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Primary central nervous system lymphoma (PCNSL) is a rare disease, especially among non-AIDS patients. Although almost all PCNSLs belong to the diffuse large B-cell lymphoma (DLBL) category, its clinical course differs from that of other types of DLBL. To elucidate the histogenesis of PCNSL, we analyzed the source of the cells from its variable region (VH) sequences using the polymerase chain reaction (PCR) method to amplify the immunoglobulin heavy chain (IgH) gene of DNA extracted from paraffin sections. Fifteen patients with AIDS-unrelated PCNSL of DLBL type, (7 males and 8 females), were evaluated. Only one case showed positive evidence of EBV infection. The prognosis was very poor with a median survival of 9 months. Analysis of the VH sequences revealed that the VH4 family was used in 4 cases and the VH3 family in 2 cases. The homology with previously published germline sequences was random, ranging from 82.7-93.2%, showing intermediate to high somatic mutations. In 3 of 6 cases, the existence of intraclonal diversity was suspected. These findings suggest that PCNSLs are histogenetically derived from antigen selected B cells in the germinal center (GC) environment.

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Movements of soft tissues surrounding the oral cavity, especially lips and cheeks, have a strong influence on mastication and phonetics. They also influence the relationship between a denture and its oral environment. The purpose of this study was to develop a three-dimensional measuring system for soft tissue movement. This system consisted of two video trackers placed stereographically and a computer. In addition, one video tracker was connected for measuring mandibular movements. The accuracy of this system was evaluated using computerized XYZ pulse stage. The resolution of this system was 0.10 x 0.10 x 0.10 mm, when the measurement was carried out in the area of the 24.0 (X) x 20.0 (Y) x 20.0 (Z) mm with a working distance of 500 mm and a frequency of 120 Hz. In the present study, the lip movements of a dentulous subject with mandibular movements during chewing peanuts were analysed using this system. The new system demonstrated its value for analysing soft tissue movement.

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Patients with pulmonary Mycobacterium avium complex (MAC) infection occasionally have neither past histories of pulmonary diseases nor underlying immunodeficiency conditions. Therefore, we hypothesized that MAC may be linked with a disease-susceptibility gene and determined human leukocyte-associated antigens (HLA) in patients with pulmonary MAC infection. HLA phenotypes were tested in 59 patients with pulmonary MAC infection, and diagnosed according to the criteria of the American Thoracic Society. Data of a Japanese population reported at the Tenth Japan HLA Workshop were used as a control. HLA-A33 (28.8% versus 12.5%, p = 5 x 10(-)(4)) and HLA-DR6 (50.8% versus 20.2%, p = 5 x 10(-)(8)) antigen frequencies in patients with MAC were significantly increased compared with those of the control population. Frequency of a haplotype A33-B44-DR6 in the MAC patients was also significantly increased compared with those of the control population (23.7% versus 4.2%; p = 3 x 10(-)(9)). These data suggest that development of pulmonary MAC infection is associated with specific HLA in a Japanese population.

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The expression of the natural killer (NK) cell antigen, CD56, in hematological malignancies is rare. However, there are several reports that some hematological malignancies, such as T/NK cell lymphoma, multiple myeloma (MM) and acute myeloid leukemia (AML), express this molecule. In B cell non-Hodgkin's lymphomas (NHL), however, very limited number of cases have been reported to express CD56 molecule. Although one study has recently described that half of microvillous B cell lymphoma (MVL), an uncommon subset of large cell lymphoma, expressed CD56, there have been no reports about most common type of B-NHL, diffuse large B cell lymphoma (DLBL) other than a mention of weak CD56 expression in one of 83 DLBL. We herein presented the first case of diffuse large B cell lymphoma expressing CD56 clearly. The immunophenotype determined by immunostaining and flow cytometric analysis was CD10+, CD19+, CD20+, CD45RO-, CD3- and CD56+. On immunohistochemical study, neither bcl-2 nor TIA-1 was positive for tumor cell. Monoclonal immunoglobulin heavy chain (IgH) gene rearrangement was detected, and the sequence analysis of the variable region of IgH (VH) suggested that this tumor was derived from antigen selected post germinal center B cell. Conventional combination chemotherapy (CHOP) was administered, and the patient has still been in complete remission for 10 months.

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There is an increasing incidence of the evolution of myelodysplastic syndrome (MDS) from aplastic anaemia (AA) with immunosuppressive treatment. In paediatric patients G-CSF is also reported to increase MDS evolution, but this process is not precisely understood in children or in adults. Therefore risk factors of MDS evolution in adults are evaluated here. Of 72 patients, five developed MDS. In 47 patients without cyclosporine (CyA) or antithymocyte globulin (ATG) therapy, only one developed MDS with trisomy 8, 242 months after diagnosis. But of 25 patients treated with either CyA or ATG, four developed monosomy 7 MDS within 3 years. Of these 25 patients, 18 were treated with G-CSF and the four patients (22.2%) who developed MDS were found in this group. The cumulative dose and the duration of G-CSF administration were significantly elevated in patients who developed MDS when compared with those who did not, 822.3 +/- 185.0 v 205.4 +/- 25.5 microg/kg (P<0.05) and 187.5 +/- 52.5 v 72.0 +/- 24.6 d (P<0.002), respectively. However these two values for CyA did not differ significantly. Statistically, treatment with CyA, G-CSF and combined G-CSF and CyA were significantly related to MDS evolution. The administration of G-CSF for more than a year was the most important factor (P=0.00). These results suggested that a close relationship exists between G-CSF and subsequent monosomy 7 MDS from AA in adults who receive immunosuppressive therapy. Long-term administration of G-CSF should be prohibited in order to prevent MDS evolution.

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The purpose of this study was (1) to assemble and verify a system to measure the three-dimensional (3-D) movement of the upper and lower complete dentures and the movement of the mandible simultaneously, and (2) to analyse the relation between denture movements and the path of closure of the mandible during function. A 3-D motion capture system with four infrared TV cameras was used for this purpose. The relation between the dentures and the mandibular movements was analysed through the change of the inner product of normal vectors of the denture occlusal planes and mandibular planes. The mandibular movements were classified into two types; the normal stroke (the path of closure was on the ipsilateral side of mastication) and the reverse stroke (on the contralateral side). The results showed that the system could measure the denture mobility within a 0.3 mm error. The mobility of the upper dentures had a correlation to the path of closure of the mandible regardless of the working side or nonworking side, and the lower dentures had a tendency to move toward the working side.

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An 80-year-old woman was referred to our hospital because of irregular genital bleeding. An abnormal mass was found in the uterine cervix, and diagnosed as non-Hodgkin's lymphoma, diffuse large B cell type. Soon after admission, the patient became anuric and was given a diagnosis of acute renal failure due to obstruction of the ureter. She was immediately placed on dose-reduced CHOP and radiotherapy of 15 Gy. As a result, not only did the malignant lymphoma go into remission, but diminished renal function was alleviated. Because malignant lymphoma of the uterus is extremely rare, it exact biocharacteristics are not well understood. We are unaware of any previous report concerning uterine lymphoma complicated by renal failure.

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A 55-year-old Jehova's Witness was treated for acute myelogenous leukemia (AML) by intensive chemotherapy with enocitabine, 6-mercaptopurine and daunorubicin. G-CSF, M-CSF and EPO were subsequently administered. Even though no blood transfusion was given for religious reasons, complete remission was achieved without serious infection and hemorrhage. The total cost for induction chemotherapy was less expensive than is the case for elderly AML patients. This case indicates that the administration of cytokines might reduce the incidence of infection and the necessity for blood products, which would result in favorable cost effectiveness for the treatment of elderly patients with AML.

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A 20-year-old male with ulcerative colitis complicated by mesalazine-associated severe aplastic anemia is described. The patient developed aplastic anemia four months after the start of mesalazine therapy. He was treated with antithymocyte globulin, cyclosporine, and granulocyte colony-stimulating factor (G-CSF) and responded well. Hematological complications of mesalazine are rare, but if bone marrow suppression is detected, immediate cessation of the drug and intensive immunosuppressive treatment with G-CSF should be considered.

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Hemophagocytic syndrome (HPS) presents with fever, pancytopenia, liver dysfunction and increase in hemophagocytic histiocytes in various organs. Although there are two major classifications of HPS in adults, malignant and reactive histiocytosis, it is often very difficult to distinguish between these disorders. We analyzed the laboratory data of patients with HPS to evaluate prognostic factors. Of 34 patients, 14 survived, and 20 died. The median age of survivors was 29.6+/-11.5 yr significantly younger than those who died (54.7+/-17.8 yr). Twenty patients had no obvious underlying disease, the other 13 had hematological malignancies or viral infections. Comparison of laboratory data revealed that nonsurvivors had significantly lower Hb and platelet values on admission. During treatment, worsening of anemia and thrombocytopenia, increase of transaminase and biliary enzymes were similarly more prominent. Risk factors associated with death were: age over 30 yr, presence of disseminated intravascular coagulation, increased ferritin and beta2-microglobulin, anemia accompanied by thrombocytopenia and jaundice. Our data suggests that patients with HPS and any of these risk factors should be treated aggressively with sufficient chemotherapy and supportive care.

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Severe anemia A 37 year-old male with therapy resistant multicentric Castleman's disease (MCD) anemia was treated by subcutaneous injection of erythropoietin. Although immunoglobulin and CRP concentration increased, anemia obviously improved with hemoglobin levels increasing from 4.8 g/dl to 8.5 g/dl without any side effects. Colony assay revealed that the bone marrow mononuclear cells responded to erythropoietin in a dose dependent manner. The mechanism of anemia of MCD is not clearly understood, and treatment is sometimes very difficult. There is no other previous report concerning erythropoietin as a treatment for anemia in MCD.

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Severe aplastic anemia was diagnosed in a 58-year-old female because of pancytopenia with leukocyte count 700/microliters, hemoglobin 3.4 g/dl, platelet count 4.2 x 10(4)/microliters and fatty hypoplastic bone marrow in August 1992. In January 1993, she was admitted with an abdominal skin infection caused by pseudomonas aeruginosa. After treatment of the infection, antilymphocyte globulin was given at a dose of 2,000 mg/day for four consecutive days in July 1993. This resulted in a gradual but steady improvement in her hematological data. In February 1995, her leukocyte count increased to 2,000/microliters, hemoglobin to 15.2 g/dl and platelet count to 11.0 x 10(4)/microliters. Although no splenomegaly or myelofibrosis was found previously, from April 1993, the spleen enlarged and was palpable 10 cm below the costal margin. Her bone marrow biopsy in June 1993 revealed prominent myelofibrosis. Thereafter no changes were found in these features. Splenomegaly and myelofibrosis are characteristic of primary myelofibrosis and although the relationship is uncertain, there is no previous report on aplastic anemia with splenomegaly and myelofibrosis.

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A 59-year-old man was admitted to our hospital because of fever in August 1991. Bone marrow showed normocellularity with 41.5% of CD13, 14, 33 positive blasts, and a diagnosis of AMMoL was made. Laboratory investigation revealed hyponatremia and elevated serum ADH level, indicating the syndrome of inappropriate secretion of antidiuretic hormone (SIADH). Intensive chemotherapy successfully induced hematological complete remission and his serum sodium level became normal. In February 1992, he developed proteinuria and findings were consistent with nephrotic syndrome (NS). Renal biopsy specimen showed membranous proliferative glomerulonephritis and massive infiltration of macrophages, and his serum interleukin 6 level was elevated. Five months later, he suffered from pancytopenia and elevation of biliary enzymes with increase of hemophagocytic histiocytes in his bone marrow (hemophagocytic syndrome). He transiently responded to low dose chemotherapy but he died due to severe infection. It is interesting that association between macrophages and/or cytokines with these various complications was suggested in AMMoL.

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A 19-year-old male who suffered from severe aplastic anemia had been treated with granulocyte colony stimulating factor (G-CSF) from September 1991. Marked increase of hematopoietic cells in his bone marrow was observed, and maintenance administration of G-CSF was continued. 15 months later, myeloblasts with nuclear abnormality increased, and 22 months later, myeloblasts with chromosomal abnormality presenting 46, XY, -7, +21 exceeded 20%, and aplastic anemia seemed to be transformed into refractory anemia with excess of blasts in transformation (RAEB in T). The usefulness of G-CSF in the treatment of aplastic anemia is now established, but there are some reports questioning the effect of long-term administration, especially transformation to MDS with monosomy 7. Leukemic transformation from aplastic anemia is very complex, but in some cases, long term administration of G-CSF may affect the natural course and may lead to the earlier development of leukemia.

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Bilateral ankle skin ulcers developed in a 61-year-old man in the chronic phase of chronic myelogenous leukemia receiving hydroxyurea therapy. The circulating immune complex (anti-C3d antibody) was high in this case, but vasculitis was not observed in the pathological findings of biopsied skin materials. This association has been reported in patients who had chronic myelogenous leukemia or other myeloproliferative disorders and were treated with hydroxyurea. It is likely that skin ulcers are caused by hydroxyurea.

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An occlusal diagnostic system was evaluated for clinical applications. This system consists of pressure sensitive sheets (DENTAL PRESCALE, Fuji Photo Film Co.) and analyzing computer (OCCLUZER FPD703, Fuji Photo Film Co.). The clinical efficiency of this system was evaluated in aged dentates, aged complete denture wearers and an aged patient suffered from periodontitis. The results were as follows: 1. There was a positive correlation between the masticatory efficiency and maximum occlusal force. 2. In the complete denture wearers, the distances from the medial line to the center of the occlusal load were statistically greater in the adjustment needed cases than in the control group. 3. In a case of periodontitis, the full arch fixed splint was adjusted using this system. After adjustment, the maximum occlusal force and contact area increased, and the maximum and medial pressure of the occlusal contact force decreased. 4. It was suggested that this system would be available for the examination of masticatory efficiency and diagnosis of various types of occlusions.

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Septicemia encountered at Kawasaki Municipal Hospital between 1985 and 1986 were studied clinically. Forty six patients had monomicrobial and 5 has polymicrobial infections, respectively. Out of these 46 patients with septicemia, 17 were due to Escherichia coli, 7 were due to Klebsiella pneumoniae and 4 were due to Staphylococcus aureus. Ten patients had hepatobiliary, 7 had hematological, 7 had malignant diseases as underlying diseases, respectively. Out of 10 patients complicated with septic shock, 7 died. Twenty three patients were community acquired infections. The age of most of the patients were over 50. The mortality rate of more than 65-year-old patients were higher than that of other patients. Our of 5 patients with septicemia due to polymicrobial infection, only 1 patient with erythroleukemia died. Fifty patients were treated mainly with beta-lactam antibiotics such as piperacillin or cefmetazole alone or in combination with aminoglycosides and so on. Three patients with infective endocarditis were encountered during this period. Two were due to alpha-streptococcus and 1 was due to Enterococcus. A 41-year-old patient with mitral valve insufficiency and metastatic gastric carcinoma to the bone marrow were complicated with disseminated intravascular coagulation. This patient, however, was successfully treated with a daily dose of 24 mega units of benzylpenicillin, and was given gabexate mesilate, concomitantly.

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