FRI0594 COMPARISON OF CLINICAL FEATURES BETWEEN HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS COMPLICATED WITH RHEUMATIC DISEASES OR TUMOR. (June 2019)

• Record Type: Journal Article
• Authors: Liao, Zetao; Jiang, Yutong; Qing, LV; Yang, Mingcan; Zhang, Yanli; Guo, Xinghua; Gu, Jieruo
• Is Part Of: Annals of the rheumatic diseases. Volume 78(2019)Supplement 2
• Page Start: 992
• Page End: 993
• Publication Date: 2019-06
• Subjects: Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/annrheumdis-2019-eular.2772
• Languages: English
• ISSNs: 0003-4967

Abstract

Abstract : Background: Hemophagocytic lymphohistiocytosis (HLH) covers a wide spectrum of related life-threatening conditions featuring ineffective immunity characterized by an uncontrolled hyperinflammatory response, which including autosomal recessive familial HLH (FHL), familial erythrophagocytic lymphohistiocytosis, viral-associated hemophagocytic syndrome, and autoimmune-associated macrophage activation syndrome (MAS). Objectives: Our study was to compare the difference between HLH related with rheumatic diseases or tumor. Methods: We retrospectively reviewed the patient data with definite bone marrow hemophagocytosis in the inpatient department of the Third Affiliated Hospital of Sun Yat-sen University between 01/11/2011 to 31/05/2018. The clinical manifestations, laboratory exam and prognosis were compared between patients related with tumor and rheumatic diseases. Results: 7 patients with rheumatic diseases and 5 patients with tumor were enrolled. In patients with rheumatic diseases, 6 with adult-onset Still's disease and 1 with SLE. In patients with tumor, 4 with lymphoma and 1 with undefined tumor. The clinical features were compared between the two types of patients as below. Conclusion: Clinical features of HLH patients complicated with tumor or rheumatic diseases were comparable in many aspects. Yet patients with tumor have more hypofibrinogenemia and lung involvement, and tend to be treated with VP-16 and Chemical therapy, and have much worse prognosis. References: Disclosure of Interests: None declared