[Hemophagocytic syndrome as primary manifestation in a patient with systemic lupus erythematosus after parturition].
ABSTRACT A 33-year-old woman presented with fever, malar rash, lymphadenopathy and pancytopenia 3 weeks after parturition. Serum C3 level was decreased and antinuclear antibody, anti-dsDNA antibody and anti-SS-A antibody were positive. Serum ferritin level was elevated (3454 ng/ml), and bone marrow aspirate revealed hemophagocytosis. She was diagnosed having systemic lupus erythematosus (SLE) associated with hemophagocytic syndrome (HPS). Oral prednisolone (55 mg/day) was initiated. Clinical manifestations and pancytopenia were improved and serum ferritin level was decreased. However, elevated anti-dsDNA antibody titer and reduced C3 level continued for a month after steroid therapy. The additional therapy of tacrolimus (3 mg/day) improved clinical and laboratory findings. This is a rare SLE case associated with HPS as primary manifestation after parturition.
- SourceAvailable from: Hiroyuki Yamashita[Show abstract] [Hide abstract]
ABSTRACT: We experienced three patients with refractory or severe hemophagocytic syndrome associated with the activity of systemic lupus erythematosus, so-called acute lupus hemophagocytic syndrome (ALHS). All patients were successfully treated with intermittent intravenous injections of cyclophosphamide (IVCY). In each patient, hemophagocytosis was found during bone marrow examination, and infectious causes of hemophagocytic syndrome were carefully excluded. Patients 1 and 2 were refractory to combination therapy with a high-dose corticosteroid and rituximab or cyclosporine and were successfully treated with additive IVCY. Patient 3 had very severe ALHS that developed after cardiac surgery but was successfully treated with a high-dose corticosteroid and IVCY. We conducted a literature review of adult ALHS and analyzed 54 cases reported from 2006 to 2013. Although the clinical and laboratory features were diverse, fever, bicytopenia or pancytopenia, and hyperferritinemia were almost universal. Including our 3 patients, a total of 16 have reportedly been treated with IVCY-containing regimens, and ALHS was successfully controlled in most of them without switching or adding other therapies. We suggest that combination therapy with IVCY and corticosteroids may be useful to treat severe or refractory ALHS.Clinical Rheumatology 12/2013; · 1.77 Impact Factor
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ABSTRACT: BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH), is a potentially fatal hyperinflammatory syndrome characterized fever, hepatosplenomegaly, and cytopenias. HLH can be either primary, with a genetic aetiology, or secondary, associated with malignancies, autoimmune diseases, or infections. Among rheumatic disorders, HLH occurs most frequently in systemic juvenile idiopathic arthritis. AIM: To draw attention on this severe syndrome that may often go undiagnosed in patient with rheumatic diseases. MATERIALS AND METHODS: PubMed search was performed by combining the terms (haemophagocytic, haemophagocytosis, hemophagocytosis, hemophagocytic, erythrophagocytosis, macrophage activation syndrome) and (rheumatic, rheumatologic, arthritis, lupus, Sjögren's syndrome, scleroderma, polymyositis, dermatomyositis, polymyalgia rheumatic, mixed connective tissue disease, polychondritis, sarcoidosis, polyarteritis nodosa, Henoch-Schönlein, serum sickness, wegener's granulomatosis, giant cell arteritis, temporal arteritis, Takayasu's arteritis, Behçet's syndrome, Kawasaki, Buerger's). RESULTS: 117 papers describing 421 patients were considered. HLH was described in systemic lupus erythematosus in 94 patients, in Still's disease in 37 patients, in rheumatoid arthritis in 13 patients, in systemic juvenile arthritis in 219 patients, in dermatomyositis in 7 patients, in Kawasaki disease in 25 patients, in systemic sclerosis in 5 patients, in Behcet disease in one patient, in polyarteritis nodosa in 6 patients, in ankylosing spondylitis in 2 patients, in mixed connective tissue disease in one patient, in sarcoidosis in 5 patients, in Sjögren's syndrome in 3 patients, in Wegener's granulomatosis in one patient, and in unclassifiable disorders in two patients. CONCLUSIONS: HLH occurring in the course of rheumatic diseases is an important and often underdiagnosed clinical entity, which can affect prognosis.European review for medical and pharmacological sciences 10/2012; 16(10):1414-1424. · 0.99 Impact Factor
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ABSTRACT: A 34-year-old woman showed liver dysfunction for the first time at 3 months after delivery. Two years later, she was referred to our department with continued liver dysfunction. She fulfilled the criteria for primary biliary cirrhosis (PBC)-autoimmune hepatitis (AIH) overlap. Liver dysfunction improved after administration of ursodeoxycholic acid and bezafibrate. To the best of our knowledge this represents the second report of PBC-AIH overlap after delivery and we discuss immunological changes during the perinatal period.Internal Medicine 01/2011; 50(12):1299-301. · 0.97 Impact Factor