Change of paraprotein from IgA to k chain in multiple myeloma.
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Humans↗
Biomedical subjects
S Arimori
Publications and source records attributed to S Arimori.
Differential expressions of CD25 or CD71 on CD4+ and/or CD8+ tumor cells from a patient with adult T-cell leukemia/lymphoma: three color flowcytometric analysis of peripheral blood and lymph node.
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Adult↗
Time course of superoxide generation by leukocytes--the MCLA chemiluminescence system.
This study was performed to examine the pattern of superoxide (O2-.) generation from leukocytes using the O2-. specific chemiluminescence (CL) method. Cypridina luciferin analog, 2-methyl-6-(p-methoxyphenyl)-3,7-dihydroimidazo [1,2-alpha]pyrazin-3-one (MCLA) was used as a CL probe. The appropriate conditions of the MCLA method was first determined for the evaluation of the time course of O2-. generation by leukocytes. The time course of O2-. generation obtained by the MCLA-CL system was compared with that by the luminol-dependent CL, electron spin resonance (ESR)/spin trapping, and cytochrome c systems. Following stimulation by three different stimulants (PMA, OZ, FMLP), leukocytes continuously generated O2-. for up to 5 h in the MCLA-CL system, irrespective of the kind of stimulation. The curves obtained by generation ceased more rapidly in the luminol-CL, ESR/spin trapping, and cytochrome c systems. A 50% activity of the initial value was observed at 70 min in the MCLA-CL system, but 30, 10 and 35 min in the other systems, respectively. The CL or O2-. generation value decreased to less than 1% (possible termination) at 300, 90, 120 and 180 min, respectively. With the exception of ESR studies with OZ, the cell viability was not significantly affected in any of the trials. These results indicate that leukocytes can generate O2-. much longer than previously estimated and that the MCLA-CL-system is the most suitable system for the measurement of the O2-. generation by leukocytes.
Cell Separation↗
Decreased plasma superoxide scavenging activity in immunological disorders--carboxyethylgermanium sesquioxide (Ge-132) as a promoter of prednisolone.
We investigated so-called superoxide scavenging activity (SSA) of plasma in patients with several immunological disorders, such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), polymyo-dermatomyositis (PM), progressive systemic sclerosis (PSS), myasthenia gravis (MG) and autoimmune thyroid disease (AT), using the electron paramagnetic resonance/spin trapping technique. Since carboxyethylgermanium sesquioxide, Ge-132, has been reported to modulate both the immune response and leukocyte functions, we have studied in vivo effect of Ge-132 on plasma SSA and other laboratory parameters in these disorders. The plasma SSA was significantly lower in RA, SLE, PM and PSS, but not in MG and AT, as compared with that in healthy controls. An inverse correlation was observed between plasma SSA and parameters such as erythrocytes sedimentation rates, absolute number of leukocytes, C-reactive protein and serum globulin levels. Furthermore, plasma SSA was significantly decreased in rheumatoid factor-positive patients as compared to negative patients. No correlation was observed between plasma SSA and factors such as ages, sex of patients or the other laboratory parameters, such as serum albumin, triglyceride, cholesterol, hemoglobin and serum iron levels. Patients treated with prednisolone, especially ones with RA, showed an increase of plasma SSA. It appears that Ge-132 promotes prednisolone effects. Our results indicate that a decrease in plasma SSA is not disease specific, but inversely correlates with the severity and activity of inflammation. The methodology to measure plasma SSA presented in this work provides a helpful tool for determining the actual activity of the diseases as well as in vivo studies of antiinflammatory agents.
Adolescent↗
Two-color flow cytometric analysis of thymic lymphocytes from patients with myasthenia gravis and/or thymoma.
Phenotypic characteristics of lymphocyte components of the thymus were determined by two-color flow cytometry using monoclonal antibodies to T cell-differentiation antigens and activation antigens, and B cells in eight myasthenia gravis (MG) thymuses and eight thymomas including four associated with MG to clarify the roles of thymus in the diseases. Fifteen normal thymuses and four thymuses from non-MG patients were used as controls. Phenotypes of lymphoid components in thymoma resembled those in normal thymuses, in which the majority of lymphoid cells were immature (common) thymocytes (CD4+CD8+ or CD1+CD3-). The proportions of immature thymocytes, however, were relatively higher and those of mature thymocytes (CD4+CD8-, CD4-CD8+, CD1-CD3+ or CD2+CD3+) were lower in thymomas than normal thymuses. The results speculate that functions which support further differentiations of immature thymocytes into mature thymocytes are deficient in neoplastic epithelial cells of thymoma. In thymomas, therefore, occasional T cells might escape from the thymic surveillance system which eliminates auto-reactive T cells. Between thymomas associated with MG and those without, however, no significant difference was found in the proportions of thymocytes at various stages of maturation. In nonthymomatous thymuses from MG patients, an increase of both B cells (CD2-DR+ or CD2-CD21+) and activated T cells (CD2+DR+ or CD2+IL-2R+) was observed. Furthermore, immature thymocytes were significantly decreased and mature thymocytes were increased in the MG thymuses, as compared with normal thymuses, non-MG thymuses, and thymomas. These alterations were relevant to the histological findings observed in MG thymuses such as thymic involutions and germinal center formations: decreased immature thymocytes are considered to reflect thymic involutions, while germinal centers, in which lymphocytes from peripheral lymphoid organs are activated, are responsible for the increase of B cells, activated T cells, and mature T cells. In conclusions, our results suggest that neoplastic epithelial cells of thymomas are functionally deficient and that MG thymuses are immunologically active and resemble peripheral lymphoid organs.
Antibodies, Monoclonal↗
[Recurrent case of subacute necrotizing lymphadenitis in young man].
A 10-year-old male was admitted to our hospital because of lymphadenopathy and fever. Biopsy of a cervical lymph node demonstrated nodular necrotic foci containing many macrophages phagocyting the karyorrhexic nuclei. He was diagnosed as subacute necrotizing lymphadenitis (SNL) and all the symptoms disappeared spontaneously within a month. Five year later, lymphadenopathy recurred, and re-biopsy showed the same histological findings and he also recovered spontaneously. Although the prognosis of SNL is generally excellent and the majority of the patients recover without any treatment, recurrences are very rare, especially in young male patient.
Adolescent↗
Serum apolipoprotein H levels in systemic lupus erythematosus are not influenced by antiphospholipid antibodies.
Anticardiolipin antibodies (aCL) were recently discovered to recognize a complex consisting of phospholipids and apolipoprotein H (apo H). In this study, we determined the serum apo H levels in 36 systemic lupus erythematosus (SLE) patients with or without antiphospholipid antibodies (aPL), including aCL and lupus anticoagulants, to clarify the possible effects of aPL on apo H levels in vivo. The apo H levels were low in SLE patients as compared with 22 healthy controls. However, no associations were found between apo H levels and circulating aPL or clinical features of the antiphospholipid antibody syndrome. A secondary hyperlipidemic state, which probably related to lupus nephritis (proteinuria) and/or prednisolone treatment, increased apo H levels in SLE patients.
Adult↗
Essential thrombocythemia developing into refractory anemia and complicated by acute myeloid leukemia.
We report a case of essential thrombocythemia (ET) that climaxed in acute myeloid leukemia after developing into refractory anemia. The male patient had ET that was stable for 8 years on carboquone therapy. However, at the age of 72 years he developed an acute terminal illness that was characterized by severe pancytopenia, circulating myeloblasts, extensive bone marrow infiltration by myeloblasts, and an abnormal karyotype [46, XY, t(8q-; 20q+)]. He subsequently died of severe bilateral pneumonia and heart failure. This case suggests that ET may be similar to polycythemia vera; progression to leukemia is unusual except after chemotherapy. Therefore, treatment of patients with asymptomatic ET may not be advisable.
Aged↗
Acquired chronic pure red cell aplasia successfully treated with intravenous pulse methylprednisolone therapy.
A 65-year-old male patient developed acquired chronic pure red cell aplasia (PRCA) associated with hypergammaglobulinemia and positive Coombs' test during the treatment of eosinophilic pneumonia with prednisolone (PSL). His PRCA was treated with oral PSL at a dose of 60 mg/day for 3 weeks, but anemia further progressed. Immediately after high-dose intravenous pulse methylprednisolone therapy (1 g/day for 3 days) however, reticulocyte crisis occurred and his anemia rapidly improved. He has been in complete remission under a maintenance dose of PSL for more than 2 years. This patient indicates that high-dose intravenous methylprednisolone therapy is one of the useful treatments, not only for constitutional PRCA, but also for acquired chronic PRCA.
Aged↗
Plasma free catecholamine level during hemopurification in a case of pheochromocytoma.
To examine the alteration of the plasma free-catecholamine level during hemopurification in a patient with pheochromocytoma, the levels of plasma free-catecholamine and total catecholamine in the filtrate were monitored pre- and post-hemopurification. Approximately 100 micrograms of total catecholamine was removed by the hemopurification procedure though the high level of plasma-free catecholamine remained unaltered.
Adrenal Gland Neoplasms↗
Thymic carcinoma associated with pinealoma and terminating with peroxidase-negative acute myeloid leukemia.
Thymoma is associated with a wide variety of syndromes. However, an association with peroxidase-negative acute myeloid leukemia and pinealoma, although feasible due to the marked influence of this tumor on the lymphoid system, has not been described previously. A patient with thymic carcinoma and pinealoma who developed peroxidase-negative acute myeloid leukemia as a late event is presented in this report.
Adult↗
Lack of deletion of complement C4 and steroid 21-hydroxylase genes in Japanese patients with primary Sjögren's syndrome.
A null allele at C4A (C4AQO) is associated with primary Sjögren's syndrome (SS) in Japanese. Since a deletion of the C4A and CyP21A genes is reported to account for C4AQO in patients with systemic lupus erythematosus (SLE) in Caucasians, we studied the restriction fragment length polymorphism (RFLP) of genomic DNA to determine whether similar deletions of the C4A and CyP21A genes occur in Japanese patients with SS. Patients with C4AQO did not show the extensive deletion of C4A and CyP21A, which would be recognized by the appearance of 8.5 kb/HindIII and 6.4 kb/TaqI fragments hybridizing with a C4 probe. It is yet to be shown whether the lack of expression of C4A genes in Japanese patients with primary SS is due to point mutations, or to small deletions or insertions that were not detected by the RFLP approach.
Alleles↗
Differential effects of IL-3, GM-CSF and G-CSF in an adult with congenital neutropenia.
Using a methylcellulose culture system, we studied the effects of recombinant human interleukin-3 (IL-3), recombinant human granulocyte-macrophage colony-stimulating factor (GM-CSF), and recombinant human granulocyte colony-stimulating factor (G-CSF) on the growth of myeloid progenitor cells (CFU-C) from an adult patient with congenital neutropenia. The moderate clinical course and the maturation arrest at blast-promyelocyte stage in the marrow differentiated this patient from those described as having Kostmann-type congenital neutropenia. CFU-C growth in bone marrow cells from the patient responded to IL-3 normally in a dose-dependent manner. GM-CSF stimulated only macrophage colony formation in a dose-dependent manner comparable to that in normal subjects. Neither GM-CSF nor G-CSF stimulated any significant granulocyte colony formation. This evidence suggests that the hematopoietic progenitor cells in this patient had the potential for developing CFU-C with IL-3, and that the neutropenia in this patient could be a result of an intrinsic defect in myelopoiesis along a granulocytic pathway responsive to GM-CSF or G-CSF.
Adult↗
Cardiac tamponade in the blastic crisis of chronic myelogenous leukemia.
A 36-year-old man with Philadelphia chromosome-positive chronic myelogenous leukemia (CML) developed hemorrhagic pericarditis with tamponade as a terminal manifestation of the blastic crisis. Cardiac tamponade should be kept in mind as an uncommon cause of death of CML patients. Based on a literature review, symptomatic pericarditis in patients with CML blast crisis suggests imminent death. This is in contrast to long-term survival for patients in the chronic phase.
Adult↗
Cell surface antigens expressed on polymorphonuclear cells in the peripheral blood from patients with inflammatory rheumatic diseases: two-color flow-cytometric analysis.
Cell surface antigens, such as CR3, Fc gamma RIII, cALLa and Leu 8 antigen, expressed on polymorphonuclear cells (PMN) in the peripheral blood (PB) are associated with certain functions of PMN. Functional alterations of PMN have been reported in some rheumatic diseases. In this study, therefore, the cell surface antigens were examined by 2-color flow-cytometry using monoclonal antibodies to clarify whether expressions of the antigens on PMN are abnormal in patients with rheumatic diseases including Behçet's disease (BD), rheumatoid arthritis (RA) and Sjögren's syndrome (SS). We found that expressions of CR3 and cALLa were increased on PB-PMN from patients with BD or RA, but not from those with SS. In BD, we further determined the phagocytic activity of PMN by a flow-cytometric method using fluorescent particles. The phagocytic activity was significantly higher in BD patients than in healthy controls, and correlated slightly with the expression of CR3 on PMN in the patients. In addition, prednisolone or colchicine seemed to decrease the expression of CR3 and/or cALLa in patients. The above results indicated that PB-PMN in BD and RA, but not SS, are phenotypically activated in vivo, and the activation probably reflects several cytokines and/or other stimuli to PMN produced in the inflammatory process.
Adult↗
[Rapid diagnosis of tuberculosis by amplification of mycobacterial DNA in blood diseases].
In patients with malignant blood disease, infection is the most serious complication. The prompt differential diagnosis of tuberculosis leads to an appropriate treatment. A method based on DNA amplification and hybridization for the rapid detection of Mycobacterium tuberculosis was used to test 10 clinical specimens (sputum, gastric aspirate and pleural effusion) from blood disease patients in whom tuberculosis was suspected. Mycobacterium tuberculosis DNA was detected in 3 specimens, including one which was negative on standard microbiological criteria (microscopy and/or culture). The other 7 specimens with fever or abnormal shadow on a chest X-ray were negative by both our method and the Standard microbiological criteria. Rapid diagnosis of tuberculosis by amplification of mycobacterial DNA in cases of blood disease in clinically useful.
Aged↗
HLA class II antigens and DNA restriction fragment length polymorphism in myasthenia gravis in Japan.
Human leukocyte phenotypes and genes in the HLA class II regions were studied in 46 Japanese patients with myasthenia gravis. When the HLA phenotypes of the patients with myasthenia gravis were compared with the controls, an increased frequency of HLA-DRw53 was observed in females less than 30 years of age. The genomic DNAs of the HLA-DRw53-positive patients and DRw53-positive controls were analyzed by using four complementary DNA probes for HLA class II genes. With DQB complementary DNA as the probe, a higher incidence of the 6.5-kb or 8.2-kb BamHI fragment was observed in the patients (76.0%) compared with the controls (19.0%). In contrast, no significant difference was observed between patients and controls when complementary DNAs for DRB, DQA, and DPB were used as probes. These results indicate that the genetic background of Japanese females with early-onset myasthenia gravis is different from other patients with myasthenia gravis, and that DQB genes can greatly influence the onset of myasthenia gravis.
Adolescent↗
Association of the complement allele C4AQ0 with primary Sjögren's syndrome in Japanese patients.
We studied allotypes of the fourth component of complement (C4) and factor B in 76 patients with Sjögren's syndrome (SS) and in 63 normal subjects. C4A-null (C4AQ0) was found in 10 of 28 patients who had primary SS, compared with 1 of 63 control subjects (P less than 0.005). In contrast, no significant difference in the frequency of any C4 allotype was observed between patients with secondary SS and control subjects. An association of HLA-DRw53 with primary SS in Japanese patients has been reported. Since there is no linkage disequilibrium between DRw53 and C4AQ0, it is possible that at least 2 genes in the major histocompatibility complex may determine susceptibility to the development of primary SS in the Japanese population.
Autoimmune Diseases↗