A CASE REPORT OF PEDIATRIC HLH MIMICKING CHRONIC LIVER DISEASE

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a clinical syndrome characterized by reactive hyperactivity of cytotoxic T cells and histiocytes, leading to hypercytokinemic injury to cells and organ system, which leads to multiorgan dysfunction and ultimate failure. It is characterized by fever, pancytopenia, splenomegaly and hemophagocytosis in bone marrow, liver or lymph nodes. It has been associated with viral, bacterial, fungal and parasitic infection. Although an early diagnosis and treatment is the key to decrease the mortality but it is difficult to diagnose this case as it involves numerous laboratory tests which are non specific.

            We report a case of  hemophagocytic lymphohistiocytosis in a 4 year old male child presenting with prolonged fever, pancytopenia, hepatosplenomegaly, progressive swelling of the abdomen, decreased urination and pedal edema. Based on our patient’s presentation and initial laboratory findings,which revealed cytopenias,hypertriglyceridemia,hypofibrinogenemia,absent natural killer (NK) cell activity, high serum ferritin level; a diagnosis of HLH was made. He was started on broad-spectrum antibiotics,antifungals,and dexamethasone.In spite of all efforts,within short span the child developed MODS and succumbed.Thus early diagnosis and prompt treatment can bring hope in this type of life threatening condition.

Keywords: pancytopenia,hepatosplenomegaly,hemophagocytosis,hypofibrinogenemia