[Hematological abnormalities of primary Sjogren's syndrome].

Sjogren's syndrome (SS) is an autoimmune disease characterized by a chronic inflammatory response mainly localized to the lacrimal and salivary glands. However, it sometimes involves extraglandular organs culminating in systemic disorders. Hematological abnormalities are not uncommon, although they rarely have clinical significance. In this study we examined 99 patients with primary SS who visited our hospital during 1989 to 1999. Patient's mean age was 54.1 years and 95 out of 99 were female. Lymphopenia and leukopenia was noted in 35 patients (35.3%) and 26 patients (26.2%) respectively, and 7 patients (7.1%) had thrombocytopenia. 43 patients (43.4%) had either of these hematological abnormalities. Patients with lymphopenia showed significantly low frequency of arthralgia and anti-SS-A/B antibody was more common in this group. Only one patient in this group required prednisolone therapy because of polyarthritis and general fatigue while others needed no specific therapy. Patients with thrombocytopenia were significantly younger and a male/female ratio was higher than those without this abnormality. They had higher tendency to accompany with skin eruption, positive anti-SS-B antibody, anti-nuclear antibody and rheumatoid factor. Three out of 8 patients with thrombocytopenia were treated with prednisolone according to the protocol for idiopathic thrombocytopenic purpura. All of 3 patients had positive PA-IgG and normocellular bone marrow. Autoimmune mechanism such as polyclonal B cell activation may play a role in the pathogenesis of thrombocytopenia.

Resident macrophages activated by lipopolysaccharide suppress muscle tension and initiate inflammatory response in the gastrointestinal muscle layer.

A great number of macrophages is found to be evenly distributed in the muscle layer of the gastrointestinal tract. We investigated their effects on smooth muscle contraction and the initiation of immune reactions such as inflammatory responses. Macrophages were demonstrated by the uptake of FITC-dextran and their ultrastructural features were elucidated by electron microscopy. Muscle layers of rats' ilea were incubated with lipopolysaccharide (LPS) for 4-8 h and the force of smooth muscle contraction was measured. The induction effect of inducible nitric oxide synthase (iNOS) on macrophages was then checked by immunohistochemistry. The expression of major histocompatibility complex (MHC) class II was also examined. Macrophages in the muscle layer were confirmed as resident macrophages and were different from a population of dendritic cells. After incubation with LPS, macrophages began to express iNOS and produced NO, and it reduced smooth muscle contraction. iNOS-immunopositive cells increased in a time-dependent manner. Macrophages also began to express MHC class II. The total number of macrophages did not alter after incubation. Results indicate that resident macrophages in the muscle layer induced iNOS as an inflammatory reaction, affected smooth muscle contraction, and initiated immune response in the smooth muscle layer of the gastrointestinal tract, when activated by LPS.

[A case of elevated serum creatine-kinase after anesthesia].

An elevation of creatine-kinase was noted postoperatively in a 50 year-old male who had cerebral aneurysm surgery under isoflurane, N2O and O2 anesthesia. Serum CK concentration reached as high as 5919 IU.l-1 immediately after surgery and elevation was associated with the temperature elevation of above 39.5 degrees C and port-wine urine. The postoperative course was uneventful and elevated serum creatine-kinase was corrected within next 6 days. Since elevated serum creatine-kinase is known to occur in acute stage of cerebrovascular accident, and since the influence of myocardial infarction, malignant hyperthermia and drugs could be neglected, we assumed that an abnormal elevation of CK values observed in the present patient resulted from stimulation of sympathetic nervous system due to cerebral bleeding and to hyperpermeability of sarcolemma of skeletal muscle.

Formation of heterotopic granule cell in mouse cerebellum after neonatal administration of cytosine arabinoside.

The mice, ICR-JCL strain, were injected s.c. with 30 mg/kg body weight of cytosine arabinoside at the age of 2, 3, and 4 days. The external granular layer of these mice was destructed selectively, and subsequently these mice developed abnormal cytoarchitecture in the cerebellum, such as disarrangement of Purkinje cells and heterotopic granule cells in the molecular layer. This study was undertaken to elucidate the mechanism in the formation of heterotopic granule cells. In the cerebellum of the treated mouse, some mossy fibers and glomerular collaterals of climbing fibers extended abnormally even into the molecular layer by the age of 10 days, since no granule cells migrated to the inner granular layer until about 10 days when granule cell production started again in the regenerated external granular layer. Subsequently, these fibers, i.e. axons which extended into the molecular layer, established synapses in the molecular layer with the dendrites of migrating granule cells. These granule cells had no need to migrate to the inner granular layer, and so they remained in the molecular layer as heterotopic granule cells.