AL amyloidosis (AL) is a systemic disorder due to extracellular tissue deposition of amyloid fibrils, composed of immunoglobulin light chains. Since the description of AL involving temporal arteries in 1986, this disorder has been known as one of the differential diagnoses of giant cell arteritis (GCA). We encountered a case of an elderly female presenting with headache and tender and enlarged temporal arteries, that was pathologically diagnosed with temporal artery involvement of AL due to Bence-Jones-type MM. To our knowledge, this was the first case of AL with temporal artery involvement in Japan, that presented with GCA-like features. Literature review of AL cases with temporal artery involvement showed close similarity between these disorders, but suggested that vasculature involvement (extremity claudication, kidney or heart), macroglossia, carpal tunnel syndrome and normal or low (<0.5 mg/dL) CRP levels may predict AL rather than GCA. Physicians should keep in mind that AL involving temporal arteries can be a pitfall in the diagnosis of GCA, as seen in our and previous cases.

A 77-year-old Japanese woman with a 21-year history of seropositive, erosive rheumatoid arthritis (RA) and a 10-year history of methotrexate (MTX) therapy was admitted with malaise and mild consciousness disturbance. Laboratory data showed hypercalcemia, acute kidney injury, normocytic anaemia, and thrombocytopenia. As we first assumed drug-induced toxicity by MTX and eldecalcitol, both were discontinued and leucovorin rescue therapy and calcitonin were administered. However, her condition continued to worsen. Serum protein electrophoresis showed only a small M-peak, immunoelectrophoresis of both the serum and urine demonstrated Bence-Jones kappa (κ) type monoclonal protein without immunoglobulin heavy chain, and bone marrow examination revealed proliferation of plasma cells. We diagnosed her with Bence-Jones κ type multiple myeloma (MM) and transferred her to the department of haematology of a higher order medical institution. Conclusively, the diagnosis of immunoglobulin (Ig) D-κ type MM, a rare variant of this disorder, was determined in accordance with serum immunofixation. Several previous studies have suggested that pre-existing RA is a risk factor for MM. Although IgD MM is characterised by its clinical severity and poor prognosis compared to other subtypes, it is often misdiagnosed or mistaken as light chain type MM, as in the present case, because of the low level of IgD M-protein, resulting in delayed diagnosis. Physicians must take MM into consideration as a differential diagnosis when inactive RA patients present with inexplicable elevated calcium, renal failure, anaemia, and bone lesion symptoms and should be aware of IgD MM to establish the correct diagnosis promptly.

Renal impairment is one of frequent and serious complications in patients with multiple myeloma (MM) and is associated with a higher incidence of infections and early death rate. The catalytic activity of Bence Jones proteins (BJP) affects the clinical processes of patients with MM, and can lead to renal impairment. Scientists point out that BJP have peptidolytic and nucleolytic activity, which can lead porcine kidney proximal tubule (LLC-PK1) to apoptosis in vitro experiments. By treating the cytotoxic BJP with serine protease inhibitor (DFP), BJP lost not only their catalytic activity, but also the cytotoxic effects. Therefore, further research on BJP will helpful to understand the pathogenesis of renal impairment in MM patients and may provide a new idea and measure for the treatment of MM with renal impairment. This article reviews the basic research and progress on the catalytic activity of BJP.

We report two patients who suffered from symmetrical polyarthritis simulating rheumatoid arthritis. Acute phase response was almost within normal limits, and autoantibodies including rheumatoid factor were negative. Both of them were diagnosed as having amyloid arthropathy (AmyA) secondary to kappa multiple myeloma based on deposition of kappa-light chain-immunoreactive amyloid in biopsied tissue and Bence Jones protein in urine. Systemic AL amyloidosis may be important in the differential diagnosis of chronic polyarthralgia.

Isolated Bence Jones proteinuria (BJP) is a distinct form of monoclonal gammopathy of undetermined significance (MGUS) rarely described. The prognosis of the disease is apparently good. We report here a case of idiopathic BJP and review the literature.

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Multiple myeloma is a rare, malignant disease of bone marrow which affects principally the vertebrae, ribs, pelvis and skull but may involve any part of the skeleton. Severe demineralization and destructive lesions of bones, producing severe pain and debility, are distinctive features. The disease is further distinguished by abnormalities of blood proteins and in some cases by the excretion in the urine of Bence-Jones protein, which seldom, if ever, is found in association with any other disease.X-ray examination is frequently helpful. In 22 of 24 cases (of a series of 26 cases) in which films were available, definite abnormalities were noted. Spontaneous fractures, particularly of vertebrae, are common. The diagnosis of the disease rests on the identification of the myeloma cell. This is best accomplished by aspiration of bone marrow. In several of the 26 cases in the series diagnosis was made by a neurosurgeon at the time of operation to relieve pressure on the spinal cord. The myeloma cell has a very characteristic appearance. In the present series the average duration of life after the onset of symptoms was only nine months. One patient, however, survived for at least ten years.

Amyloidosis typically manifests with disseminated infiltration of multiple organ systems. Rarely, amyloidosis may be localized. We report a patient with localized subcutaneous nodular amyloidosis, without systemic amyloid involvement or myeloma, whose presenting symptom was multiple discrete neck nodules. Immunohistochemical analysis showed the amyloid deposits to be derived from lambda light chains. Twenty-four month follow-up showed minimal disease progression. A literature review showed only 5 reported cases of subcutaneous nodular amyloidosis. This is the first description of a patient with subcutaneous nodular amyloidosis derived from lambda light chains. HUM PATHOL 32:346-348.

The demonstration of acquired isolated factor X deficiency and of a biclonal homogeneous lambda and kappa light polypeptide chain proteinuria in a patient of 49 years having an obscure hepatomegaly gave clinical evidence for the rate, amyloidosis-associated factor X deficiency, which could be proven by postmortem examination. The mechanisms by which amyloid may affect factor X levels in this case may be preferentially binding to the amyloid fibrils of lambda light chain type.

A new case of IgE myeloma is described. Patient G.M., a 60-year-old woman, complained with a 3-months\' history of generalized bone pain, weight loss, profound weakness and severe anemia. A bone survey revealed multiple lytic lesions, and a heavy replacement by plasma cells was disclosed in the bone marrow examination of a sternal sample. In addition, an IgE serum M-component and gamma-type Bence Jones proteinuria were identified by immunochemical studies. The disease course was rapid, death occurring within 6 months of clinical onset. A molecular weight of 188,500 daltons was calculated for the purified protein G.M., whereas the molecular weights of its subunits were found to be 72,000 for the epsilon chains and 43,000 for the gamma chain dimers. This case is compared with 15 reported cases of IgE monoclonal gammapathy, an expanding clinical and laboratory spectrum emerging from the review of the literature.