1/43. poems syndrome, steroid-dependent diabetes mellitus, erythema elevatum diutinum, and rheumatoid arthritis as extramedullary manifestations of plasma cell dyscrasia.poems syndrome is a rare synopsis of different multisystemic disorders (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammapathy, and skin lesions) associated with plasma cell dyscrasia. We herein report the atypical case of a 44-year-old white man presenting with glomerulopathy, poems syndrome, and erythema elevatum diutinum with a few-year history of non-insulin-dependent diabetes mellitus (NIDDM) and seronegative rheumatoid arthritis (RA) as early manifestations of IgAlambda multiple myeloma. The prescription of 1 mg/kg/day prednisone improved the patient's features dramatically. skin lesions improved by the association of glucocorticoids and plasma exchange, recurred when plasmapheresis ceased, and remitted when plasma exchange was reintroduced. NIDDM requiring insulinotherapy recurred when corticoids were discontinued and remitted when prednisone was reintroduced. However, prednisone and plasmapheresis had no effect on polyneuropathy, M-paraprotein, and plasma cell dyscrasia in our patient, who developed indolent multiple myeloma a few years later. We thus concluded that poems syndrome, steroid-dependent diabetes mellitus, rheumatoid arthritis, RA, and skin vasculitis in our patient were triggered by plasma cell dyscrasia.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
2/43. Clonal expansion of gammadelta-T lymphocytes in an HTLV-I carrier, associated with chronic neutropenia and rheumatoid arthritis.We report on an HTLV-I carrier showing clonal proliferation of gammadelta-T lymphocytes associated with chronic neutropenia and rheumatoid arthritis (RA). A 75-year-old Japanese woman had a 20-year history of RA and was found to have neutropenia and lymphocytosis by routine examinations. Her white cell count was 5,800/microl with 89% lymphocytes. The proliferating gammadelta-lymphocytes did not show the typical morphology of large granular lymphocytes (LGL) and were positive for CD3, TCRdelta1, and HLA-DR but negative for CD4, CD8, and deltaTCS1. Clonally rearranged TCRgamma-chain (Jgamma) and TCRbeta-chain (Cbeta1) genes were detected by Southern blot analysis. Clonality of these proliferating gammadelta-T cells was confirmed by CDR3 size analysis for the TCRdelta-chain. Anti-HTLV-I antibody was positive and the pX region of HTLV-I proviral dna was detected by PCR analysis, but clonal integration of HTLV-I proviral dna was not detected by Southern blotting analysis. The patient's clinical course has been stable, except for infrequent infectious episodes. The association of HTLV-I/II infection with T-LGL leukemia has been reported by several groups, although most cases exhibit TCRalphabeta type T cells. Analysis of the junctional sequence of TCR on T-LGL leukemia cells may clarify the role of HTLV-I/II infection in clonal T-cell proliferation.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
3/43. A clinico-pathological study of cervical myelopathy in rheumatoid arthritis: post-mortem analysis of two cases.Two patients who developed cervical myelopathy secondary to rheumatoid arthritis were analyzed post mortem. One patient had anterior atlanto-axial subluxation (AAS) combined with subaxial subluxation (SS), and the other had vertical subluxation (VS) combined with SS. In the patient with AAS, the posterior aspect of the spinal cord demonstrated severe constriction at the C2 segment, which arose from dynamic osseous compression by the C1 posterior arch. A histological cross-section of the spinal cord at the segment was characterized by distinct necrosis in the posterior white columns and the gray matter. In the patient with VS, the upper cervical cord and medulla oblongata showed angulation over the invaginated odontoid process, whereas no significant pathological changes were observed. At the level of SS, the spinal cord was pinched and compressed between the upper corner of the vertebral body and the lower edge of the lamina. Histologically, demyelination and gliosis were observed in the posterior and lateral white columns.- - - - - - - - - - ranking = 2keywords = white (Clic here for more details about this article) |
4/43. Multiple cutaneous metaplastic synovial cysts.The cutaneous metaplastic synovial cyst is a recently described lesion that characteristically presents as a solitary, tender subcutaneous nodule. On histopathologic examination, the lesion is characterized by a cystic structure with villous-like projections and a lining resembling hyperlastic synovium. The cause of cutaneous metaplastic synovial cysts is unclear, but trauma is presumed to be a precipitating factor, as most reported cases have a history of antecedent cutaneous injury. Here we present a case of multiple, bilateral cutaneous metaplastic synovial cysts in a 72-year-old white man with rheumatoid arthritis. This is the first reported case of a patient with multiple lesions. The characteristic clinical and pathologic features of this lesion, along with proposed cause, are reviewed herein.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
5/43. Hashimoto's encephalopathy: documentation of mesial temporal seizure origin by ictal EEG.Hashimoto's encephalopathy is a chronic relapsing and remitting encephalopathy associated with antithyroid antibodies. seizures are a frequent manifestation, but are not well characterized in the literature with respect to their onset. We describe a 48-year-old patient with recurrent encephalopathy and seizures, and elevated antithyroid antibodies. One seizure was documented with video-EEG monitoring using scalp and sphenoidal electrodes. The ictal discharge originated in the left mesial-basal temporal region. MRI showed an increased T2 signal in the white matter of the centrum semiovale, but no temporal pathology. Symptoms resolved after treatment with prednisone and azathioprine. Hashimoto's encephalopathy should be considered in patients with unexplained encephalopathy and seizures, including those originating in the temporal lobe.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
6/43. Leflunomide-associated skin ulceration.OBJECTIVE: To report a case of skin ulceration as a result of treatment with leflunomide for rheumatoid arthritis. CASE SUMMARY: A 78-year-old white woman developed bilateral leg ulcers after 6 months of treatment with leflunomide for rheumatoid arthritis. A history of leg ulcers after methotrexate therapy had been documented. Serologic and diagnostic tests did not support an alternate process. Other medications prescribed were oral ethinyl estradiol 0.05 mg/d, felodipine 5 mg/d, and paroxetine 20 mg/d, for which no documented correlation with the skin breakdown could be made. DISCUSSION: This is the first published case describing a possible relationship between the use of the immunosuppressant agent leflunomide and skin ulceration. CONCLUSIONS: skin breakdown and ulceration is a recognized adverse effect of drugs with immunosuppressant activity such as methotrexate. Leflunomide, a newer agent prescribed in the treatment of rheumatoid arthritis, may now be listed among the drugs in this category associated with this adverse drug effect.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
7/43. Listeria meningitis associated with infliximab.OBJECTIVE: To report a case of listeria monocytogenes meningitis in a 73-year-old man receiving infliximab for rheumatoid arthritis. CASE SUMMARY: A 73-year-old white man taking infliximab for rheumatoid arthritis developed listeria meningitis following his second dose. He was receiving other immunosuppressants; however, these remained constant immediately prior to the infection. diagnosis was confirmed with L. monocytogenes isolated in the cerebrospinal fluid. The patient received 21 days of antibiotic therapy and recovered without any complications. DISCUSSION: L. monocytogenes is a gram-positive, non-spore-forming rod that has been associated with the ingestion of undercooked foods. This organism can cause sepsis or meningitis; however, immunocompromised patients, elderly patients, pregnant women, and neonates appear to be at greater risk for this type of infection. Tumor-necrosis factor-alpha (TNF-alpha) plays an important role in resistance to this type of infection, and listeria infections have been reported in 26 patients receiving TNF-alpha inhibitors. In our patient, the listeria infection occurred following his second course of infliximab, which provides a temporal relationship between the listeria infection and infliximab. However, his underlying rheumatoid arthritis and chronic steroid therapy would also increase his risk for a listeria infection. CONCLUSIONS: The listeria infection in our patient was a possible adverse event of infliximab according to the Naranjo probability scale. Because the majority of listeria infections occur in patients who are immunosuppressed, it would be reasonable to provide education for healthcare professionals on preventing these infections in all patients receiving immunosuppressants, including anti-TNF-alpha therapy. Those at risk due to their underlying health conditions should also be monitored closely.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
8/43. Eosinophilic cellulitislike reaction to subcutaneous etanercept injection.BACKGROUND: Injection site reactions are well recognized in patients treated with etanercept. Previous reports describe histologic findings of a cell-mediated T(H)1 reaction, with CD8 T cells composing the majority of the dermal infiltrate. OBSERVATIONS: A pruritic, erythematous, edematous patch occurred on the right thigh of a 57-year-old white woman treated for rheumatoid arthritis within 12 to 24 hours after her second dose of subcutaneous etanercept. The patient had a similar reaction to adalimumab injection 2 weeks prior to presentation. While benzyl alcohol is present in the etanercept preparation, and mannitol in both drugs, dermal injection revealed no reaction to these additives. biopsy specimens from the etanercept injection site demonstrated papillary dermal edema accompanied by a brisk polymorphous infiltrate with a predominance of eosinophils and scattered flame figures. CONCLUSIONS: Histologic features of eosinophilic cellulitis as a response to etanercept have not been reported to date. Although most injection site reactions contain T cells and represent a T(H)1 immune response, the findings we report suggest a T(H)2-mediated phenomenon.- - - - - - - - - - ranking = 1keywords = white (Clic here for more details about this article) |
9/43. New-onset rheumatoid arthritis after anthrax vaccination.BACKGROUND: anthrax vaccine was licensed in 1970 and is used to protect individuals exposed to biological warfare and those who may come in contact with bacillus anthracis in infected animals or in laboratory settings. The current adsorbed anthrax vaccine is regarded as effective and safe. Adverse effects reported include fever, chills, myalgia, arthralgia, and nausea. Four cases of rheumatoid arthritis (RA) temporally related to anthrax vaccine have been reported. As the number of administered doses increases, a better understanding of its adverse events profile will be forthcoming. OBJECTIVE: To describe another patient with RA temporally related to anthrax vaccination. methods: A 42-year-old man developed bilateral knee stiffness and pain in all the proximal interphalangeal joints 5 days after receiving the first dose of anthrax vaccine. He reported chills, fever, and joint and neck pain, with a tender nodule at the injection site after dose 2. Hours after receiving dose 3 he experienced fever, chills, nausea, vomiting, and neck, hand, and shoulder pain. The vaccination series was terminated after the third dose. RESULTS: physical examination revealed moderate swelling and tenderness of his bilateral proximal interphalangeal joints. His complete blood cell count was normal; rheumatoid factor level, 198 IU/mL; erythrocyte sedimentation rate, 53 mm/h; antinuclear antibodies, negative; c-reactive protein level, 2.7 mg/L; and anti-cyclic citrullinated peptide antibody level, 168 EU. Radiographs revealed mild degenerative changes in his hands and knees bilaterally. CONCLUSIONS: This case represents a fifth patient with RA temporally related to anthrax vaccine.- - - - - - - - - - ranking = 1.14742877417keywords = blood cell (Clic here for more details about this article) |
10/43. Infectious myositis involving the piriformis in a patient with rheumatoid arthritis.A 49-year-old Japanese woman treated with oral corticosteroids, methotrexate, and infliximab for malignant rheumatoid arthritis was admitted because of fever and low back pain. The white blood cell count and c-reactive protein concentration were elevated. Lumbar and pelvic computed tomography showed enlargement of the piriformis muscle including a hypodense area consistent with gas formation. The patient was treated successfully for infectious myositis with intravenous antibiotics.- - - - - - - - - - ranking = 2.14742877417keywords = blood cell, white (Clic here for more details about this article) |
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