Association of Macrophage Activating Syndrome with Castleman’s Syndrome in Systemic Lupus Erythematosus
Publish place: IRANIAN JOURNAL of PATHOLOGY، Vol: 11، Issue: 3
Publish Year: 1395
Type: Journal paper
Language: English
View: 375
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Document National Code:
JR_IJP-11-3_011
Index date: 23 September 2019
Association of Macrophage Activating Syndrome with Castleman’s Syndrome in Systemic Lupus Erythematosus abstract
Macrophage Activating Syndrome (MAS) is a life-threatening disease seen in autoimmune diseases including lupus erythematosus, rheumatoid arthritis, Still s disease, polyarteritis nodosa. It is characterized by fever, pancytopenia, liver failure, coagulopathy, and neurologic symptoms and high serum ferritin. A 27 yr. old female patient was admitted in shahid Mostafa Khomeini Hospital (Tehran-Iran) in May 2011 because of lower extremities edema and ascites and fever from 1.5 month ago. In physical examinations she had generalized lymphadenopathy, splenomegaly and pleural effusion. In laboratory tests she had pancytopenia, positive ANA and Anti DNA (ds), hypocomplementemia, hypertriglyceridemia and high ferritin level. Gradually she had signs of RPGN and ARDS. The patient had no skin and musculoskeletal signs of SLE and no liver failure nor coagulopathy of MAS. Her lymph node biopsy was reported as Castleman syndrome. Unlike other studies, the patient showed MAS before treatment with cytotoxic for lupus nephritis.
Association of Macrophage Activating Syndrome with Castleman’s Syndrome in Systemic Lupus Erythematosus Keywords:
Systemic Lupus Erythematosus , Macrophage activating syndrome hemophagocytic lymphohistiosytosis , Castleman syndrome
Association of Macrophage Activating Syndrome with Castleman’s Syndrome in Systemic Lupus Erythematosus authors
Shamsa Shariatpanahi
Dept. of Internal Medicine, Shahed University, Tehran, Iran
Shahryar Pourfarzam
Dept. of Pathology, Shahed University, Tehran, Iran
Mohammad hosein Gheini
Dept. of Pathology, Shahed University, Tehran, Iran