Hemophagocytic Lymphohistiocytosis in a 9-years-old girl with Common Variable Immunodeficiency – A fatal complication

Abstract

A 9-years-old girl presented with complaints of fever, abdominal pain and weight loss for 1 month preceded by chronic bilateral ear discharge for 3 years. She was treated for a culture positive urinary tract infection initially and then for suspected abdominal tuberculosis due to chronic non-resolving symptoms and suggestive radiological findings. Workup suggested an underlying common variable immunodeficiency. During admission, she developed a generalized rash followed by blood and urine cultures showing growth of Klebsiella pneumoniae. She later had progressive liver dysfunction and pancytopenia, hyper-triglyceridemia, hyper-ferritinemia, hypo-fibrinogenemia, raised d-Dimers, hypo-albuminemia and deranged coagulation profile. Bone marrow biopsy confirmed a diagnosis of Hemophagocytic Lymphohistiocytosis. She was managed with intravenous antibiotics, immunoglobulins, steroids, cyclophosphamide and supportive management. However, she developed fulminant sepsis and Multi-Organ Dysfunction Syndrome, continued to deteriorate and ultimately expired.