A 74-year-old woman was admitted to our hospital in March 1998 for low-back pain. In 1990, she had a chemotherapy for diffuse mixed cell lymphoma. Biochemical and serologic assays revealed a total protein level of 9.7g/dl and an IgG level of 4, 530mg/dl.
Immunoelectrophoresis showed monoclonal IgG protein associated with two monoclonal κ and λ light chain components. Bone marrow examination showed proliferation of myeloma cells comprising up to 25% of all nucleated cells. Myeloma cells were immunohistochemically positive for IgG and κ and λ light chains. IgG contained equal amounts of IgG 1 and IgG 2 subtypes and the complementarity determining region 3 (CDR 3) of myeloma cells showed oligoclonality by polymerase chain reaction, suggesting the myeloma cells may have two components. The patient received melphalan and prednisone in combination, resulting in only a minor response. She eventually developed angioimmunoblastic T-cell lymphoma. Biclonal gammopathy associated with malignant lymphoma is rare in case of multiple myeloma and may provide some insight into the pathogenesis of plasma cell tumors.

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A case is reported of a 56-year-old male who presented with bilateral pleural effusion as an initial manifestation of idiopathic fibrosing mediastinitis. The patient showed shortness of breath with severe loss of vital capacity and weight loss. A mediastinal mass surrounding the thoracic aorta and bilateral pleuritis was identified by the chest CT scan. The mass extended, along the abdominal aorta, to the upper portion of retroperitoneum. Laboratory data showed elevated levels of C-reactive protein (CRP), erythrocyte sedimentation ratio (ESR), and IgG. Biopsy of the mediastinal and the pleural mass showed adipose tissue and fibrosis with mild perivascular inflammatory infiltration. A diagnosis of idiopathic fibrosing mediastinitis was made, and 40mg/day of prednisolone was administered. Although CRP and ESR was normalized, the mass size and vital capacity were almost unchanged.

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We experienced a girl with polyarteritis nodosa (PN) diagnosed by myocardial biopsy. The symptoms began with high fever and skin rash. These symptoms and laboratory findings temporarily improved by oral prednisolone, however, she flared up with chest pain about 40 days after onset of the disease. Electrocardiogram indicated the elevation of ST-T levels and low voltage, and blood examination showed remarkable elevation of creatine phosphokinase (CK), white blood cell count (WBC), aspartate aminotransferase (AST) and lactic dehydrogenase (LDH) levels. We suspected systemic vasculitis and damage of coronary artery or/and heart muscle. Finally, she was diagnosed with classical polyarteritis nodosa by myocardial biopsy. Coronary angiograhy revealed no abnormalities. The combination therapy of cyclophosphamide pulses and plasma-exchange was very effective to suppress the disease activity.

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We describe the case of a 61-year-old woman diagnosed with primary Sjögren's syndrome (SS) after an 8-year history of IgA nephropathy and a 3-year history of recurrent purpuric rashes. Her two daughters had previously been diagnosed with other autoimmune diseases. One daughter had Graves'disease and the other had Hashimoto's disease and systemic lupus erythematosus. The diagnosis of SS was made based on dryness of mucous membranes, Shirmer test, and parotid sialography. Thrombocytopenia, high platelet-aggregated IgG (PA-IgG) level, and normal megakaryocytes count in bone marrow suggested that her recurrent purpuric rashes were due to idiopathic thrombocytopenic purpura (ITP). Patients with SS may develop other autoimmune diseases. This case aids understanding of the immune pathogenesis and genetic background of SS.

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70 years old woman was admitted to our hospital for the evaluation of multiple nodular shadows on chest X-ray. She had no clinical symptoms despite of mild increase of LDH and decrease of PaO₂ on laboratory examination. Video associated thoracic surgery was performed to obtain lung tissue for the pathological diagnosis. Pathological findings suggested a diagnosis of lymphomatoid granulomatosis (LYG) for her lung nodules, which was confirmed by rearrangement of IgJH with nested PCR of lung tissue. Nodular shadows in her lung had a trend to regress in accordance with decreases of makers for lymphoma such as sIL-2 R, thymidine kinase, and beta 2-microglobulin without any medication. This is a rare case report of LYG with spontaneous attenuation in the clinical couerse.

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We described two female patients with primary Sjögren's syndrome associated with localized cutaneous nodular amyloidosis (LCNA), in which amyloid protein was derived from immunoglobulin light chain.
Case 1: a 70-year-old female had complained with polyarthralgia, low-grade fever and parotid gland swelling. She was diagnosed as primary Sjögren's syndrome. Three years later she noticed brown color small tumor on the thigh and yellow to brown nodules on the bilateral calves of legs. Skin biopsy from the left thigh revealed amyloid L protein deposi-tion, which was positive for anti-λ light chain staining, in almost entire dermis. Infiltration of lymphocytes and plasma cells around the amyloid deposit were prominent.
Case 2: a 51-year-old female had noticed increasing eruption on the hip. Skin biopsy revealed amyloid L protein deposition in the dermis, which was negative for anti-λ nor κ light chain staining. When she was refereed to our hospital, she complained of xerostomia and xerophthalmia. She was diagnosed as primary Sjögren's syndrome.
In both cases, histological examination of a minor salivary gland biopsy revealed infiltra-tion of lymphocytes and plasma cells but not amyloid deposit. Serum M protein and urine Bence-Jones protein were not detected. These cases represent localized amyloidosis with-out systemic involvement.
It is widely recognized that Sjögren's syndrome is frequently accompanied by B cell lymphoproliferative disorders. In LCNA, infiltration of plasma cells around the amyloid deposits was frequently prominent. The relation between these two disorders is discussed.

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