Crescentic glomerulonephritis with positive antineutrophil cytoplasmic autoantibody specific for myeloperoxidase associated with autoimmune hemolytic anemia and thrombocytopenic purpura.

A 41-year-old woman was admitted to the hospital with severe uremia, hemolytic anemia, and thrombocytopenic purpura. Emergency hemodialysis with plasmapheresis was started in view of consideration of hemolytic uremic syndrome (HUS), which resulted in improvement of renal function and platelet count. Positive antineutrophil cytoplasmic autoantibody specific for myeloperoxidase (MPO-ANCA) suggested crescentic glomerulonephritis, which was pathologically evidenced by renal biopsy. The diagnosis of MPO-ANCA associated crescentic glomerulonephritis with autoimmune hemolytic anemia (AIHA) and thrombocytopenic purpura were confirmed. Three courses of steroid pulse therapy with heparin were successfully performed, followed by oral prednisolone and warfarin. Such a case has not been previously reported to our knowledge.

Extensive cerebral infarction due to involvement of both anterior cerebral arteries by Wegener's granulomatosis.

The central nervous system is often affected in Wegener's granulomatosis (WG), but massive cerebral infarction due to occlusion of branches of the anterior cerebral arteries (ACA) by granulomatous lesions or thrombosis, or both, has seldom been reported. A case is reported here of a 67 year old man with WG complicated by generalised necrotising vasculitis in the lung, kidney, and gastrointestinal tract, and cerebral infarction in the territory of both anterior cerebral arteries, probably caused by thrombosis and a contiguous invasion of granulomatous lesion from the nasal cavity.

Discordant interleukin 2 production in monozygotic twins concordant for systemic lupus erythematosus.

We present data from monozygotic twins concordant for SLE. IL-2 production by peripheral blood mononuclear cells from the patients, when they were discordant for SLE activity, were measured employing cellular mixing experiments. Macrophages from active SLE had a suppressive effect on IL-2 production. Furthermore, SLE T cells appeared to have a defect in IL-2 production irrespective of disease activity. These defects might play an important role in the disordered immunoregulation in SLE.

Interleukin 2 defect in the peripheral blood and the lung in patients with Sjögren's syndrome.

We studied interleukin 2 (IL-2) production both in the peripheral blood and the lung in patients with Sjögren's syndrome (SS). IL-2 production of the peripheral blood mononuclear cells (PBMC) was significantly impaired in patients with SS (P less than 0.001). The patients with extraglandular disease and with associated connective tissue disease were more defective in IL-2 production. The defect could not be attributable to culture conditions. Both OKT4+ and OKT8+ T cells were deficient in producing IL-2. However, impaired IL-2 production could be partly restored by either (1) adding PMA to the PHA-stimulated culture, or (2) supplementing indomethacin (IM) from the initiation of the culture, or (3) depletion of adherent cells from PBMC. Furthermore, SS T cells were more sensitive to PGE1 than normal controls. In contrast, the response of PBMC to IL-2 was not disturbed in SS. IL-1 production of SS PBMC was not defective although there seemed to be suppressive factor(s) produced by SS adherent cells. In addition, IL-2 production of SS pulmonary lymphocytes was also decreased, suggesting that IL-2 producing cells might not be sequestrated in the lung. These data suggest that qualitative T cell defects and suppressor macrophages might be responsible for defective IL-2 production in SS and that IL-2 deficiency may contribute to the disordered immunoregulation in SS.

Gastric plasmacytoma and multisystem autoimmune disease.

A 54-year-old women with solitary gastric plasmacytoma, Ig M and kappa-light chain type, associated with multisystem autoimmune disease is described. The gastric plasmacytoma developed seven years after the diagnosis of Hashimoto's thyroiditis, primary biliary cirrhosis and Sjögren's syndrome. We speculate that this plasmacytoma developed in association with an immunodeficient and/or immunosuppressed state resulting from multisystem autoimmune disease and therapy.

Malignant histiocytosis: a clinico-pathological study of three autopsied cases.

Clinicopathological features of three male cases with malignant histiocytosis (MH) are described. Case 1, aged 27, had had an indurated swelling of the left mandibular region, histologically being chronic lymphocytic inflammation, with ebb and flow for 4 years prior to the onset of MH, with low grade fever, lymphadenopathy and pulmonary infiltration. Histology of the lymph node was compatible with MH, and only a temporary improvement was obtained by COP therapy. Case 2, aged 32, showed acute febrile onset with severe anemia and splenomegaly. Diagnosis of MH was determined by bone marrow histology. COP therapy appeared effective, but caused severe leukopenia and thrombocytopenia resulting in fatal gastrointestinal bleeding. Case 3, aged 16, had high fever and cutaneous mass of the left chest wall, histology of which suggested MH. Bone marrow biopsy was also diagnostic. Severe pancytopenia allowed only a limited therapy. Morphology of the histiocytes was variable in each case. Diffuse infiltration of neoplastic histiocytes in many organs and erythrophagocytosis in the bone marrow were commonly found in all the cases. The present cases suggested a diagnostic value of bone marrow biopsy and possible effect of antineoplastic combination therapy on earlier stage of MH.

Phytohemagglutinin (PHA) skin test. Characterization of immunological properties and clinical application.

Immunological properties of phytohemagglutinin (PHA) skin reaction were investigated by animal and clinical experiments. In the guinea pigs an intradermal dose of PHA-P produced erythema and induration with a maximal response at 24 hours after the injection. Histologically it was characterized by perivascular infiltration of lymphoid cells in the dermis and subcutis, being similar to that of tuberculin (PPD) skin reaction. PHA skin reaction, however, showed some difference from that of PPD in the initial cellular response in that the former was composed of small mononuclear cells and granulocytes with rapid development and the latter was composed of large mononuclear cells (macrophages) and granulocytes with slow development. Intradermal injection of 1:1000 dilution of PHA-P produced a similar erythema in man. In 39 of 59 patients with connective tissue diseases, the results of the in vivo (skin test) and in vitro (lymphocyte transformation) response to PHA correlated well. In the 59 patients, the incidence of the positive rate of the PHA tests (55.9%) was significantly higher than that of the DNCB test (33.9%) and of the PPD test (23.7%). These observations suggest that the PHA skin test has properties of delayed hypersensitivity and is highly sensitive and that it may be a useful measure of cell-mediated immunity.