To detect hybrid acute leukemia (HAL), we analyzed the surface markers of leukemic cells in 51 patients with acute leukemia and blastic crisis of chronic myelogenous leukemia (CMLBC). By means of dual staining of surface antigens of leukemic blasts using FACScan, we found there were more HAL cases than expected.
HAL was defined as the state in which leukemic cells had at least co-existence of one myeloid marker and two lymphoid markers. Myeloid markers consisted of CD 13, CD 33 and peroxidase and lymphoid markers consisted of CD10 (CALLA), CD 19, CD 20 and terminal deoxynucleotidyl transferase activity (TdT).
According to this definition, 7 of 20 acute lymphoblastic leukemia (ALL) cases, 2 of 9 CMLBC cases, and 1 of 22 acute non-lymphocytic leukemia (ANLL) cases corresponded to HAL.
Except for one case of ANLL, all HAL cases consisted of biphenotypic leukemia. In most of the biphenotypic leukemia cases, leukemic cells commonly presented CD 10 (CALLA) and CD 19 as lymphoid antigens, and CD 33 as myeloid antigen. In addition to this, leukemic cells in two ALL cases were positive for CD 13 antigen which was considered to be more specific for myeloid lineage. However these cells were positive for CD 10, CD 19 and TdT, and negative for peroxidase, so ALL was diagnosed both phenotypically and with cytochemistry. Pathological cells in one case of CMLBC were positive for CD 10, TdT, CD 13 and CD 33. In one case of ANLL, leukemic cells were peroxidase-positive and M2 of the FAB classification was diagnosed, but those cells were mixed with a small number of peroxidase-negative leukemic cells. The latter cells were positive for CD10 and TdT. Therefore this case was regarded as bilineal leukemia.
These results clearly indicated that HAL occurred at a higher rate than we had expected in acute leukemia and CMLBC.

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Clinical and immunological characteristics of 30 patients with euthyroid ophthalmopathy (EO), who had typical Graves' ophthalmopathy but had never been persistently thyrotoxic, were analyzed.
As for their thyroid states, EO had normal sized-thyroid or small goiter with normal 99 m-Tc pertechnetate uptake, but were found to have low serum TSH concentrations in one third of the studied subjects. Anti-thyroid microsomal antibody was detected in 34%, and TSH receptor antibodies (TBII and TSAb) were also detected in rather low frequency and low titers. These abnormal thyroid findings were not associated each other, and EO was considered to show frequent abnormality on their thyroid.
To clarify whether EO has common genetic back-ground with Graves' or Hashimoto's patients, HLA-typing including class 2 antigens using restriction fragment length polymorphism method was carried out. Comparing to the control subjects, EO was found to be significantly associated with increases of HLA-B 12, B 40 (w 61), DR 9 and DQw 3, and decreases of Cw 1 and Dw 15. Graves' (88 cases) and Hashimoto's (46 cases) patients also showed significant associations with 8 and 4 antigens, respectively. However, except for DQw 3 the indicated antigens in EO did not overlap with those seen by 2 thyroid disorders.
Further, comparisons among these 3 disease groups revealed that EO had essentially different genetic back-ground from 2 thyroid disorders. This finding was confirmed even from comparisons with subgroups of Graves' patients; those with ophthalmopathy, those without family history of diffuse goiter (19 of 23 EO studied did not claim for the family history) and those with small goiter. Comparisons between Graves' and Hashimoto's patients gave non-significant results in most of antigens indicated by the comparisons with the control subjects, and only DPw2 and DPw4 remained significant. This appeared relevant to their intimate relationship.
In conclusion, EO patients have frequent immunological abnormality related to the thyroid, and their incapability to develop into persistent hyperthyroidism is considered to relate with their unique genetic back-ground and also with poor productions of TSH receptor antibodies.

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When heaat-killed Propionibacterium acnes (P. acnes) is intravenously injected into mice, a remarkable infiltration of mononuclear cells, mainly macrophages, in the liver tissue is observed a week later, and when lipopolysaccharide (LPS) is additionally injected into these mice, massive hepatic cell necrosis is induced. In order to clarify the mechanism by which P. acnes causes the infiltration of monomuclear cells in the liver tissue, various fractions of P. acnes were injected into mice and their effects were studied. As a result, when the phenolwater extract of P. acnes obtained by fractionation with the Sepharose 6 B column was injected, the infultration of mononuclear cells was observed, and massive hepatic cell necrosis was subsequently induced in these mice by the injection of LPS.

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Frequencies of HLA class I and II antigen speificities were determined in Japanese patients with Behcet's disease (BD), and their association with various clinical features or immunogenetic predisposition to this disease were studied. Compared to controls, a significantly high frequency of B 51 and a low frequency of DQw 1 were observed in patients with ocular involvement, especially in those who did not have genital ulcer. In patients without ocular lesions, however, no significant difference of HLA antigen frequency was observed compared to the control group. The positive association of B 51 and the negative one of DQw1 were also observed in male patients but not in female patients. When patients were further subdivided into 2 groups according to the age of onset, Dr1 was found to be significantly Frequent in patients with early-onset compared to those with late-onset or controls.
These results suggest that BD is genetically complicated disease and the susceptibility genes in the HLA region are different among male and female patients, among patients with or without ocular lesions, or those with early or late-onset.

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Anti-lymphocyte antibodies, which have been thought to be participated closely in the course of SLE, in sera from 88 patients with SLE and 15 normal controls were detected by cell-ELISA (enzyme linked immunosorbent assay) with human lymphocyte cell lines as the antigen. The antibodies reacted with B cell line _??_Wa_??_ and T cell lines _??_P 12 (CD4^-, CD8⁺), Jurkat (CD4^-, CD8^-) and Hut 78 (CD4⁺, CD8^-)_??_ were detected in sera from 12 to 14 out of 28 patients by cell-ELISA, and each titer of anti-P 12 antibody was correlated with that of anti-Wa antibody (r=0.91, p<0.01), that of anti-Jurkat antibody (r=0.99, p<0.01) and that of anti-Hut 78 antibody (r=0.94, p<0.01). The IgM and IgG antibodies reacted with P 12 cells were detected in sera from 54 and 27 of the 88 patients, respectively. Positive rate of IgG anti-P 12 cells antibody in sera was higher than that of IgM. IgG anti-P 12 antibodies were found to be associated with decrease level of complement (CH 50) in sera (r=0.27, p<0.05) as well as lymphopenia (r=0.33, p<0.01), and all of 10 patients with SLE, who had high titer of IgG anti-P 12 antibody, had lymphopenia. By TLC-immunostaining, the antibodies in sera from 2 of 10 patients with SLE, who had high titer of IgG anti-P 12 antibody and lymphopenia, were found to be reacted with three monosialoglyco-sphingolipids and two neutralglycosphingolipids from P 12 cells.
These results indicate that anti-lymphocyte antibodies in sera from patients with SLE detected by cell-ELISA are reactive not only with suppressor T cells and helper T cells but also with another kinds of T cells and B cells by connection with surface antigens (partially glycosphingolipids) and IgG anti-lymphocyte antibodies might be related with the active phase of SLE.

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Polymyositis in pregnancy is uncommon, especially rare in postpartum. We reported a case of polymyositis associated with postpartum, with literature review. The patient was 28-yearold woman, gave birth to twins prematurely in 25 weeks gestation due to hydramnion and they died after a few days.
She noticed symmetrical muscular weakness in the shoulder and limb girdle at 3 to 4 days postpartum. The CPK level in serum elevated to 10, 089mU/ml, myogenic pattern was shown in EMG, then the patient admitted to our hospital. In the laboratory findings on admission, the elevated levels of CPK, myoglobin, aldolase and creatine were found in serum, and also myoglobin and creatine in urine showed the high level. The patient was diagnosed as polymyositis based on the abnormal pathological findings of muscle biopsy, EMG of limb muscle and above mentioned results. As the value of CPK in serum, however, already decreased to 3, 474mU/ml, she was observed without drugs. She recovered day by day, and she appeared to be improved after 3 weeks.
Although the challenge test of the drugs administered to the patient prior to the initiation of polymyositis, was done, all of them were negative. Furthermore, signs and symptoms of preceding viral infections were not detected. So we considered the disease as polymyositis associated with postpartum, and discussed this case in the literature, compared with other cases.

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We reported a patient with SLE and severe thrombocytopenia who underwent partial splenic embolization (PSE).
This patient was a 30-year-old female on steroid therapy for SLE with positive platelet-associated Ig G (PAIgG) and lupus anticoagulant (LAC).
Following a decrease of the dose of steroids, the platelet count fell significantly to less than 1.0×10⁴/μl. Therefore, we performed semi-pulse therapy with betamethasone (50mg/day for 3 days) on 3 occations and also gave high-dose r-globulin therapy 3 times. No sustained improvement could be obtained, so we next performed PSE since it was too dangerous toresect the spleen. The platelet count is maintained at a level of 5×10⁴/μl after one year from PSE. When there is no response to drug therapy in SLE with impaired renal function and steroid-resistant thrombocytopenia, PSE may be useful because it can increase the platelet count without a severe invasion.

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A case of adult onset Still's disease (AOSD) with the elevation of antistreptolysin O (ASO) and antistreptokinase (ASK) titers is presented.
A 38-year-old female admitted to the Yokosuka National Hospital because of high grade fever (39.6°C), skin eruption, myalgia and arthralgia in November, 1988.
On physical examination, body temperature was 38.4°C. Bilateral knee joints were slightly swollen but not deformed. Cervical lymphadenopathy and salmon-pink coloured rash were seen. Laboratory data showed leukocytosis (14, 300/mm³), anemia, thrombocytosis, elevated erythrocyte sedimentation rate (135mm/h.), positive C-reactive protein (10.3mg/dl) and mild liver function abnormalities. Serological test for rheumatoid factor and antinuclear antibody were not demonstrated. However, highly elevated ASO and ASK titers were noted. Multiple blood, urine, and oropharyngeal cultures were negative.
AOSD was diagnosed by these clinical and laboratory findings.
Low dose methylprednisolone was effective for her clinical signs and laboratory features. ASO and ASK titers were gradually decreased.
It was suggested that systemic response for streptococcal infection might be likely to be implicated in the pathogenesis of this case.

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It is well known that both pernicious anemia and Hashimoto's thyroiditis are organspecific autoimmune disease, and that both sometimes co-exist each other. Now, we experienced a 54-year-old woman complicated with both disease. She complained of general malaise, and she had white hair, an atrophy of lingual papilla, thyroid struma (grade III, elastic hard) and slight disturbance of sensation. Laboratory data was that RBC 1.36×10⁶, MCV 131 fl, vitamin B₁₂ 44.9pg/dl in the peripheral blood, and NCC 2.8×10⁵ and megaerythroblast 15.2% in the bone marrow. Another was that Schilling's test, anti-microsome antibody (Ab), anti-parietal cell Ab and anti-intrinsic factor Ab were positive, but anti-thyroglobulin Ab was negative. These thyrogastric autoimmune diseases are popular in Europe and North America, but they are rare in Japan. The published reports on thyrogastric autoimmune disease are only 26 cases in Japan including our case. The majority of patient is female and the average age is 52.2years old. In 12 cases, patients first suffered from Hashimoto's thyroiditis, and ones did from both disease on almost same time in 9 cases. It is very interesting why some autoimmune disease tend to cluster. So, we described some fact and speculation about the relationship of pernicious anemia and Hashimoto's thyroiditis. And we discussed the reason why two organ-specific autoimmune disease associated each other, i. e. pernicious anemia and Hashimoto's thyroiditis.

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[in Japanese]

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A case of Wegener's granulomatosis with anti-neutrophil cytoplasmic antibody (ANCA) is reported.
A 42-year-old female has complained of nasal pain, nasal bleeding and deformity of nose since September 1988. In March 1989 she complained of fever, hard hearing, cough, conjunctivitis and arthralgia. She was admitted in April 1989 because of further examination.
On admission to our hospital she had scleritis, saddle nose deformity, sinusitis and otitis media. Laboratory findings on admission were as follows: the erythrocyte sedimentation rate was 95mm/hour. CRP 18.8mg/dl, RAHA 1: 2, 560, antinuclear antibody negative, 24-hour urinary protein was 0.7g/day and urinalysis revealed 8 white blood cells and many red blood cells, 1 hyaline cast per field. The creatinine clearance was 40.2l/day. Nasal biopsy showed a granulomatous inflammation with vasculitis.
She was diagnosed as Wegener's granulomatosis and was treated with prednisolone 60mg/day and cyclophosphamide 100mg/day. Her clinical symptoms soon improved but her renal function became progressively worse. In May pulse therapy and plasmapheresis were performed and her renal function gradually improved. Prednisolone was tapered. In June she suffered from infection, and cefmetazole was started. Her liver function became worse, so cefmetazole and cyclophosphamide were discontinued. But her liver function did not improved. We thought that her liver dysfunction was due to fresh frozen plasma in plasmapheresis in May. Cyclophosphamide, 50mg/day, was started again. At present she is in remission.
The titer of ANCA was 1: 32 (by an indirect immunofluorescence assay) on admission, but negative in June when she had infection and in July when Wegener's granulomatosis was inactive. ANCA was first reported in a patient with active Wegener's granulomatosis by Woude et al. in 1985. Our case showed that ANCA was thought to be a serologic marker of diagnosis and disease activity in Wegener's granulomatosis.

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