoclonal antibodies to DAF and CD59/MACIF.

We investigated the relationship between the complement lysis sensitivity test and two-colour flow cytometric analysis using monoclonal antibodies to decay accelerating factor (DAF) and CD59/membrane attack complex inhibitory factor (MACIF) in patients with paroxysmal nocturnal haemoglobinuria (PNH) and other haematological diseases. Flow cytometry showed that all 59 PNH patients had two or three erythrocyte populations, while all 74 patients with other haematological diseases and all 31 healthy volunteers had a single erythrocyte population. We compared the percentage of PNH III erythrocytes in the lysis test with the percentage of negative cells shown by flow cytometry in 52 PNH patients, and found a significant correlation (r = 0.960, P < 0.001). However, in 13 patients the erythrocyte phenotypes did not correspond in both tests. This was generally related to difficulty of detecting PNH II erythrocytes in the lysis test. In the PNH patients the ranges of mean fluorescence intensity for the negative, intermediate and positive erythrocyte populations were respectively 1.1-2.5, 2.2-29, and 61-600 for CD59/MACIF positivity and 1.9-7.2, 3.6-22. and 31-350 for DAF positivity. In contrast, the mean intensities in healthy volunteers ranged from 190 to 720 for CD59/MACIF and from 150 to 350 for DAF. These findings suggest that PNH can be diagnosed and phenotypic analysis of PNH erythrocytes can be performed by respectively assessing the fluorescence profiles and mean fluorescence intensities of both proteins using flow cytometry. Flow cytometry may provide a superior diagnostic method to the traditional tests for PNH.

Adult↗
PubMed1992

[Immunocytochemical staining of decay-accelerating factor (DAF) on erythrocytes: paroxysmal nocturnal hemoglobinuria (PNH)].

Affected erythrocytes in patients with paroxysmal nocturnal hemoglobinuria (PNH) have been detected as complement-sensitive cells by the complement sensitivity assay. Decay-accelerating factor (DAF), a molecular mass of 70 kDa complement-regulatory membrane glycoprotein, has been reported to be deficient on affected PNH blood cells. In the present study, DAF on erythrocytes from 12 patients with PNH were stained by an immunocytochemical method and the ratio of DAF+ erythrocytes was compared with their laboratory data concerning hemolysis, almost all normal human erythrocytes stained positively for DAF. In contrast, various percentages of DAF+ erythrocytes were found in the patients with PNH. The percentages of DAF+ erythrocytes correlated positively with the total amounts of DAF on erythrocytes measured by an enzyme-linked immunosorbent assay (ELISA), negatively with % hemolysis in Ham's test and in sucrose hemolysis assay and with percentages of PNH type III (PNH-III) erythrocytes. In some patients with PNH, subpopulations of erythrocytes weakly-positive for DAF were demonstrated in addition to DAF- erythrocytes. The immunocytochemical method for staining DAF on erythrocytes developed in the present study is useful for the detection of affected PNH erythrocytes and applicable to further studies on membrane defects in PNH.

Adult↗
PubMed1991

Heterogenous expression of decay accelerating factor and CD59/membrane attack complex inhibition factor on paroxysmal nocturnal haemoglobinuria (PNH) erythrocytes.

In order to clarify the characterization of phenotypes of paroxysmal nocturnal haemoglobinuria (PNH) erythrocytes (E), we analysed the expression of decay accelerating factor (DAF) and CD59/membrane attack complex inhibition factor (MACIF) on the membrane surface of PNH-E by means of the flow cytometric method using anti-DAF and/or CD59/MACIF monoclonal antibodies in nine PNH-patients. In two-colour analysis, this expression of PNH-E was classified into three fractions; negative, intermediate and positive according to intensity. The negative fraction was classified into two groups; one group an exclusively negative population, and the other a negative population having slightly DAF-positive E. The intermediate fraction was recognized on PNH-E of cases with PNH II-E and extremely heterogenous. In the positive fraction, this expression was almost the same as on normal E except for case 8. In single-colour analysis for DAF or CD59/MACIF, three fractions were classified as well as the definition in two-colour analysis. In single-colour analysis, the expression on PNH-E was also heterogenous in each fraction and among PNH-patients. However, the intermediate fraction for CD59/MACIF was not found on PNH-E of cases without PNH II-E, although intermediate fraction for DAF was recognized on PNH-E of some cases with PNH III-E in addition to those with PNH II-E. The results suggest that expression of CD59/MACIF and DAF on the membrane surface of PNH-E phenotypes is heterogenous and varies among PNH patients.

Adult↗
PubMed1991

[Successful cholecystectomy in a patient with paroxysmal nocturnal hemoglobinuria].

We report a case of paroxysmal nocturnal hemoglobinuria (PNH) and review the literature regarding transfusion of red blood cells in PNH patients. A 42-year-old woman with myelodysplastic syndrome (MDS) complaining of right hypochondralgia and high fever was admitted to the hospital for acute cholecystitis with gall stone. Blood examination revealed bicytopenia (leukocytes, 1,700/microliters and hemoglobin, 8.5 g/dl) and bone marrow examination showed normocellular but hypererythroid bone marrow and dyshematopoiesis, which suggested MDS. Laboratory data revealed obstructive jaundice and hemolytic anemia. Positive sucrose and Ham tests, which were compatible with the diagnosis of PNH. Cholecystectomy was successful, and the patient showed no postoperative complications of increased hemolysis or thrombosis. As hemoglobin level gradually decreased for the first two postoperative weeks, filtrated white cell-depleted red blood cells (total, 1,000 ml) were transfused instead of washed red blood cells. No side effects of the transfusion were noted. On the basis of findings in this case and those reported in the literature, it is concluded that in some case of PNH, the use of washed red blood cells is unnecessary, and that the use of white cell-depleted red blood cells is indicated.

Adult↗