A RARE CASE OF SLE PRESENTING AS HLH

Abstract

Hemophagocytic Lymphohistiocytosis (HLH) presentation in systemic lupus erythematosus (SLE) is a very rare entity. The current incidence is 1 person per million population per annum. A 24-year-old male presented with fever for one month along with loss of appetite and severe weight loss. Vital signs were within normal limits except for body temperature of 102 °F. The patient is also presented with pancytopenia, hepatosplenomegaly, liver function abnormalities, coagulopathy, and hyperferritinemia. All other tests for infectious diseases were negative. So, rheumatological examination was done and DCT was found be strongly positive, ICT- negative , c3- <40 ( 88-165), c4- 28.2 mg/dl( 14-44 mg/dl), ANA immunoblot- smith antibody- +++, SSA- +, dsDNA- negative. So SLE was confirmed and he is treated with inj methylpred for 3 days f/b tab wysolone 1 mg/ kg po.patient improved dramatically.