In this study, HL-60, a human granulocyte cell line was differentiated into granulocyte-like cell by DMSO and then the change of the amount of Fc receptors and chemotaxis-associated antigen (TM 316) was examined using monoclonal antibodies and fluorescence analysis of cell sortor. The results showed that the amount of chemotaxis-associated antigen increased slightly and chemotactic activity was poor even after HL-60 was well-differentiated. FcγR I (CD 64) and FcγR II (CD 32) receptors in differentiated HL-60 were slightly recongnized. However, there was no FcγR III B (CD 16 b) receptor in differentiated HL-60 cells. Although DMSO induced HL-60 was morphologically similar to peripheral granulocytes, differentiated HL-60 was different from peripheral granulocytes in terms of chemotaxis and surface receptors.

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Sjögren's syndrome (SS) is a systemic as well as an organ-specfic autoimmune disease, characterized by multiple organ damages, autoantibody production such as rheumatoid factor (RF), and also by the development of lymphoproliferative disorders such as monoclonal gammopathy or malignant lymphoma. We produced two monoclonal anti-idotypic (ld) antibodies (A-SF 18/2 and A-AMB 1/5) against monoclonal RFs (IgA-L and IgM-K) derived from patients with SS. Expression of the cross-reactive idiotype (CRI) of these monoclonal RFs was studied in 101 patients with SS, 71 with rheumatoid arthritis (RA) without complication of SS and 93 normal subjects. The results showed the followings: (1) By the enzyme-linked immunosorbent assay (ELISA), 17.8% and 15.8% of patients with SS and 15.5% and 21.7% of patients with RA showed CRI of SF 18/2 and AMB 1/5, respectively, whereas 6.5% and 5.7% of normal subjects showed these CRIs, respectively, (2) In the SF 18/2 Id system, there was one group (45%) in SS patients who showed weak positive CRI, whereas there was no such group in the AMB 1/5 Id system, (3) Out of 15 patients with SS, there were 4 patients whose peripheral blood lymphocytes had a signficant amount of surface membrane Id of SF 18/2 (9.1-37.7%), suggesting the existence of a monoclonal population in the blood. These data suggest that clones with one particular SF 18/2 Id were markedly activated in SS patients and these may be related to the monoclonal proliferation of RF Id-positive B cells in patients with SS.

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We observed the effects of the retinoic acid (13-cis retinoic acid; 13-cis RA, and all-trans retinoic acid; ATRA) for the cell growth and the expression of CD 25 on peripheral blood mononuclear cells (PBMC) from 17 patients with adult T cell leukemia (ATL). Fourteen had acute type, 1 had chronic type, and 2 had smoldering type of ATL. We divided those patient into 3 groups (hyper-sensitive, sensitive and resistant group) by determined with reduction rate of [³H]-thymidine incorporation obtained before and after treatment with 13-cis RA or ATRA respectively. Growth inhibition was not observed in normal PBMC by 13-cis RA or ATRA. However, no down-regulation of CD 25 expression was observed on PBMC in all patients and normal individuals after treratment with 13-cis RA or ATRA. In the aspect of growth inhibition on PBMC in ATL patients, we tried to clarify the mechanism of the phenomenon. In agarose gel electrophoresis, extracted genomic DNA from retinoic acid treated PBMC in hyper-sensitive and sensitive ATL patients showed multimer DNA fragmentation pattem. On the other hand, genomic DNA from PBMC after treatment with retinoic acid in resistant ATL patients and normal individuals showed high molecular DNA pattern. without fragmentation. Taken together, it is suggested that retinoic acid could induce growth inhibition of PBMC in some ATL patients resulting in DNA fragmentation, apoptosis. We deeply consider that retinoic acid may be an useful agent for ATL patients in clinical aspect.

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The hepatocyte growth factor (HGF) regulates growth, motility, and morphogenesis of epithelial and endothelial cells. It has been reported that serum level of HGF was elevated in patients with having fulminant hepatitis. In inflammatory myopathies (IM), muscle cells are damaged by the inflammatory process and subsequently regenerated. HGF may be involved in the regeneration process of muscle cells in IM.
We examined serum HGF was measured by ELISA from 13 patients with having polymyositis (PM), 18 patients with having dermatomyositis (DM), 3 patients with amyopathic dermatomyositis (ADM) and 14 normal individuals. The muscle of IM patients was examined by immunofluorescence staining using a monoclonal anti-HGF antibody.
The serum HGF level was significantly higher in IM patients (0.63±0.11 (p=0.028) in PM, 0.58±0.07 (p=0.023) in DM) than in normal controls (0.26±0.01ng/ml). However, there was no relationship between the serum HGF level and hepatic enzyme level in IM. The levels of serum HGF were significantly higher in active disease (1.05±0.26ng/ml) than in inactive disease (0.29±0.03 ng/ml) (p=0.044). The serum HGF levels (0.77±0.12ng/ml) were significantly higher in IM patients with pulmonary fibrosis than in those (0.42±0.04ng/ml) without pulmonary fibrosis (p=0.049). There was a positive relationship between serum HGF levels and the presence of opaque fiber and/or regeneration/degeneration fiber in biopsied muscles.
HGF was detected in muscles from IM patients by immunofluorescence.
Serum HGF levels are elevated in IM and correlated with disease activity and complication of interstitial pneumonia.

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A 27-year-old female was admitted to a hospita because of severe anemia (hemoglobin 4.9g/dl) after taking PL^® (a drug for common cold consisted of Salicylamide, Acetaminophen, Caffeine and Promethazine methylene di-salicylate) and Cefadroxil (an oral antibiotic) for ten days. History and laboratory data leaded to a diagnosis of drug induced hemolytic anemia. 6 units of concentrated red blood cells were transfused and the suspected drugs were discontinued immediately. Though resolution of anemia and no further hemolysis were observed, progressive leukocytopenia developed since four days after the admission. Bone marrow aspiration revealed marked decrease of granulocytic series. The patient was transferred to our hospital and was isolated under laminar air-flow to prevent her from bacterial and fungal infections. She was treated with prednisolone and granulocyte-colony stimulating factor. She recovered from leukocytopenia in two weeks without suffering from any life-threatening infection.
We extensively analyzed the suspected drugs and mechanism of hemolysis and granulocytopenia. Cefadroxil is turned out to be contributed to hemolysis by an immune complex mechanism. Cefadroxil and Salicylamide were suggested to be involved in granulocytopenia by the induction of antibodies against the leukocytes to which these drugs were bound. Thus Cefadroxil was regarded as a causative drug of both hemolysis and granulocytopenia. This case is of interest for analyzing drug-induced blood abnormality because it is very rare that two lineage of blood were injured by one drug at the same time as far as we know.

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We report a 33-year-old female with nephrotic syndrome associated with Henoch-Schönlein nephritis (HSN) during pregnancy. She presented purpura in the legs at 20 weeks in her third pregnancy. A biopsy of her purpuric skin lesion showed leukocytoclastic vasculitis. After a month she was admitted to Sapporo City General Hospital because of development of a nephrotic syndrome. She was treated with heparin as anticoaglants therapy and delivered of a healthy girl by Cesarean section at 34 weeks. Renal biopsy, carried out beyond a month after delivery, revealed diffuse proliferative glomerulonephritis with prominent lgA deposits, which made the diagnosis of HSN (grade III of classification of the renal histopathology of HSN from the International Study of Kidney Disease in Childhood). Prednisolone 40mg, dipyridamole 300mg per day and pulse doses of steroid were administrated. Two months later proteinuria was not detected. A sister of the patient had also Henoch-Schönlein Purpura in her childhood. They shared HLA DR 4, DQ 4 which are known to be associated with lgA nephropathy. Fifty percent of pregnant women with chronic glomerulonephritis shows increased proteinuria. Pregnancy may have influence on the increased proteinuria in this case.

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A 41-year-old woman was admitted to Tokyo Women's Medical College Aoyama Hospital in January 1994. She presented cough and dyspnea in September 1991. The diagnosis of interstitial pneumonia was made based on TBLB. Interstitial pneumonia was responsive to initial prednisolone of 40mg daily. When dose of prednisolone was reduced to 15mg daily, she complained of cough and dyspnea again. She was referred to Institute of Rheumatology, Tokyo Women's Maedical College in November 1993. She was diagnosed as systemic sclerosis associated with interstitial pneumonia based on proximal scleroderma and digital pitting scar. Double immunodiffusion and immunoprecipitation assay revealed the presence of anti-Ki, anti-Wa, and anti-RNA polymerases antibodies in the serum, On admission in March 1994, she was treated with intravenous cyclophosphamide therapy at 500mg/day once a mouth. After the third infusion, respiratory symptoms, pulmonary function test values, findings of chest X-ray and CT scan were improved without adverse drug effects.
Intravenous cyclophosphamide therapy seems to be useful in this case. The efficacy of Intravenous cyclophosphamide therapy in the treatment of the interstitial pneumonia associated with systemic sclerosis was discussed.

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A patient with multiple myeloma was treated with several cycles of chemotherapy and developed monoclonal IgA rheumatoid factor.
The monoclonal rheumatoid factor in this case reacted with 2 types of monoclonal antiidiotypic antibody derived from monoclonal rheumatoid factors in a patient with Sjögren's syndrome and a patient with macroglobulinemia.
Two possible mechanisms accounting for the developement of rheumatoid factor activity during a course of chemotherapy are discussed. The first possibility is that the rheumatoid factor or activity had been initially covered by an antiidiotypic antibody but was disclosed by the decreased production of this antibody following by the immunosuppressive therapy. The second possibility is that monoclonal IgA acquired rheumatoid factor activity by a point mutation resulting in a change in the molecular structure of the idiotype.

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The case is a boy who developed progressive dyspnea and had since been followed over a long period time as a case of unknown fever without respiratory symptoms.
He showed mucosal symptoms such as intraoral aphta, eosinophilic pyuria and diarrhea and was clinically diagnosed as hypersensitivity pneumonitis. The affected child showed high levels of circulating immune complex, a decline in DLco on the respiratory function test and a decrease in CD 4/8 in bronchoalveolar lavage lymphocytes; furthermore, an antibody specific to Aureobasidium pullulans was positive in the precipitated antibody test. As for the immune function of the affected child during the follow-up, hypergammaglobulinemia and various immune abnormalities were observed.
We thought the clinical feature of the affected child to be of pathophysiological value in dealing with unknown fever in childhood, so we reported our findings in this paper.

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