A Fatal Case of Hemophagocytic Lymphohistiocytosis Due to Extra-Pulmonary Sarcoidosis

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SESSION TITLE: Wednesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/23/2019 09:45 AM - 10:45 AM INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening condition of overwhelming systemic inflammation. It can lead to multi-organ failure and circulatory shock with high reported intensive care unit mortality rate of 50-80%. Sarcoidosis association with HLH is very rare, as only eight previous cases have been reported in literature so far. In our case report, we present a fatal case of HLH associated with non-pulmonary sarcoidosis to raise awareness of the diagnosis and facilitate earlier treatment. CASE PRESENTATION: The patient was a 48-year-old male newly diagnosed with sarcoidosis who was transferred to our intensive care unit with persistent fever and chest pain. Two months prior to his presentation, imaging of his abdomen showed a large mesenteric mass, large splenic masses, and heterogeneous liver parenchyma. Biopsies showed non- necrotizing granulomatous inflammation. The patient was diagnosed with sarcoidosis and started on oral steroids. On presentation, he was febrile, tachycardic and hypotensive. His blood work was significant for pancytopenia, elevated liver function, low fibrinogen and high serum ferritin. He was fluid resuscitated and empirically treated with broad spectrum antibiotics for presumed severe sepsis. An extensive infectious work up was negative. A bone marrow biopsy exhibited a hypo-cellular bone marrow with histiocytic hemophagocytosis. Patient was started on dexamethasone, intravenous immunoglobulin and etoposide as part of the HLH treatment protocol. His medical condition deteriorated, as he developed a shock state unresponsive to maximum vasopressors. Patient expired soon after. DISCUSSION: HLH results from uncontrolled activation of histiocytes, natural killer cells and cytotoxic T-cells which lead to persistent systemic inflammatory response syndrome. The HLH -2004 protocol defines the diagnostic criteria as fever, splenomegaly, cytopenia, hypertriglyceridemia and/or hypofibrinogenemia, low NK -cell activity, hyperferritinemia, elevated serum soluble interleukin 2 receptor and hemophagocytosis in tissue biopsy. The presentation of HLH in the intensive care unit is very similar to sepsis, however the development of cytopenia in the setting of severe sepsis and multi-organ failure should raise the suspicion of HLH. Infections and malignancies are the most common triggers of HLH. Some autoimmune diseases such as systemic lupus erythematous are also complicated by HLH. Association with sarcoidosis, however, is extremely rare. Out of the eight cases of HLH associated with sarcoidosis, four were triggered by an infectious cause. In our patient no infectious trigger was identified CONCLUSIONS: It is imperative to diagnose and differentiate HLH from sepsis in the ICU in a timely manner. Early recognition and prompt treatment of this disorder is needed to prevent fatal outcomes. Reference #1: Henter JI, Horne A, Aricó M, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007. Reference #2: Kapoor S, Morgan CK, Siddique MA, Kalpalatha K Guntupalli. Intensive care unit complications and outcomes of adult patients with hemophagocytic lymphohistiocytosis: A retrospective study of 16 cases. World J Crit Care Med. 2018;7(6):73-83. Reference #3: Filipovich AH. Hemophagocytic Lymphohistiocytosis and Other Hemophagocytic Disorders. Immunol Allergy Clin North Am. 2008.





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