6 cases of systemic lupus erytemtosus (SLE) patients associated leukopenia were studied to evaluate the effect of their IgG fraction and peripheral blood mononuclear cells (MNC) on growth of granulocyte-macrophage progenitor cells (CFU-C) from normal human bone marrow.
IgG fraction markedly suppressed the growth of CFU-C in all cases, although their serum suppressed in 3/6 cases. In 3/4 cases, bosh IgG fraction and MNC suppressed the CFU-C growth. These results suggest that the suppression of granulopoiesis by IgG or MNC may by participating with the pathogenesis of the leukopenia seen in SLE patients.

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A nine year-old girl with juvenile laryngeal papillomatosis (JLP) was treated with systemic administration of alpha-type interferon (IFN-α), and was investigated the immunological response to IFN-α.
The patient had the onset of the disease under two years of age, and was of agressive type with frequent recurrence.
The initial dosage of IFN-α, 1×10⁶ units (alternate day), or 3×10⁶ units (every day), and maintenance dosage of it, 1×10⁶ units (weekly or two times a week), were given to the patient.
Results were as follows.
1. Clinical effects: The patient had no recurrence of the disease for three months laryngoscopically, and was markedly improved in speaking without hoarseness.
2. Immunological effects: IFN-α in vivo inhibited the proliferative response of lymphocytes to mitogens and decreased immunoglobulin secreting cells in peripheral blood lymphocytes on the initialdosage of 3×10⁶ units, IFN-α (every day).
Furthermore, we showed that the addition of IFN-α to the culture in vivo gave the effects on the proliferative response of lmyphocytes to mitogen and immunoglobulin synthesis.
These results indicated that IFN-α should be used in vivo with caution, since it gave various effects on the immune functions.

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A wide variety of clinical and histologic pulmonary manifestations have been documented in patients with systemic lupus erythematosus (SLE). These include infection, lupus pleuritis and atelectasis. However, clinical discriptions are few in number with regard to pulmonary hemorrhage and its etiology. Here we report two cases of SLE patients complicated with pulmonary hemorrhage.
Case 1. A 22-year-old woman suffered from polyarthralgia, butterfly rash, Raynaud's phenomenon, general edema, and later cough, hemosputum and dyspnea. Renal insufficiency with elevated BUN of 140mg/dl and severe proteinuria was noted with a pulmonary X-ray finding of lung edema and pleural effusion. These findings were not improved by the treatment with steroid but improved by dialysis. Autopsy findings showed a severe blood congestion in the lungs with intraalveolar hemorrhage. Alveolar walls were thick and destructed. Rapidly progressive glomerulonephritis was also noted with deposits of IgG and C3.
Case 2. A 24-year-old woman suffered from arthralgia, slight fever, general fatigue and hemosputum. A chest roentgenogram disclosed an interstitial infiltration. No renal damage was found. Autopsy findings showed a blood congestion in the lung and hemorrhage in the alveolar cavity. Membrano-proliferative glomerulonephritis was noted with a slight thickening of basement membrane.
As a cause of hemorrhage, severe congestion due to uremia was considered in case 1 and lupus pneumonitis most probably due to immune deposits, though they were not examined, was considered in case 2.

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Raynaud's phenomenon is an important sign which is occasionally associated with certain connective tissue disorders. This study was designed to establish a quantitative evaluation for Raynaud's phenomenon by means of recording changes in the skin temperature.
Raynaud's phenomenon was induced by immersing both hands in the ice-water in a temperature-monitored room. Thereafter, the finger skin temperature was measured with the thermocupler thermometer for 30 minutes together with the observation of the skin color.
Basal skin temperature (the skin temperature before the immersion) was significantly lower in Raynaud's phenomenon group (28.6±4.2°C, m±S.D.) than in the control group (33.9±1.3°C) (p<0.005).
Basal skin temperature recovery time (the time taken to return to the basal skin temperature after the immersion) was significantly delayed in Raynaud's phenomenon group (29.3±2.9min.) than in the control group (15.5±6.6min.) (p<0.005).
The rate of rise in the skin temperature was significantly lower in Raynaud's phenomenon group (0.34±0.18°C per min.) than in the control, group (1.25±0.39°C per min.) (p<0.005). Both groups could be separated by the borderline at 0.60°C per min.
Changes in the skin color after the immersion in the ice-water were variable without consistent tendency in Raynaud's phenomenon group, whereas there was no particular change in the skin color in the control group.
Oral administration of prostaglandin E₁ (OP-1206 α-CD) improved the rate of rise in the skin temperature in 6 of 11 subjects in Raynaud's phenomenon group.
The foregoing results suggest that the measurement of the skin temperature, especially the rate of rise in the skin temperature, is a useful method in the quantitative evaluation for Raynaud's phenomenon.

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The evidence has been provided that in vitro schistosomula of Schistosoma mansoni activate alternative pathway of complement directly and classical pathway of complement indirectly by binding of IgG antibodies to antigens presented on the schistosomula surfaces.
We had the opportunity to study a patient with left-sided pleural effusion caused by paragonimus. Paragonimus has been said to be present in the pleural space and to have no cyst around its body. We analysed total hemolytic complement titers, levels of complement components, anti-complementary activities and immune complexes in pleural fluids from the patient with paragonimiasis to determine whether paragonimus activates complement system. Total hemolytic complement titers and levels of complement components ware corrected by each corresponding serum titer and each corresponding pleural fluid albumin concentration/serum albumin concentration ratio. Total hemolytic complement titers and levels of three complement components (C1q, C4 and C3) in pleural fluids from the patient with paragonimiasis were lower than each mean value for those in pleural fluids from a group of patients with infectious pleurisy. These were within each mean value for those in pleural fluids from a group of patients with malignant pleurisy, in which complement system, especially classical pathway of complement has been suggested to be activated by malignant tumor cells.
These findings suggested that classical pathway of complement was mainly activated in the pleural space of the patient with paragonimiasis as well as in those of patients with malignant pleurisy. Since immune complexes or anti-complementary activities were not detected in pleural fluids from the patient with paragonimiasis, the complement depletion might be related to the activation of classical pathway of complement by binding of IgG antibodies to antigens presented on the paragonimus surfaces. The possibility was raised that the activation of complement around paragonimus could contribute to killing of paragonimus by eosinophils in the immune host.

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Dysgammaglobulinemia type 1 is one of congenital immunodeficiency syndromes which is characterized by higher or normal serum IgM level and deficiency of IgG and IgA.
Recently we encountered a one-year-old boy who had recurrent respiratory infections and lymphadenitis; IgG and IgA levels in his serum were extremely low in spite of the upper limit of normal range of serum IgM level. Salivary IgA was also deficient. The percentage of EAC-rosette forming cells and surface IgA bearing lymphocytes was lowered. Delayed hypersensitivity skin tests with PHA, PPD and candida antigens were normal. lymphocyte stimulations induced by the mitogens, PHA and pokeweed were also normal.
Treatment of this patient was started with intravenous administration of sulfonated γ-globulin. With this therapy serum IgM level was gradually decreased. γ-globulin replacement therapy was repeated every three or four weeks. As the patient was suffered from interstitial pneumonitis, transfer factor (1 unit/week, 4 units) therapy was also started. During transfer factor therapy the serum IgG levels of this patient was kept at the normal range for three months without γ-globulin replacement therapy.
Although the mechanism of transient recovery of serum IgG level by transfer factor is not clear, we suspect that the switch from IgM to IgG synthesis may have been accomplished by a nonspecific facilitation by transfer factor of T-B cell interaction in our patient.

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We report here a case of systemic cryptococcosis with Lennert's lymphoma. The significance of the titer of the antigen and antibody of Cryptococcus neoformans was studied throughout the clinical course of the patient.
The patient, a 42-year-old female, was admitted to Tsukuba University Hospital in November, 1980, because of fever and skin rash. She had widespread skin rash and lymphadenopathy, and hepato-splenomegaly. A diagnosis of Lennert's lymphoma was made from the patholopy of the biopsy specimen of her right cervical lymphnode. The clinical stage of the disease was IVB. The eight courses of combination chemotherapy brought about a little effect on the disease. However, she subsequently had a sudden onset of high fever, with a severe headache and nausea and further, different, skin rash on the face. C. neoformans were detected from venous blood, cerebrospinal fluid (CSF) and skin secretion. A diagnosis of systemic cryptococcosis was made. She was simultaneously given amphotericin B intravenously and intrathecally for 3 weeks. Thereafter she was further treated with intrathecal amphotericin B for 6 weeks and oral flucytosine for 7 months. Her cryptococcal meningitis immediately improved following the start of the initial therapy. Two months later all the cultures of C. neoformans from CSF, blood and skin secretion was negative. She completely recovered from cryptococcosis and lymphoma and was still well on March 30, 1985.
The antigen titer of C. neoformans in her serum and CSF was serially measured by latex particle agglutination method and the antibody titer by slide agglutination test from December, 1980 to July, 1983. Antigen titer in the serum was negative until 2 weeks prior to the diagnosis of cryptococcosis. The antigen titer was very high when meningitis occured but gradually fell as meningitis recovered clinically. The antibody was never detected in serum and CSF.
These results suggest the following conclusions:
1. C. neoformans antigen titer in the serum indicated the state of systemic cryptococcosis. The titer is, therefore, valuable for the diagnosis and prognosis of the disease.
2. The inability to detect C. neoformans antibody in both her serum and CSF indicates the antibody titer may not be of clinical value in immunodeficient patients.

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Five-year-old girl with immunodeficiency with short-limbed dwarfism is reported. She manifested deficient cell-mediated immunity but normal antibody-mediated immunity. Her T-lymphocyte had maturation arrest at stage I thymocyte.
Two fetal thymus and fetal liver cell transplants were performed on her. Two weeks after the first transplant, mild graft versus host reaction was observed and after four and half months, new HLA antigen was detected. The transplanted fetal stem cells were considered to differentiate in this patient, but no clinical improvement or immunological reconstitution could be observed. The patient continued to deteriorate and expired with interstitial pneumonia five months after the transplantation.
Mild GVH phenomenon and the detectin of new HLA antigen in this patient makes this therapeutic approach promising, and it will be reasonable when histocompatible donors are unavailable.

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The defective C3b receptor (CRl) activity on erythrocytes is frequently seen in systemic lupus erythematosus (SLE) and leukemia. Defective CRl activity on erythrocytes in SLE is thought to be inherited as an autosomal codominant manner. CRl exsists on cell types other than erythrocytes, including neutrophils, eosionphils, monocytes, macrophages, B and some T lymphocytes, mast cells, and glomerular podocytes. The relationship of the expression of erythrocytes with other cell types is unclear. In this study we investigated the relationship of CRl activity between erythrocytes and polymorphonuclear leukocytes (PMN).
The CRl activity on erythrocytes was measured by immune adherence hemagglutination (IAHA) using aggregated human IgG sensitized erythrocytes and guinea pig complement. Patients with SLE were grouped into two, group 1, those who possess CRI activity on erythrocytes, and group 2, those who showed defective CRl activity on erythrocytes.
The activity of CRl on PMN was studied by; 1) rosette formation of PMN by EA (IgM) C3b, and 2) inhibition test of IAHA using EA (IgM) C3b lysate by PMN.
Compared with healthy controls, SLE patients revealed significantly decreased rosette formation.

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