A Case Report of Hemophagocytic Lymphohistiocytosis Masquerading as Sepsis

Abstract

Profound inflammation due to cytokine storm is often the underlying cause of death in patients with hemophagocytic lymphohistiocytosis (HLH). Sepsis, while a precipitant, is also the great masquerader that may hide early signs of HLH. Prompt recognition is important to prevent rapid clinical decline and death. A patient presented with two weeks of unremitting fever of 103°F, dysuria, bilateral flank pain, and confusion. Obstructive uropathy and pyelonephritis were treated with a Foley catheter and antibiotics. There were abnormal developments during his hospitalization including a deep vein thrombus despite prophylactic anticoagulation. Antibiotics and Foley management did not improve fevers or renal injury so he eventually required continuous renal replacement therapy and blood product transfusions. In rapid progression, the patient developed pancytopenia, neutropenia, hyperferritinemia, hypertriglyceridemia, and hypofibrinogenemia suspicious for HLH. A bone marrow biopsy was consistent with progressive T-cell lymphoma, the likely cause of secondary HLH. Antineoplastics, corticosteroids, and opportunistic prophylaxis were pursued. Unfortunately, the cytopenias worsened, and the patient developed shock with hypoxemia and hypotension, followed by cardiac arrest and demise.

Keywords: continuous renal replacement therapy (crrt); deep vein thrombosis (dvt); endocarditis; fever; hemophagocytic lymphohistiocytosis (hlh); hlh; hyperferritinemia; pancytopenia; sepsis; splenomegaly.

Figure 1. CT of the abdomen/pelvis without contrast shows bilateral hydronephrosis.

Figure 2. CT of the abdomen/pelvis on hospital day 13 demonstrating retroperitoneal edema, a 5 mm left renal calculus, and the Foley catheter in place.

Figure 3. Declining cell lines over the course of hospitalization.

Reference ranges: white blood cells =  3.5-10.9 K/μL; hemoglobin = 13.0-17.7 g/dL; platelets = 140-400 K/μL.