A Rare Case of Haemophagocytic Syndrome in a HIV Seropositive Patient with Cytomegalovirus Induced Paraparesis

Hemophagocytic syndrome or hemophagocytic lymphohistiocytosis (HLH ) is a rare but potentially life threatening condition characterized by uncontrolled activation of CD8+ T lymphocytes and macrophages that leads to organ damage. HLH can be primary (familial or genetic) and secondary. Secondary HLH occurs due to infections caused mostly by viruses like Ebstein Barr virus, Cytomegalovirus (CMV), Parvo B19 etc. Early diagnosis and treatment is the key to management of HLH. We present here a case of a 30 year old female who presented with fever, jaundice, cough and progressive weakness of both lower limbs. There was no girdle like sensation, no bladder/bowel involvement. She was recently diagnosed to be seropositive for Human Immuno-deficiency Virus (HIV)-1 and had a CD4 count of 27/μL. She had hepatomegaly, pancytopenia, hyperbilirubinemia, hypoalbuminemia, raised liver enzymes and hypertriglyceridemia. CSF had raised protein, reduced sugar and neutrophils. DNA PCR for CMV was positive and there was evidence of CMV disease on fundoscopy. Her bone marrow revealed presence of haemophagocytes. She was treated successfully with Ganciclovir, Dexamethasone and antiretroviral drugs and was discharged. Though often fatal and difficult to diagnose, HLH is potentially treatable disorder.