Cases reported "Arthritis, Rheumatoid"

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21/26. Hemophagocytic syndrome in a patient with rheumatoid arthritis.

    A 63-year-old female, who had been diagnosed with rheumatoid arthritis (RA) 3 years previously, was admitted due to progressive pancytopenia, lymphadenopathy, fever, and weight loss. The physical and laboratory findings fulfilled all of the American Rheumatism association (ARA) revised criteria for RA. Her bone marrow aspirate revealed a decreased nuclear cell count (1.8 x 10(4) microliters) and megakarocyte count (0/microliter), and macrophages phagocytizing blood cells (4%), indicating the presence of hemophagocytic syndrome. The serological tests for several viruses revealed no obvious viral etiology. However, a slight Epstein-Barr virus (EBV) reactivation could not be excluded. Administration of 40 mg prednisolone daily improved her abnormal hematological findings and immunological laboratory parameters. This is a case of RA accompanied by hemophagocytic syndrome, which has not been reported previously as a complication of RA.
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22/26. Fatal cholestatic hepatitis caused by D-penicillamine.

    A 37-yr-old woman with RF-positive RA developed cholestatic hepatitis after 10 days of D-penicillamine therapy. This was discontinued immediately. The cholestasis persisted for the remaining 14 months of her life. Severe hypercholesterolemia developed with xanthelasmata and eventually pancytopenia, which was caused by a massive infiltration of the bone marrow by lipid-containing foam cells. The patient died of sepsis. review of the literature shows intrahepatic cholestasis to be a rare and idiosyncratic complication of D-penicillamine therapy. To our knowledge, ours is the first documented case of persistent cholestatic icterus.
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23/26. pancytopenia and severe cytopenia induced by low-dose methotrexate. Eight case-reports and a review of one hundred cases from the literature (with twenty-four deaths)

    Severe adverse effects of low-dose methotrexate (less than 20 mg per week) are believed to be rare. We report eight cases of severe tricytopenia or pancytopenia seen in two medical departments of the same hospital in patients receiving low-dose methotrexate. Three patients had been under methotrexate for less than one month. Of the six patients with joint disease, five had rheumatoid arthritis and one psoriatic arthritis. A review of the literature found 92 previously reported cases of severe tricytopenia or pancytopenia induced by low-dose methotrexate. Of the total of 100 cases, 24 were fatal and 25 occurred within one month of treatment initiation. Potential risk factors were identifiable retrospectively in at least 50% of cases but were not all predictable or present at treatment initiation. In 30% of cases, no explanation for the hematologic complication was found, and in an additional 20% missing data precluded definite conclusions. The role of the risk factors incriminated in the literature is discussed. Although infrequent, cytopenia is a severe complication of methotrexate therapy that warrants a number of precautions, including periodic creatinine clearance and serum albumin determinations. Furthermore, the weekly dosing schedule should be printed on methotrexate boxes.
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keywords = pancytopenia
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24/26. pancytopenia secondary to methotrexate therapy in rheumatoid arthritis.

    OBJECTIVE: To assess the frequency of methotrexate (MTX)-induced pancytopenia in rheumatoid arthritis (RA). methods: A medline literature search was conducted to identify articles published during the last 15 years (1980-1995) that presented data on MTX-associated pancytopenia. Two case reports of our own experience are also presented. In addition, articles that examined risk factors associated with MTX-related pancytopenia were identified. RESULTS: A total of 70 patients with pancytopenia related to MTX therapy were identified (68 reported in the literature, 2 from our own experience). Sixty-one of the patients were described in published case reports, 7 patients were from 5 long-term prospective studies. In many of these cases, predisposing factors for the development of pancytopenia were described. The 5 long-term prospective studies reported toxicity data on patients who had been treated with MTX for at least 13 weeks. A total of 511 patients were included in the prospective trials, yielding an overall incidence of pancytopenia of 1.4% (7 of 511). Of the 70 cases reported, 12 patients died (17%). Most of them had impaired renal function, hypoalbuminemia, concurrent infection, and/or concomitant medication with more than 5 drugs. The minimal cumulative MTX dose leading to fatal pancytopenia was 10 mg, observed in one of our patients. CONCLUSION: pancytopenia is not an uncommon side effect of low-dose pulse MTX therapy in RA. It can lead to serious complications, including death.
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keywords = pancytopenia
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25/26. Benefit of simultaneous rhG-CSF and methylprednisolone 'pulse' therapy for methotrexate-induced bone marrow failure in rheumatoid arthritis.

    A 74-year-old female with seropositive rheumatoid arthritis developed severe bone marrow failure after the treatment with very low-dose methotrexate (5 mg/week for 3 weeks). Hematological data showed severe pancytopenia with 0% neutrophils and bone marrow disclosed thoroughly hypocellular marrow. Shortly after the treatment with simultaneous rhG-CSF and methylprednisolone 'pulse' therapy, pancytopenia was ameliorated and bone marrow examination revealed hypercellular marrow with increasing maturation of hematopoietic components. Hematological parameters were maintained within the normal range with a low dose of prednisolone.
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26/26. pancytopenia secondary to hemophagocytic syndrome in rheumatoid arthritis treated with methotrexate and sulfasalazine.

    Hemophagocytic syndrome is an exceptional cause of pancytopenia. Its etiologies are most commonly viral or bacterial infections, lymphoproliferative syndromes, acquired or congenital immunodeficiencies, systemic diseases, or immunomodulatory treatment. We describe a patient with rheumatoid arthritis (RA) treated with methotrexate (MTX), sulfasalazine, and low dose corticosteroids, whose case was seriously complicated by the occurrence of acute febrile pancytopenia. The pancytopenia appeared secondary to hemophagocytic syndrome triggered by escherichia coli septicemia. The evolution was marked by severe aggravation of RA, probably due to release of cytokines from macrophages (tumor necrosis factor-alpha, interleukin 6). Reintroduction of MTX (without sulfasalazine) resulted in partial remission and there was no reappearance of new hematological anomalies after 16 month followup. A knowledge of this syndrome is particularly important, since it mimics drug toxicity and other complications such as lymphoproliferative diseases.
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