Macrophage activation syndrome in a patient with pulmonary inflammatory myofibroblastic tumour
| Autor: | Kuppe Christoph, Westphal Saskia, Bücher Eva, Moeller Marcus J, Heintz Bernhard, Schneider Marion E, Floege Jürgen |
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| Jazyk: | angličtina |
| Rok vydání: | 2012 |
| Předmět: | |
| Zdroj: | Allergy, Asthma & Clinical Immunology, Vol 8, Iss 1, p 6 (2012) |
| Druh dokumentu: | article |
| ISSN: | 1710-1492 1710-1484 |
| DOI: | 10.1186/1710-1492-8-6 |
| Popis: | Abstract We describe for the first time a case of macrophage activation syndrome (MAS) in a patient with a history of inflammatory myofibroblastic tumour (inflammatory pseudotumour, IPT) of the lung and thoracic spine. The patient was admitted to the intensive care unit with a history of prolonged remitting fever, hepatosplenomegaly, bilaterally enlarged thoracic lymph nodes and an acute severe inflammatory response syndrome (SIRS). Up-regulated cytokine production (e.g. IL-1ß and IL-6), increased levels of ferritin and circulating soluble interleukin-2 receptor (sIL-2R, sCD25) led to the differential diagnosis of MAS. Bone marrow aspiration, the main tool for a definite diagnosis, revealed macrophages phagocytosing haematopoietic cells. Immunosuppressive therapy with corticosteroids and cyclosporine was an effective treatment in this patient. |
| Databáze: | Directory of Open Access Journals |
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