Petros Ioannou1, Evangelia Akoumianaki2, Konstantinos Alexakis3, Athanasia Proklou2, Maria Psyllaki4, Efthimis Stamatopoulos5, Mairi Koulentaki6, Eumorfia Kondili2, Diamantis P Kofteridis7. 1. MD, MSc, PhD, Department of Internal Medicine, University Hospital of Heraklion, Stavrakia and Voutes crossroad, Heraklion, PC 71110, Crete, Greece. 2. MD, PhD, Intensive Care Unit, University Hospital of Heraklion, Stavrakia and Voutes crossroad, Heraklion, PC 71110, Crete, Greece. 3. MD, Department of Internal Medicine, University Hospital of Heraklion, Stavrakia and Voutes crossroad, Heraklion, PC 71110, Crete, Greece. 4. MD, PhD, Department of Hematology, University Hospital of Heraklion, Stavrakia and Voutes crossroad, Heraklion, PC 71110, Crete, Greece. 5. MD, Department of Gastroenterology, University Hospital of Heraklion, Stavrakia and Voutes crossroad, Heraklion, PC 71110, Crete, Greece. 6. MD, PhD, Department of Gastroenterology, University Hospital of Heraklion, Stavrakia and Voutes crossroad, Heraklion, PC 71110, Crete, Greece. 7. MD, PhD, Department of Internal Medicine, University Hospital of Heraklion, Stavrakia and Voutes crossroad, Heraklion, PC 71110, Crete, Greece.
Abstract
INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening rare disease resulting from the uncontrolled activation of the immune system, leading to unrestrained cytokine release and macrophage activation. It can be either hereditary or acquired due to infections, hematological disease or malignancy. CASE REPORT: We present the case of a 19-year old woman that presented with high fever and acute cholestatic hepatitis. She was initially admitted to the Gastroenterology department and the following days she developed respiratory distress and multiorgan insufficiency that necessitated intubation and support in the Intensive Care Unit. Fever, splenomegaly, hypertriglyceridemia, increased ferritin levels and hemophagocytosis in the bone marrow were found, thus, fulfilling the criteria of hemophagocytic lymphohistiocytosis. Laboratory examination was notable for positive serology (IgM and IgG) and PCR for EBV in the serum. An extensive workup including virology and immunologic workup, blood cultures, a CT of the thorax and the abdomen and a bone marrow biopsy did not reveal any cause of secondary HLH other than the EBV infection. The patient was treated with high dose corticosteroids and intravenous immunoglobulins with slow resolution of her symptoms. CONCLUSIONS: In patients with EBV infection who exhibit persistent high fever and unresponsiveness to antibiotics, the possibility of HLH should be considered. Early diagnosis and rapid initiation of appropriate treatment may avert an unfavorable outcome. GERMS.
INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening rare disease resulting from the uncontrolled activation of the immune system, leading to unrestrained cytokine release and macrophage activation. It can be either hereditary or acquired due to infections, hematological disease or malignancy. CASE REPORT: We present the case of a 19-year old woman that presented with high fever and acute cholestatic hepatitis. She was initially admitted to the Gastroenterology department and the following days she developed respiratory distress and multiorgan insufficiency that necessitated intubation and support in the Intensive Care Unit. Fever, splenomegaly, hypertriglyceridemia, increased ferritin levels and hemophagocytosis in the bone marrow were found, thus, fulfilling the criteria of hemophagocytic lymphohistiocytosis. Laboratory examination was notable for positive serology (IgM and IgG) and PCR for EBV in the serum. An extensive workup including virology and immunologic workup, blood cultures, a CT of the thorax and the abdomen and a bone marrow biopsy did not reveal any cause of secondary HLH other than the EBV infection. The patient was treated with high dose corticosteroids and intravenous immunoglobulins with slow resolution of her symptoms. CONCLUSIONS: In patients with EBV infection who exhibit persistent high fever and unresponsiveness to antibiotics, the possibility of HLH should be considered. Early diagnosis and rapid initiation of appropriate treatment may avert an unfavorable outcome. GERMS.
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