1/22. Severe transient pancytopenia associated with procainamide ingestion.A 73-year-old woman was found to have clinically significant pancytopenia in association with procainamide hydrochloride ingestion. The syndrome, resembling systemic lupus erythematosus, which has been reported to develop in patients treated with this agent, is characterized by mild to moderate anemia and mild to moderate granulocytopenia. Severe granulocytopenia in patients taking procainamide and unrelated to a lupus syndrome has not previously been reported in association with significant thrombocytopenia. The clinical severity of this patient's presentation, suggesting an aleukemic leukemia, and its complete remission after cessation of procainamide administration occasional this report.- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
2/22. Myelofibrosis in systemic lupus erythematosus.In this study we present a case of coexisting systemic lupus erythematosus (SLE) and myelofibrosis. literature review supports the fact that the two diseases rarely occur together in the same patient. The young female patient studied was admitted with pancytopenia and a clinical picture which met the criteria of SLE. Histological examination of the bone marrow biopsy revealed severe myelofibrosis with hypocellularity of the myeloid cell lines. Treatment with immunosuppressive and colony stimulating factor led to slow but complete regeneration of the bone marrow and subsequently to an improved haematological status, and the patient was spared bone marrow transplantation.- - - - - - - - - - ranking = 4.7896794180818keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
3/22. Aplastic anemia complicating systemic lupus erythematosus--report of a case and review of the literature.Aplastic anemia is a very unusual feature of systemic lupus erythematosus (SLE). A 32-year-old lady presented with generalized purpuric lesions and was diagnosed as having immune thrombocytopenic purpura. Fourteen months later, she developed progressive pancytopenia, arthritis of small joints, and oral ulcers. Investigations confirmed SLE with aplastic anemia. High-dose methylprednisolone therapy had been unsuccessful in controlling the pancytopenia. She had a progressive course and died due to septicemia. Even though pancytopenia is common in SLE, a bone marrow examination should be done in all cases of persistent pancytopenia to exclude bone marrow aplasia. This will help in tailoring the treatment with more aggressive immunosuppressants.- - - - - - - - - - ranking = 4.7896794180818keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
4/22. Neonatal lupus erythematosus with recurrent pancytopenia: a case report.Neonatal lupus erythematosus is an immune-mediated disease associated with the transplacental passage of maternal autoantibodies, primarily anti-Ro (SS-A) and anti-La (SS-B). The major clinical manifestations are isolated congenital heart block and cutaneous lupus lesions. Other symptoms include hematologic and hepatic abnormalities. We report a male neonate, born to a mother with sjogren's syndrome, who experienced cutaneous lupus lesions and 2 episodes of pancytopenia. Both anti-Ro and anti-La antibodies were positive in infant and mother.- - - - - - - - - - ranking = 4.0839060042336keywords = lupus erythematosus, erythematosus, lupus (Clic here for more details about this article) |
5/22. Aplastic anemia complicating systemic lupus erythematosus: successful management with cyclosporine.anemia is common with connective tissue disorders, but pancytopenia is rare. We report a 22-year-old female who presented with menorrhagia, seizures, anemia, leukocytosis, thrombocytopenia, pericardial effusion, positive ANA, and evidence of vasculitis on CT head scan and was diagnosed with systemic lupus erythematosus (SLE). After 7 months of remission, she was readmitted with menorrhagia and pancytopenia. Investigations revealed aplastic anemia. She survived on transfusion support for 6 weeks, during which period she received methylprednisolone and cyclophosphamide pulses, and phenytoin was omitted but to no avail. cyclosporine (300 mg/day) was started and the aplastic anemia responded. After 4 months of therapy, the cyclosporine was gradually tapered over the next 2 months. The patient has been on 10 mg/day of prednisolone for the last 6 months. Aplastic anemia is rare in SLE and the response to immunosuppressants is variable, but here is a success story.- - - - - - - - - - ranking = 4.7896794180818keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
6/22. Acute lupus hemophagocytic syndrome: report of a case and review of the literature.The authors reported a case of systemic lupus erythematosus (SLE) with an unusual presentation. The patient presented with acute febrile illness along with progressive pancytopenia related to increasing hemophagocytic activity of histiocytes in the bone marrow. Concomitant polyarthritis, myositis, nephritis, high titer of antinuclear factor (1:2,560) and positive test for anti-dna antibody made him fit the diagnostic criteria of SLE. No definite evidence of associated infections was confirmed by bacteriologic, serologic and viral studies. He did not respond to empiric antibiotic therapy but dramatically responded to corticosteroid treatment. Therefore, diagnosis of acute lupus hemophagocytic syndrome was made. The clinical presentation, laboratory diagnosis, and management of the patient are discussed and the literature was reviewed and presented.- - - - - - - - - - ranking = 1.1682564655345keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
7/22. Leflunomide-associated pancytopenia with or without methotrexate.OBJECTIVE: To report 18 cases of pancytopenia associated with leflunomide use in australia, 5 of which were treated at Princess Alexandra Hospital, Brisbane. case summaries: Leflunomide was used in the treatment of rheumatoid arthritis in 17 of 18 patients; the other patient was diagnosed with systemic lupus erythematosus. Median age was 65.5 years (range 18-79), and 15 of the patients were female. Fourteen patients were on combined treatment with methotrexate. pancytopenia was typically severe, requiring hospital admission, withdrawal of the immunosuppressant(s), intensive supportive therapy, and treatment of neutropenic sepsis. Five patients died, 4 of whom were receiving concomitant methotrexate. time to onset of pancytopenia ranged from 11 days to 4 years (median 4 mo). In one case in which the patient had been stable while receiving leflunomide, methotrexate, and hydroxychloroquine for 4 years, fatal pancytopenia was triggered by acute renal failure secondary to dehydration and use of nonsteroidal antiinflammatory drugs. The Naranjo probability scale suggested a probable causal association in 5 cases and possible association in the remainder. DISCUSSION: Leflunomide, indicated for treatment of active rheumatoid arthritis, inhibits pyrimidine synthesis in lymphocytes and other rapidly dividing cells and may rarely be associated with life-threatening pancytopenia. Combination therapy with methotrexate may increase the risk. time to onset is variable, and clinicians should remain vigilant, particularly when there is a change in the patient's baseline health status. CONCLUSIONS: The risk of pancytopenia during leflunomide therapy appears to be increased when the drug is combined with methotrexate and in older patients. Onset may be delayed, and ongoing monitoring of blood counts is essential.- - - - - - - - - - ranking = 0.95793588361637keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
8/22. histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto's disease) mimicking systemic lupus erythematosus: a review of two cases.Kikuchi-Fujimoto's disease (KFD), or histiocytic necrotizing lymphadenitis, is a benign and self-limited lymphadenitis commonly found in young women. It often shares clinical features with systemic lupus erythematosus (SLE), such as arthralgias, fever and leukopenia. The etiology of KFD remains unknown and controversial. Clinical course is favorable, with spontaneous remission in less than four months in almost all cases. Herein, we present two cases. The former is a 53-year old woman presenting with cervical lymphadenopathy, arthralgia, pancytopenia and positive antinuclear antibody (ANA). Lymph node biopsy revealed histopathological features compatible with Kikuchi-Fujimoto histiocytic necrotizing lymphadenitis. The latter patient was a 20-year old woman presenting with left cervical lympadenopathy, a butterfly rash that was reminiscent of SLE, and a positive antinuclear antibody. Based upon clinical, histological and laboratory findings, the diagnosis of SLE was excluded. Careful attention should be paid to differentiating between KFD and SLE, because of their similar presentations, yet different clinical courses and therapeutic requirements.- - - - - - - - - - ranking = 4.7896794180818keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
9/22. leukemoid reaction after methotrexate-induced pancytopenia in a patient undergoing continuous ambulatory peritoneal dialysis.methotrexate has been used as an important alternative therapy in the treatment of various rheumatic diseases. life threatening marrow suppression in end-stage renal disease patients undergoing hemodialysis or continuous ambulatory peritoneal dialysis has been reported. A 33-year-old woman with systemic lupus erythematosus undergoing chronic peritoneal dialysis developed severe mucositis and pancytopenia after low-dose methotrexate treatment for arthritis. The leukocyte count recovered after methotrexate was withdrawn but a leukemoid blood picture developed during her recovery. No evidence of leukemia was found on bone marrow biopsy. The leukocyte count gradually returned to normal with conservative therapy.- - - - - - - - - - ranking = 0.95793588361637keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
10/22. Malignant histiocytosis as a fatal complication of systemic lupus erythematosus.We present the case of a 26-year-old Caucasian woman with systemic lupus erythematosus (SLE) of 9 years duration, mainly involving the kidney. Her clinical course was complicated in the last year by several infectious and noninfectious events, and after pancytopenia, fever, and extremely high levels of lactate dehydrogenase developed, the woman died. light and electron microscopy of bone marrow, together with immunophenotyping, disclosed malignant histiocytosis, a complication of SLE that has not been reported.- - - - - - - - - - ranking = 4.7896794180818keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
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