2009
DOI: 10.1007/s00296-009-0932-5
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Presenting manifestations of hemophagocytic syndrome in a male patient with systemic lupus erythematosus

Abstract: Hemophagocytic syndrome (HPS) is an unusual but sometimes fatal disorder. We reported a case of 21-year-old man who developed HPS and SLE simultaneously. Febrile pancytopenia, hyperferritinemia, and abnormal liver function tests were observed. Hemophagocytic cells were observed by means of bone marrow biopsy and diagnosed as HPS. The patient was treated with high-dose prednisolone, resulting in an excellent outcome. Early diagnosis of HPS by bone marrow biopsy is important for the successful treatment.

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Cited by 12 publications
(10 citation statements)
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“…749,750 There are a few reports of simultaneous development of HLH and systemic lupus erythematous. 751 Patients with rheumatic disorders, especially juvenile chronic arthritis, appear particularly at risk, the condition being often referred to as macrophage activation syndrome in this setting. 752,753 Haemophagocytic features are often seen in livers of patients with AIDS ( Fig.…”
Section: Down Syndromementioning
confidence: 99%
“…749,750 There are a few reports of simultaneous development of HLH and systemic lupus erythematous. 751 Patients with rheumatic disorders, especially juvenile chronic arthritis, appear particularly at risk, the condition being often referred to as macrophage activation syndrome in this setting. 752,753 Haemophagocytic features are often seen in livers of patients with AIDS ( Fig.…”
Section: Down Syndromementioning
confidence: 99%
“…On follow up child did not have fever, bleeding manifestations, anemia or serositis. His hemoglobin was 11g/dL, TLC-7800/mm 3 , and platelets-1.2 lakh/mm 3 .…”
Section: Lymphohistiocytosis As Presenting Feature Of Lupusmentioning
confidence: 97%
“…Rheumatic diseases rarely cause secondary HLH. Systemic lupus erythromatosus (SLE) as a cause of HLH is rare [2][3][4].…”
Section: Lymphohistiocytosis As Presenting Feature Of Lupusmentioning
confidence: 99%
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“…2076,2077 There are a few reports of simultaneous development of HLH and SLE. 2078 Patients with rheumatic disorders, especially juvenile chronic arthritis, appear particularly at risk; the condition is often referred to as macrophage activation syndrome in this setting. 2079,2080 Haemophagocytic features are often seen in livers of patients with acquired immunodeficiency syndrome (AIDS) ( Fig.…”
Section: Haemophagocytic Lymphohistiocytosismentioning
confidence: 99%