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Volume 9, Issue 3 ( September 2017 2017)
Iranian Journal of Blood and Cancer 2017, 9(3): 93-96 |
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Abolghasemi H, Shahverdi E, Niknam R, Beiraghdar F, Afkhami Fard S. Macrophage Activation Syndrome as the First Presentation of Juvenile Idiopathic Arthritis. Iranian Journal of Blood and Cancer 2017; 9 (3) :93-96
URL: http://ijbc.ir/article-1-720-en.html
URL: http://ijbc.ir/article-1-720-en.html
Hassan Abolghasemi1 
, Ehsan Shahverdi *2 , Reyhaneh Niknam , Fatemeh Beiraghdar3 , Shirin Afkhami Fard4
, Ehsan Shahverdi *2 , Reyhaneh Niknam , Fatemeh Beiraghdar3 , Shirin Afkhami Fard4
1- Pediatric Congenital Hematologic Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran
2- Blood Transfusion Research Center, High Institute for Research and Education in Transfusion Medicine, Tehran, Iran ,shahverdi_ehsan@yahoo.com
3- Department of Pediatrics, Baqiyatallah University of Medical Sciences, Tehran, Iran
4- Mashhad Azad University of Medical Sciences, Mashhad, Iran
2- Blood Transfusion Research Center, High Institute for Research and Education in Transfusion Medicine, Tehran, Iran ,
3- Department of Pediatrics, Baqiyatallah University of Medical Sciences, Tehran, Iran
4- Mashhad Azad University of Medical Sciences, Mashhad, Iran
Abstract: (5290 Views)
Macrophage activation syndrome (MAS) is a rare feature of rheumatic disorders in children and adolescence and its presentation as the first symptom of rheumatic disorders is very infrequent.
A 9-year-old girl, in whom MAS developed, was admitted to our Hospital in Tehran, Iran. She suffered from high grade fever and rash followed by multiple joint swelling months afterwards. Bone marrow aspiration and biopsy showed normocellular marrow with a cellularity of 90%. Benign-looking macrophages were remarkably increased; many of them showed hemophagocytic features. According to the presentation of long-standing fever and observation of “hemophagocytic macrophage” in bone marrow, MAS was diagnosed for the patient. Additionally, due to recurrent joint swelling in following months, she was diagnosed to be affected by “Juvenile Idiopathic Arhtritis” complicated by MAS.
MAS is a rare complication of rheumatic disorders which should be considered as the first presentation of rheumatic disorders in children specifically in those presenting with high fever, hepatosplenomegaly, lymphadenopathy and severe cytopenia.
A 9-year-old girl, in whom MAS developed, was admitted to our Hospital in Tehran, Iran. She suffered from high grade fever and rash followed by multiple joint swelling months afterwards. Bone marrow aspiration and biopsy showed normocellular marrow with a cellularity of 90%. Benign-looking macrophages were remarkably increased; many of them showed hemophagocytic features. According to the presentation of long-standing fever and observation of “hemophagocytic macrophage” in bone marrow, MAS was diagnosed for the patient. Additionally, due to recurrent joint swelling in following months, she was diagnosed to be affected by “Juvenile Idiopathic Arhtritis” complicated by MAS.
MAS is a rare complication of rheumatic disorders which should be considered as the first presentation of rheumatic disorders in children specifically in those presenting with high fever, hepatosplenomegaly, lymphadenopathy and severe cytopenia.
Keywords: Macrophage activation syndrome, Juvenile idiopathic arthritis, Hemophagocytic macrophage, First presentation
: Case report |
Subject:
Pediatric Hematology & Oncology
Received: 2017/04/24 | Accepted: 2017/10/8 | Published: 2017/11/25
Received: 2017/04/24 | Accepted: 2017/10/8 | Published: 2017/11/25
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