Estimation of cost and environmental load of fluorocarbon recovery and destruction system based on fluorocarbon stock distribution.

We developed a simple method to estimate the cost and environmental load of a fluorocarbon recovery and destruction (R&D) system for commercial refrigerators (CRs) and building air conditioners (BACs). In order to estimate the fluorocarbon recovery process in detail, we also developed a method to obtain the regional fluorocarbon stock distribution with GIS (geographic information system). Then the distribution of fluorocarbon stock is visualized and the amount of fluorocarbon stock in the region can be calculated. Results show that the cost and CO2 emission of extraction, storage and destruction processes are a major part of the total cost of fluorocarbon R&D system. Also the cost and CO2 emission of a fluorocarbon R&D system of BACs is more than of CRs. This information is useful to devise a plan for the fluorocarbon R&D system and to fairly share the burden of the R&D cost.

[A role of visiting nurses in providing the terminal patients with their desirable life-discussion regarding the place of death of the patients who were registered for visiting nurse system].

AIM: To evaluate the prognosis of the patients who had visiting nurse service and discuss the place of death (life at the terminal stage). To determine the roles of visiting nurses in providing the patients at a terminal stage with their desirable life till death. METHODS: A total of 180 patients, who were registered for their home healthcare service in our Shonan Kamakura General Hospital and died between January 2000 and February 2001, were subjected to the study. All the subjects were classified into 3 groups according to the places of their death, 1) death at home, 2) death in the hospital and 3) death upon arrival after the admission to the hospital. Moreover, the following items were also surveyed and analyzed: 1) diagnosis (name of diseases), 2) cause of death, 3) age, 4) family structure, 5) whether their primary care physicians explained the prognosis and possible expected conditions to the patients and their family before hand, and 6) how the visiting nurses interact with the patients and their family members. RESULTS: Sixty-six patients died at home, 105 in the hospital and 9 upon arrival at the hospital. During this survey period, there were a total of 5,274 and 5,574 visits by primary care physicians and visiting nurses, respectively. The patients who died at home were more often observed in the patients whose primary care physicians explained their conditions to them and whose visiting nurses closely related to them. Moreover, the patients with malignant tumor also more often died at home. On the contrary, there were very few patients with chronic diseases, with whom death at home was accepted and agreed before hand, and there were some cases with chronic diseases who died inside of the ambulance transported on the way to the hospital after a sudden change in their conditions. DISCUSSION AND CONCLUSION: In order to have the patients live their desirable life till their death, it is required for the caretakers to prepare their mind for the day of the patient's death in addition to the patient's own wishes. For the patients with malignant tumor, it is easy to predict their prognosis, thus the caretakers can get prepared for the day of the patient's death. On the contrary, in case of the patients with chronic diseases, it is more difficult for the caretakers to experience an indefinite time with the patients since their prognosis is generally longer but the sudden change in their conditions may give the caretakers a high anxiety. Thus, it is essential for the visiting nurses to play a role as a mediator to interact between the patients and their family members, and their primary care physicians, and to establish a trustful relationship with the patients while their conditions are still stable. Moreover, similar to the malignant patients, the visiting nurses should explain the situations to the patients with chronic diseases, that they can choose the place of their own death and specific medical treatment at emergency and can decide the detail for their terminal stage with their family members. Thus, it was considered to be very important that the visiting nurses should frequently confirm these issues with the patients and their family according to their conditions.

Rearrangement of the rheumatoid factor-related germline gene Vg and bcl-2 expression in lymphoproliferative disorders in patients with Sjögren's syndrome.

Of 250 patients with Sjögren's syndrome (SS) (190 with 1 degree SS and 60 with 2 degrees SS), 60 patients demonstrated lymphoproliferative disorders (LPD): 41 patients had monoclonal gammopathy (MG), 4 had pseudolymphoma, 3 had in situ monoclonal lymphoproliferation, and 12 had malignant lymphoma. Rearrangement of the rheumatoid factor (RF)-related germline gene Vg (associated with the monoclonal RF SF18/2) was demonstrated in 24 of 50 genomic DNAs from the peripheral blood leukocytes of SS patients. It was found in none of 20 DNAs from normal subjects. This suggests an almost 50% incidence of a germline Ig gene rearrangement in SS patients without clinical MG. Lymphocytes composing the lymphoepithelial lesion (LEL) in the major salivary glands of SS patients expressed the oncogene bcl-2 protein in 4 out of 6 patients. The progression of SS from benign to malignant lymphoproliferation may be related to suppression of apoptotic death by bcl-2. These findings suggest that (i) RF clones are activated in SS with little or no somatic mutation resulting in monoclonal proliferation and (ii) the LEL in the salivary gland is one site for monoclonal B cell proliferation and emerging malignant lymphoma.

Lymphomatous polyarthritis in patients with peripheral T-cell lymphoma.

Direct involvement of the joints is a rare complication of malignant lymphoma and lymphoma cells in synovium or synovial fluid have been characterized in only a very few cases. We report two cases of CD4-positive, HTLV-I-negative peripheral T-cell lymphomas that manifested polyarthritis infiltrated with lymphoma cells which we further characterized. Patient 1, with a prior 7-year history of cutaneous T-cell lymphoma (mycosis fungoides) and polyarthralgia, developed pain and swelling in the right knee joint and right femoral region. Patient 2 was initially diagnosed with immunoblastic lymphadenopathy, later rediagnosed as the prodromal stage of T zone lymphoma. Seven years later she developed skin eruptions, cervical lymph node swelling, polyarthritis, and pleural effusion. Synovial fluid analysis in both cases showed predominant CD3+ or cytoplasmic CD3+, CD4+, and CD8- atypical lymphoid cell infiltration. In both cases the T-cell receptor beta and gamma chains were rearranged in the synovial fluid mononuclear cells. Analysis of these two cases and a review of the literature suggest that lymphoma cell infiltration of synovium occurs preferentially in patients with CD4+ peripheral T-cell lymphoma.

[A case of Sjögren's syndrome at the age of 91 with a lymphoepithelial lesion].

A 91 year old woman with Sjögren's syndrome who developed a lymphoepithelial lesion and showed an active state of the disease is described. Since June, 1990, the patient had been complaining of dry eyes and mouth and a left submandibular tumor (1.0 x 1.5 cm in diameter). A biopsy of the tumor revealed lymphoepithelial lesions in the salivary gland. Mild anemia (Hb 9.6 g/dl) and an elevated erythrocyte sedimentation rate (80 mm/hr) were noted. The gamma-globulin level was 2.7 g/dl, IgG 2789 mg/dl, IgA 469 mg/dl, RAHA x80, antinuclear antibody x20, anti-SS-A x256, thyroid test x400 and microsome test x102400. The Schirmer's test showed decreased tear secretion (Lt. 3mm, Rt. 9mm) and keratoconjunctivitis sicca were noticed by an ophthalmologist. Salivary scintigraphy revealed decreased uptake and slow excretion of the isotope (grade II). A biopsy of a minor salivary gland showed periductal lymphocytic infiltration and acinar cell destruction. Immunohistochemical analyses revealed the cross-reactive idiotype of a monoclonal rheumatoid factor which is associated with a patient with Sjögren's syndrome, in the infiltrating lymphocytes and plasma cells of the minor salivary glands, but not in the lymphoepithelial lesions of the left submandibular gland. This was a rare case concerning a 91-year old patient with Sjögren's syndrome who developed a lymphoepithelial lesion and showed high activity in the serum and gives us valuable information on the relationship between aging and autoimmunity.

[Malignant lymphoma developing from the wall of chronic empyema following artificial pneumothorax].

A 60-year-old man who had had chronic empyema following an artificial pneumothorax for pulmonary tuberculosis when he was 26 years old developed malignant lymphoma of the chest wall. The patient was admitted because of right pyothorax as a result of pseudomonas aeruginosa infection and underwent right thoracotomy. During lavage of the right thoracic cavity a tumor was found arising from the empyematic wall. Pathologic examination revealed that it was malignant lymphoma (diffuse large, immunoblastic, B cell type). Treatment with VEAP-Bleomycin elicited a good response. Seven months after chemotherapy, the patient underwent thoracoplasty in addition to packing the cavity with the latissimus dorsi and the greater omentum. Following this, the patient received chemotherapy once a month for one and a half years, after which he was kept under close observation without treatment. Complete remission has now lasted for 49 months since the initial treatment. This is the first reported lymphoma case with closure of the empyematic wall and is remarkable since this patient has remained in complete remission for the last two years without any treatment.

Interleukin-7 (IL-7)-induced proliferation of CD8+ T-chronic lymphocytic leukemia cells.

Interleukin-7 (IL-7) is a growth factor for pro-B cells, pre-B cells, and thymocytes and is known to induce the proliferation of normal human peripheral T cells. Moreover, human B and T acute leukemia cells with immature surface markers proliferate in response to IL-7. Here we describe a case of T-chronic lymphocytic leukemia, in which the leukemic cells showed a proliferative response to human recombinant IL-7 in vitro. The patient was a 74-year-old woman with anemia and thrombocytopenia, whose bone marrow was fibrosed and infiltrated with pathologic cells. Surface markers of the leukemic cells were CD2(+), CD3(+), CD5(+), CD7(+), CD8(+), and CD4(-). Both T-cell receptor beta-chain and gamma-chain genes were found to be rearranged by immunogenotypic analysis. The leukemic cells proliferated in response to IL-7 dose dependently. The DNA synthesis of CLL cells was stimulated by not only IL-7 but also IL-2 and IL-4. The IL-7-induced proliferation was not inhibited by antibodies to IL-2 receptors or the anti-IL-4 antibody. These findings indicate that IL-7 may induce the proliferation of peripheral CD8+ T cells, even on its pathological counterpart.

Light and electron microscopic study of vacuolated cells in immunoblastic lymphadenopathy-like T-cell lymphoma.

Three cases of immunoblastic lymphadenopathy (IBL)-like T-cell lymphoma were analyzed immunologically, and the ultrastructure of mononuclear cells in the lymph nodes and peripheral blood was examined. In the peripheral blood, light microscopic examination revealed vacuolated lymphocytes. These vacuolated lymphocytes formed rosettes with sheep erythrocytes, and they were CD3- and CD4-positive using the avidin-biotin method in cases 1 and 2. Electron microscopic examination revealed two kinds of abnormal lymphocytes. One kind was of B-cell nature with rich lamellated rough-surfaced endoplasmic reticulum and mitochondria. The other kind had a large cytoplasm, in which Golgi apparatus, some endoplasmic reticulum, some mitochondria and a few vacuoles were seen. Some of these vacuoles had remnants of mitochondrial cristae or were enlarged endoplasmic reticulum. The vacuolated T lymphocytes and activated lymphocytes of B-cell nature disappeared with chemotherapy but reappeared with relapse of the disease. These observations suggest that vacuolated cells are related to pale cells in lymph node sections. In other words when these vacuolated cells are found in the peripheral blood of patients with lymphoid malignancies, IBL-like T-cell lymphoma can be suspected.

DNA autosensitivity in two Japanese sisters.

Two cases of DNA autosensitivity in Japanese sisters are reported. Both patients developed painful ecchymoses and other bleeding disorders. Skin tests with autologous leukocyte lysates and calf thymus DNA produced intermediate-type reactions that were identical to spontaneous skin reactions. Pretreatment of DNA with either DNase or chloroquine sulfate inhibited these reactions. Our studies suggest that anti-DNA antibodies might contribute to the clinical symptoms of this disorder.

A high incidence of rheumatoid factor idiotypes in monoclonal proteins in the serum and in lymphoma cells in patients with Sjögren's syndrome.

Patients with Sjögren's syndrome (SS) develop lymphoproliferative disorders such as monoclonal gammopathies and non-Hodgkin's lymphomas. Cross-reactive idiotypes (CRI) were studied in 22 serum monoclonal immunoglobulins (Igs) and in cytoplasmic Ig in four B-cell lymphoma cells in patients with SS. This was done by utilizing monoclonal anti-idiotypic antibodies which were produced against monoclonal rheumatoid factors (RF) derived from three patients with SS and one patient with Waldenström's macroglobulinemia. By the Western blotting or dot immunobinding technique, CRI was detected not only in monoclonal RFs but in monoclonal Igs which had different heavy- or light-chains from the original monoclonal RF used for immunization. A higher incidence of CRI was found in 22 monoclonal Igs associated with SS than in 27 monoclonal Igs in patients with Waldenström's macroglobulinemia, multiple myeloma or malignant lymphoma. In four patients with malignant lymphoma associated with SS, three showed one or three CRI in the lymphoma cells, whereas only two out of 20 patients with other malignant lymphoma showed CRI, demonstrating a significant difference between two groups. These data indicate that monoclonal proliferation of B-cell lineage in patients with SS, benign or malignant, takes place more often among RF-producing clones than other B-cell disorders.

[Ga-67 citrate accumulation in heart in malignant lymphoma of B-cell type accompanied with repeated ECG abnormalities which were normalized with chemotherapy--report of an autopsy case].

A 37y/o male developed mass formation in right tibia in Oct. 1986. He had generalized lymphadenopathy. Diagnosis of malignant lymphoma of B cell type (follicular, large cell type) stage IV B was made because of bone marrow involvement. Surface marker analysis of pathologic cells in the peripheral blood, bone marrow, lymph nodes revealed that they were of B cell type with positive EA gamma rosette formation, positive surface immunoglobulin with IgM-kappa and cytoplasmic immunoglobulin with IgM-kappa and positive responses to monoclonal antibodies of OKIal and B1. He responded partially to VEPAM therapy but in Jan. 1987 he developed Ga accumulation in heart and ECG abnormalities such as depression of ST and inverted T in II, III, AVF, V1-V6 leads. After treatment with VEP, ECG abnormalities disappeared in a month. The same ECG abnormalities recurred once more and disappeared again. Autopsy findings revealed accumulation of lymphoma cells in epicardium and myocardial degeneration in right ventricle. Ga scan appears to be a valuable method for detection of early cardiac involvement by malignant lymphoma.

Hypergammaglobulinemic purpura in patients with Sjögren's syndrome: a report of nine cases and a review of the Japanese literature.

Nine patients with hypergammaglobulinemic purpura associated with primary Sjögren's syndrome were presented. All patients were female (mean age 45.6) and showed recurrent purpura on the lower extremities and typical findings of Sjögren's syndrome with high gammaglobulin and IgG levels, rheumatoid factors, anti-SSA/SSB antibodies (5/5) and anti-nuclear antibodies (6/9). Vasculitis was seen in 6 patients, 4 were of the mononuclear cell type and 2 of the neutrophilic cell type. Six patients had intermediate complexes between 7S and 19S shown by serum ultracentrifugation. Immunofluorescent staining showed immunoglobulin deposition along the blood vessel walls of the skin in these patients. These data suggest that hypergammaglobulinemic purpura in Sjögren's syndrome is vasculitis caused by immunologic processes.

Establishment of two interleukin 6 (B cell stimulatory factor 2/interferon beta 2)-dependent human bone marrow-derived myeloma cell lines.

Two IL-6-dependent human multiple myeloma cell lines, ILKM2 and ILKM3, were established from the bone marrow of patients with IgG-K multiple myeloma. Both cell lines had the typical morphology and immunocytochemical features of myeloma cells. The surface phenotype of both cell lines was PCA-1+, OKT10+, CD10(J-5)-, CD19(B4)-, CD20(B1)-, CD21(B2)-, and OKIa-1-. A monoclonal cytoplasmic Ig, IgG-K or K L chain, was positive in ILKM2 or ILKM3, respectively. EBV nuclear antigen was negative in both cell lines. They proliferated in the presence of macrophages or macrophage-derived factors (MDF). Among the recombinant cytokines examined, IL-6 most strongly augmented the growth of both cell lines. The anti-IL-6 antibody completely inhibited the IL-6-dependent growth and almost completely inhibited the MDF- or purified MDF-dependent growth of both cell lines, ILKM2 and ILKM3 are now being maintained in the culture medium containing 2 ng/ml rIL-6. These results suggest that IL-6 produced by macrophages may play an important role in the growth of myeloma cells in vivo and that macrophages or IL-6 can be used for establishing human myeloma cell lines.

Monoclonal gammopathies in patients with Sjögren's syndrome.

We studied 18 sera of Sjögren's syndrome (SS) with monoclonal gammopathy. Monoclonality was established by typical immunoelectrophoretic findings in all patients and confirmed by idiotypic (Id) studies in 12 patients. Four of the monoclonal gammopathies were of the IgG class, 8 were of the IgA class and 4 were of the IgM class, and 2 patients had 2 M proteins (IgMK/IgGK and IgAK/IgGK). The monoclonal rheumatoid factor (RF) was found in 6 patients (4 IgA and 2 IgM). A review of the literature revealed additional 19 monoclonal gammopathies (2 IgG, 9 IgA, 7 IgM and one Bence Jones protein) in Japanese SS patients. In non-Japanese SS patients, 27 monoclonal gammopathies (4 IgG, 2 IgA, 20 IgM and once Bence Jones protein) were reported. Both Japanese and non-Japanese patients showed a higher incidence of monoclonal gammopathies in primary than in secondary SS. The non-IgM class monoclonal gammopathies were predominant in Japanese SS patients, whereas monoclonal gammopathies were mostly confined to the IgM class in non-Japanese SS patients. These results indicate that monoclonal gammopathy is another significant complication of SS.

Malignant lymphomas in patients with autoimmune diseases: a report of 6 cases and a review of the Japanese literature.

Six patients with malignant lymphomas in autoimmune diseases are described. Four patients who had Sjögren's syndrome (SS) alone or with progressive systemic sclerosis (PSS) or rheumatoid arthritis (RA) developed non-Hodgkin's lymphomas of the B cell type. One patient who had systemic lupus erythematosus (SLE) developed a B cell lymphoma. Another patient with chronic thyroiditis (ChTD) and idiopathic thrombocytopenic purpura (ITP) had a T cell (OKT3/T4) lymphoma. In 5 patients, the autoimmune diseases (2SS, SS/PSS, SS/RA and SLE) preceded B cell lymphomas by one to 11 years. In the patient with ChTD/ITP, ChTD and a malignant lymphoma were found simultaneously. A review of Japanese reports on lymphoproliferative disorders associated with these autoimmune diseases is given. This report offers the suggestion that disorders in the immunoregulatory system caused by autoimmune diseases may predispose lymphoproliferative disorders.

Metastatic calcification in the stomach demonstrated by a bone scan in Bence Jones lambda myeloma.

A 53-year-old male with Bence Jones lambda myeloma developed hypercalcemia and acute renal failure (calcium 14.4 mg/dl, BUN 40 mg/dl, creatinine 3.0 mg/dl) after initial response to chemotherapy. A 99mTc-MDP bone scan revealed unusual isotope accumulation in the left hypochondrium. Extensive calcium deposition was confirmed in the gastric mucosa in the postmortem examination. Detection of gastric calcification by a bone scan is very rare. Only two cases of gastric calcification visualized on bone scans can be found in the literature, one with multiple myeloma) and one in Hodgkin's disease).